A 67-year-old Chinese man with underlying comorbidity of diabetes mellitus was referred to the pulmonology clinic after a chest X-ray (CXR) done in January 2019 (workup prior to elective hernioplasty) showed an incidental finding of a left pulmonary mass. This gentleman had no respiratory or constitutional symptoms. Physical examination was unremarkable and his tumour markers were all within normal limits.
A contrast computed tomography (CT) of the thorax showed a lobulated mass of 3.4 × 2.7 × 2.6 cm in the posterior segment of the left upper lobe. Positron emission tomography (PET)-CT showed a lobulated FDG-avid mass in the left upper lobe with metabolic size of 3.7 × 3.0 × 3.8 cm without any evidence of distant lesions (Fig. ). A CT-guided biopsy was done suggesting a high-grade myogenic sarcoma.
He underwent left upper lobectomy that suggested a poorly differentiated PPSS on histological and immunohistochemistry (IHC) studies (Fig. ).
Post-operatively, a repeat PET-CT in March 2019 revealed only fluorodeoxyglucose (FDG) avidity at the right hilar region [standardized uptake values (SUVmax): 3.4], but no obvious nodule was seen on low-dose non-contrast CT. He was then treated with radiotherapy to the previous operative site after an oncology consult. No subsequent surveillance imaging was performed.
In September 2019, he presented with an acute history of fever, cough, and tachypnoea for three days. His blood investigations showed a raised white blood cell (WBC) count of 30.7 × 109, Hb 11.4 g/dL, an elevated C-reactive protein 318 g/dL, and erythrocyte sedimentation rate (ESR) of 73. The CXR on admission showed a left mid-zone opacity with air bronchogram. Physical examination only revealed coarse crepitations over the left lower zones.
He required endotracheal intubation and mechanical ventilation and was treated as pneumonia with intravenous antibiotics. A bronchoscopy found a fleshy growth at the main carina, obstructing both the right and left main bronchi (Fig. ). Multiple biopsies were done, for which the histopathology was reported as a metastatic synovial sarcoma. An urgent CT of the thorax and abdomen showed irregular peripherally enhancing lesions in the mediastinum with local extensions into the carina compressing bilateral main bronchus and ill-defined liver nodules were suggestive of metastases. An urgent referral was made for tumour debulking and stenting of the airway, but the patient's family members did not approve further intervention. After a week in intensive care unit (ICU), the patient succumbed to sepsis and disease progression.


A 61-year-old male presented to the emergency room with a three-day history of upper respiratory infection symptoms including cough, fever and myalgia. At presentation, he did not endorse any limb, ocular or bulbar weakness. Initial physical exam was unremarkable, and vital signs were within normal limits. Neurologic exam was also noted to be normal with no focal muscle weakness. Electrocardiogram (EKG) showed normal sinus rhythm with shortened PR interval of 96 ms (normal: 120-200ms). Shortly after the presentation, the patient was found to be unresponsive and pulseless. Telemetry monitoring was indicative of cardiac arrest with PEA. Advanced cardiac life support was initiated, the patient was subsequently intubated and cardiopulmonary resuscitation was continued. Return of spontaneous circulation (ROSC) occurred within seven minutes, and the patient was admitted to the medical intensive care unit (MICU). Post resuscitation EKG showed sinus tachycardia with shortened PR interval, similar to prior. The EKG immediately post cardiac arrest is shown in Figure .
Laboratory data on presentation were not obtained due to cardiac arrest, and first available set of laboratory investigations was after ROSC. Only significant abnormality noted on initial laboratory findings was lactic acidosis, which is expected after cardiac arrest. Lactate level quickly normalized within four hours' time. Laboratory results are shown in Table . Pulmonary embolism (PE) and pneumonia were highly suspected. However, CT angiogram of the chest was negative for PE and bronchoscopy was essentially normal. In addition, sepsis workup including blood cultures, chest x-ray and urinalysis were all found to be negative. Pre-excitation syndrome was ruled out by electrophysiology, and it was suggested that the shortened PR could be due to enhanced atrioventricular (AV) node conduction. A diagnosis remained unclear for the first three days of the MICU stay until the patient’s prior history of MG was verified by his primary care provider. Treatment with plasmapheresis and methylprednisolone was initiated resulting in significant improvement of the patient’s condition. Short PR-interval resolved after the completion of five sessions of plasmapheresis, and the patient was extubated shortly thereafter. The patient fully recovered and was discharged to regular follow-up and has remained stable thus far.


A 28-year-old woman presented with an expanding painful left-sided soft tissue swelling of the face. The mass was present for only a few months and without any associated facial nerve palsy. Examination revealed a round, well-circumscribed mass in the region of the parotid gland without overlying erythema, edema, or skin breakdown. Intraoral examination showed intact mucosa without evidence of invasion or erosion. CT scan images revealed a heterogeneous mass of the left parotid gland measuring approximately 4.0 × 2.7 × 3.9 cm with hypodense foci suggesting necrosis (). A nonspecific level II lymph node was also visualized. displays the ultrasound images of the mass.
Fine needle aspiration and biopsy performed were consistent with a pleomorphic adenoma. At that point, the decision was made to perform a left-sided parotidectomy with intraoperative facial nerve monitoring. The Blair incision () was utilized to help with a deep lobe dissection. The procedure revealed a mass extending to the deep parotid lobe. Careful attention was taken to preserve all branches of the facial nerve, and the mass was surgically excised ().
Histological findings displayed an intermediate grade myoepithelial carcinoma arising from a pleomorphic adenoma (Figures and ). The carcinoma showed greater than 1.5 mm invasion in to the surrounding adipose tissue (invasive type) with positive tumor-inked margins. The parotid lymph node did not show any evidence of malignancy. A pathological diagnosis of myoepithelial carcinoma ex pleomorphic adenoma was made. The patient successfully underwent adjuvant radiation therapy with a goal of 60 Gy.
Postoperatively, she experienced transient grade II House–Brackmann facial nerve dysfunction that completely resolved over the next few weeks. To date, she is asymptomatic and disease-free.


History
A 52-year-old African American male presented to the emergency department complaining of pleuritic, sharp pain in his right chest wall that had begun three days prior to arrival. The pain had since worsened in intensity, and did not radiate. He had taken acetaminophen without relief of his symptoms. The patient denied any fever, cough, shortness of breath, headache, or rash. His review of symptoms was otherwise negative. The patient had no significant past medical history and was taking no medications. He denied any recent history of surgery or trauma, but did note weight lifting two days prior to the onset of symptoms. Family history was negative for coagulopathies and autoimmune conditions.
Exam findings
Vital signs on arrival were blood pressure 146/97, heart rate 97, temperature 36.2°C, respiratory rate 20, and SpO2 99% on room air. He was alert and oriented, and in no acute distress. Lung sounds were clear bilaterally and heart sounds were normal. A raised cord was visible crossing from the epigastrium across the right breast toward the axilla, that was tender to palpation and mobile. There was no warmth, erythema, or rash overlying the cord. Head/eyes/ears/nose/throat, abdominal, and neurological examinations were unremarkable.
Diagnostic evaluation
Basic metabolic panel and troponin levels were normal, and a complete blood count and differential was unremarkable with platelets of 212 bil/L. Chest radiograph showed clear lung fields but did reveal remote right-sided posterior fractures of ribs five through eight. Given the pleuritic nature of the pain, a computed tomography pulmonary angiogram was ordered to rule out pulmonary embolism. As shown in Figure , it revealed a subtle density in the right chest wall over the area of the palpable cord, with no evidence of a pulmonary embolism. The diagnosis of superficial chest wall thrombophlebitis, or Mondor’s disease, was made.
Patient course
The patient was treated with ibuprofen in the emergency department and discharged with a naproxen prescription. He was referred to his primary care physician for further work-up. The pain improved in the days following his emergency department visit, resolving within a week of discharge. The patient was contacted two months after his emergency department visit. He noted that he was still able to appreciate a firm mass in his chest wall with some reduction in size since it was first diagnosed.


History
A 44-year-old woman with no past medical history presented to the emergency room at the insistence of her husband one day after a low impact motor vehicle collision. Airbags did not deploy; there was no loss of consciousness and the patient was ambulatory at the scene. The patient complained of a frontal headache, which was unchanged from daily headaches over the previous five months that a primary care provider had attributed to sinusitis. Her husband also reported aggressiveness, short temper, and increased sleeping over the previous four weeks. She drank alcohol socially and did not smoke cigarettes. She had remote family history of brain tumors. Both parents were alive and healthy.
Examination
Vitals signs are temperature 36.2ºC, blood pressure 119/62 mm Hg, pulse 57/min, respiration rate 16/min, oxygen saturation 99%, and body mass index 22.6 kg/m2. The patient was in no acute distress and cooperative during the examination. She was alert and oriented to self, time, and place with no focal neurological deficits. Vision was grossly intact with mild papilledema on the fundoscopic exam. She displayed aggressive behavior, arguing with her husband. The Karnofsky Performance Status was 90.
Diagnostic imaging
Computed tomography (CT) head without contrast demonstrated moderate obstructive hydrocephalus with mass effect between the frontal horns of the lateral ventricles at the level of the foramen of Monro, suggestive of an obstructive mass (Figure ). Magnetic resonance imaging (MRI) brain demonstrated an avidly enhancing, heterogeneous mass with significant necrosis, centered in the septum pellucidum (Figures , ). The mass measured 4.5 cm x 4.0 cm x 4.0 cm. There was an indication of intraventricular extension with subependymal enhancement and transependymal flow along the trigone and occipital horns of the lateral ventricles.
Hospital course
The patient was admitted to the intensive care unit with frequent neurological evaluations and started on dexamethasone. The next day, she underwent right frontal burr hole for stereotactic-guided biopsy. Frozen section suggested a high-grade glioma. She remained neurologically intact and was discharged home on post-operative day number two on a dexamethasone taper.
Pathology
Biopsy showed hypercellular glial tissue with pseudopalisading necrosis, microvascular proliferation, nuclear pleomorphism, and nuclear atypia (Figures -C) consistent with glioblastoma, WHO Grade IV. IDH1 and IDH2 were negative. MGMT promoter methylation was present.
Treatment and outcome
The patient was treated with radiation therapy for six weeks (30 cycles of 200 cGy), followed by maintenance temozolomide 200 mg/m2 days one through five every four weeks for 12 cycles. By completion of temozolomide, the tumor had shown remarkable regression. One year after treatment, there remained little evidence of abnormal MRI signal in the area of the neoplastic lesion with complete resolution of the hydrocephalus (Figure ). She noted improvement of her headaches and normalization of her aberrant behavior.
She underwent routine MRI brain every three months. More than 25 months after her last dose of temozolomide, two foci of enhancement were noted in the right ventricle (Figure ). Brain MRIs two and four months later clearly demonstrated recurrence of the tumor. She completed two rounds of temozolomide with no decrease in tumor size. A repeat biopsy for genetic analysis was positive for BRAF. After two months of lomustine, she developed thrombocytopenia. She was offered BRAF and MEK inhibitors but declined therapy.
Routine MRI scans remained stable for nine months without intervention; at that time MRI demonstrated remarkable local progression of the tumor (Figure ). She underwent no further treatment. She died six months later from progression of her brain tumor, more than five years from diagnosis.
Of note, the patient remained active and functionally independent until the last months of her life. She had an extensive network of support and exhibited an attitude of battling until the last moment.


A 52-year-old male patient with symptoms of lower abdominal pain, dysuria and pollakiuria was admitted to Urology department of our Hospital. The patient did not have gross hematuria, no past surgical history and other disease. The patient had some antibiotics for urinary tract infection for the past 2 year. Physical examination revealed severe tenderness in lower abdomen. Vital signs were normal. Digital rectal examination revealed normal prostate. Urinalysis showed that pH5.0 and presence of calcium oxalate crystals and leukocyturia but erthrocyturia and nitrite were absent. Routine hemogram was normal. Blood urea nitrogen, serum creatinine level and uric acid level were 4.17 mmol/L,167 μmol/L, 59.7 μmol/L respectively. Kidney and liver function were normal. Abdominal ultrasonogry revealed hydronephrosis, thickened bladder wall and large single stone. Plain radiography showed a large bladder stone measuring 12 × 10 cm (). 24-h urine specimen analysis showed that calcium was 7.1 mmol/day and oxalate was 0.62 mmol/day.
Patient underwent open cystolithotomy under general anesthesia and larger bladder stone was removed. The bladder stone weighed 950 g and measured 12.8 × 9.2 × 7.2 cm (). Stone analysis showed stone composed of 91% calcium oxalate monohydrate and 9% oxalate.
Hydronephrosis recovered to normal level within 5days. Patient decreased the consumption of oxalate-rich food. Patient was discharged on day 8. At 3 months of follow-up, the patients renal function was normal and voiding dysfunction disappeared.


A 68-year-old male presented to the outpatient endoscopy unit for a repeat EUS drainage of a PP.
The patient has a background history of necrotising gallstone pancreatitis that was treated supportively. A subsequent laparoscopic cholecystectomy was performed. Unfortunately, he developed a PP that required EUS-guided drainage approximately 12 months following his index presentation. A surveillance CT abdomen demonstrated a large recurrence of his PP (approximately 13 cm in largest diameter); thus, a decision for repeat drainage was made. His other comorbidities include chronic obstructive pulmonary disorder, hypertension, and a solitary kidney (donor nephrectomy).
A linear echoendoscope was advanced to the second part of the duodenum. External compression of the posterior gastric wall was observed endoscopically. Endosonographically, the PP measured 13 x 10 cm and was punctured using a 19-gauge access needle. Approximately 10 ml of clear fluid was aspirated, and then injected with 20 ml of contrast. A 450-mm Jagwire was passed through the needle into the pseudocyst under fluoroscopy. The access needle was exchanged for a cystotome. A cystpogastrostomy was performed using the cystotome, and a second 450-mm Jagwire was inserted. The cystotome was removed, and two 7 French plastic double pigtail stents were introduced into the PP over the two guidewires under direct fluoroscopy. Both stents appeared to have been positioned satisfactorily, and were draining clear fluid into the stomach (Figure ).
The patient developed severe abdominal pain in the recovery bay. An urgent CT abdomen demonstrated free fluid and gas in the upper abdomen especially anterior to the liver (Figure ). A decision was made to proceed to the operating theatre given concerns for a perforated hollow viscus.
A midline laparotomy was performed. Significant amounts of gastric and pancreatic juices were found throughout the entire peritoneal cavity and evacuated. The gastrocolic ligament was taken down with the harmonic scalpel to allow access to the pancreas. The cystogastrostomy tract and stents were found to have inadvertently entered the peritoneal cavity (Figure ), and thus were removed.
A decision was made to surgically decompress the PP given the high likelihood of recurrence. An anterior longitudinal gastrotomy was performed. There was difficulty locating the PP through the posterior stomach wall as it had collapsed, having spilled its contents into abdominal cavity. The PP cavity was not identifiable despite using an intraoperative ultrasound (IOUS). An 8-French infant feeding tube was inserted through the peritoneal defect caused by the transgastric stent, and placed into the PP cavity. The feeding tube was subsequently injected with saline (Figure ).
A subsequent attempt at transgastric localisation of the PP was successful using IOUS given the re-expansion of the PP. A seeking needle was then used to confirm the location of the PP. A 45-mm Endo-GIA (Medtronic plc, Minneapolis, MN) stapler was used to complete the cystogastrostomy, and the edges oversewn with 3-0 prolene (Figure ). The anterior gastrotomy wound was closed with 3-0 PDS. The PP defect and corresponding gastrotomy defect were closed with 3-0 PDS. A feeding jejunostomy was fashioned. The abdomen was washed with saline and two drains placed: one on the anterior stomach and one on the closed pseudocyst defect.
The patient recovered well postoperatively. A CT contrast swallow was organised on day 10 after the operation, which demonstrated no evidence of any leak. The abdominal drains were removed after drain amylase levels were found to be normal. The patient was initially supported with postpyloric feeds through his feeding jejunostomy, and then slowly transitioned back into a regular diet. He was discharged from hospital two weeks after his operation. He remains free from any recurrence 13 months after his surgery.


The patient was a 66-year-old Caucasian female with a history of HS since 2010 with Hurley stage III lesions in the groins with six nodules and six sinus tracts. She had a HSS score of 63, DLQI score of 13, and visual analogue scale (VAS) for overall disease-related distress of 5.5 and VAS for boil-associated pain from boils in the past month was 0. The patient also had hypertension, smoked 20 cigarettes per day for 54 years and had a BMI of 26.4 kg/m2. She had no family history of HS. The patient was first seen in our clinic in 2016, where treatment with topical clindamycin twice daily and oral tetracycline 500 mg twice daily was initiated along with topical corticosteroids for local eczema. At 3-month follow-up, the skin was characterized by fibrotic and thickened cicatricial areas with purulent drainage and inflamed nodules. Topical metronidazole and oral lymecycline 300 mg twice daily were administered and the patient was referred to wide excisional plastic surgery of the groins, provided she stopped smoking 2 months prior to surgery.
Surgery was canceled due to continued smoking, and no further surgery was planned. She failed to seek further help for her HS until she was admitted 3 years later after severe worsening of HS, with multiple severe fistulae and a large foul-smelling tumor with drainage in the left groin and necrosis of lymph nodes (Figure ). A PET-CT showed ingrowth and destruction of the left ramus inferior of the pelvis and gland metastasis under the diaphragm. Biopsy demonstrated invasive poorly differentiated SCC, transformed from several years of chronic HS. The clinical picture was compatible with ulcerating vulvar cancer, and the patient received radiation therapy as a palliative measure. However, she was shortly thereafter deemed terminal and died 1 month after the initial metastatic SCC diagnosis.
A paper in 2016 by Jourabchi et al and in 2018 by Chapman et al described 80 and five cases, respectively, on SCC in patients with HS cases in the English literature. We found five additional cases of SCC arising from HS reported in the literature since the last updated review by Chapman et al (Table ). Among these five new cases, the mean history of HS prior to SCC diagnosis was 16 years, and the mean age at diagnosis was 51.2 years. SCC developed predominantly from long-standing HS of the gluteal and perianal areas. A history of tobacco use was reported in two of the five cases. Interestingly, the occurrence of SCC in the context of axillary HS was not reported in the English literature before Dessinioti.


A 46-year old Caucasian woman presented with a recently diagnosed m.3243A>G mutation (60% level of heteroplasmy in the biceps brachii muscle) without harbouring a MELAS phenotype. Clinical findings included optic neuropathy, sensorineural hearing loss and diabetes mellitus type I. Stroke-like episodes were not reported. Her medical history included intermittent atrial fibrillation, ketoacidosis, pneumogenic sepsis, liver and kidney failure, cor pulmonale and tricuspid and mitral regurgitation. Family history revealed no evidence for neuromuscular disorders. Clinical examination showed pronounced sensorineural hearing loss, generalized tetra paresis (MRC 2–3/5), and muscle tendon reflexes could not be obtained. Serum creatine kinase (CK) levels were normal. Neurophysiological examinations showed evidence for critical-illness polyneuropathy without signs for acute Guillain–Baré Syndrome. Lumbal puncture and brain magnetic resonance imaging (MRI) were not performed, due to the poor general condition.
After 30 days, the patient developed distinct hypokalaemia. In case of suspected sepsis with hypotension and increasing inflammatory values, the patient was transferred to the neurological intensive care unit. The patient developed a rapid state of shock with pronounced lactate acidosis, acute kidney and liver failure and hypothermia (33°). Thorax and abdominal CT (computed tomography) scan showed no sepsis focus. Calculated antibiosis was initiated, among the inflammatory values, and improved significantly. On haemodialysis, lactate acidosis was also declining. However, the cardiopulmonary situation worsened, and the patient became catecholamine dependent. In addition, the liver enzymes increased, and a plasmatic coagulation was no longer detectable. Echocardiography showed cardiomegaly with decreasing ejection fraction. In the further course, the patient was in need of reanimation, but could be stabilized. The patient quickly developed a pulseless electrical activity, which caused death after resuscitation. Autopsy revealed an acute right-left heart failure with multi-organ failure after re-edivating pulmonary embolisms, starting from a multi-stage phlebothrombosis and a parietal thrombosis of the right atrium. Aggravating was an advanced high-grade coronary atherosclerosis with more than 90% stenosis in all main vessels and evidence of diffuse myocardial scars and multi-organ failure, as well as florid purulent bronchopneumonia.


A 74-year-old man had two tumors in the right upper lobe that were resected through right upper lobectomy. Both tumors morphologically had an irregular surface; thus, they were diagnosed as primary lung cancers (A,B). Pathologically, the peripheral lesion was identified as an adenosquamous carcinoma comprised of squamous cell carcinoma and acinar-predominant adenocarcinoma, whereas the central lesion was identified as papillary-predominant adenocarcinoma (C,D). On the basis of the histopathological differences, the tumors were judged as double primary tumors. Pathologically, the cancer stage was determined to be pT1cN2M0, stage IIIA. However, the genetic mutation profiles were completely consistent between these two tumors, suggesting they are metastases (E). Moreover, their mutation profiles were also consistent with the mutation profile of the metastatic lymph node. (E). Based on the genetic diagnosis, the cancer stage was ultimately upgraded to T3N2M0, stage IIIB. At the patient’s request, he was placed on follow-up without any postoperative adjuvant chemotherapy. The patient has remained alive for 2 years postoperatively without any recurrence.


A 59-year-old woman presented with 0.7-cm nodules in the right lower lobe 1.5 years after undergoing right upper lobectomy for cancer. The tumors were round and had a smooth surface. Because of their morphology, they were suspected of being metastatic lesions. After 4 months of follow-up, there was no increase in the number of lung lesions, suggesting solitary intrapulmonary metastasis. Subsequently, wedge resection was performed. Although both tumors were pathologically papillary-predominant adenocarcinoma (C,D), a lepidic pattern was observed in the periphery of the smaller nodule (E), leading to a diagnosis of double primary lung cancers. However, the genetic mutation profile was consistent between the two tumors, suggesting them to be metastases (F). The patient was positive for a mutation in the epidermal growth factor receptor (EGFR) gene (exon 19 deletion); hence, oral administration of an EGFR-tyrosine kinase inhibitor (gefitinib) was continued. The patient has remained alive without recurrence for 4 years after the second surgery.


A 74-year-old man presented with tumors measuring 4.0 cm and 1.8 cm in the left upper lobe, so left upper lobectomy was performed (A,B). As both tumors were closely located and pathologically similar squamous cell carcinomas, they were assumed to be single origin pulmonary metastases (C,D). However, the mutation profile was completely different between the two tumors genetically, suggesting double primary cancers (E).


A 74-year-old man, described as case C in the previous section, presented with paratracheal and mediastinal lymph node metastases 1 year after left upper lobectomy (F). Although it was not possible to pathologically identify the metastasizing primary lesion (G), the mutation profile of the metastatic lymph node was genetically consistent with that of the larger cancer. The genetic diagnosis was lymph node metastasis of the larger cancer ().


A 77-year-old man with lung cancer underwent left lower lobectomy (A). One year later, a nodule appeared in the middle lobe (B). Middle lobectomy was performed based on the assumption that the lesion was a double primary tumor. However, after 1 year, subcarinal lymph node metastasis occurred (C). Pathologically, all three lesions were of squamous cell carcinoma type and it was impossible to determine which primary lesion had metastasized (D–F). Given the tumor size, the tumor in the left lobe was clinically more likely to have metastasized. However, mutation analysis revealed that the two lung lesions had different mutation profiles; therefore, they were diagnosed as double primary lung cancers. Furthermore, the mutation profiles were consistent between the middle lobe lung cancer and the metastatic lymph node. Thus, lymph node metastasis of the middle lobe lung cancer was determined (). Programmed death-ligand 1 (PD-L1) staining of tumor cells was 0% and 90% in the left lower lobe and middle lobe tumors, respectively. Treatment with an anti-PD-1 antibody (nivolumab) was administered and a complete response has been maintained for 1 year since the recurrence in the lymph node.


A 50-year-old female patient presented with hematuria, massive weight loss (45 kg), asthenia and right lumbar pain 7 months prior to the consult. Previous blood work-up revealed anemia and previous CT and ultrasound revealed a solid injury of 10 cm on the right kidney and a mass in the right flank (). The patient denied previous family history of neoplasia and previous diseases, although she confirmed smoking habits.
Physical exam revealed a palpable mass with roughly 10 cm at the right hypochondrium and another palpable mass situated at the dorsal surface of the left lumbar region with roughly 5 cm. The patient had regular and stable vital signs.
Routine admission blood work-up confirmed anemia, and a contrasted CT scan revealed a solid mass on the right kidney (12 cm) and a solid mass situated on the muscle plane of the abdominal wall muscles (7 cm). The patient was promptly submitted to a right total nephrectomy, retroperitoneal lymphadenectomy, right total adrenalectomy and a cavotomy with the purpose of removing the caval thrombus.
The tumor and the excised kidney () were submitted to a histopathological analysis, which confirmed renal cell carcinoma of clear cell type with sarcomatoid cell type component (). The histologic grade of the tumor was 4 and the tumor was associated with neoplastic thrombus in the inferior vena cava (pT3b).
The surgery was uneventful and since the patient made a good recovery from the procedure, she was subsequently discharged from the hospital. One month later, the patient returned to the hospital for a tumor resection of the abdominal wall, which raised suspicion of metastasis.
After resection, the tumor was sent to histopathological analysis, which revealed a neoplasia with myxoid component and a predominance of fusiform cells (). The immunohistochemical profile was unspecific, with characteristics of low grade fibromyxoid sarcoma.
Since the surgery was successful and there were no complications during post-op, the patient was discharged and subsequently monitored during routine check-ups.


Patient 1 who was a 43-year-old male was first referred to our institute because of an allergic reaction to metronidazole with oral mucosal erosions during his sixth treatment for amebic colitis. He had a history of 5 recurrent episodes of amebic colitis (last treatment was 3 years earlier, using metronidazole followed by paromomycin) (). Besides oral mucosal erosions, he complained of soft or loose stools 2 to 3 times daily without abdominal pain or fever. Although we proposed admission for close observation during his treatment, he selected outpatient treatment at a nearby hospital. Three months later, the patient returned to our hospital because his wife was also diagnosed with Entamoeba histolytica infection. The couple operated a Japanese inn in a suburban area of Tokyo. They had no travel history to developing countries within the past 10 years. He denied extramarital sexual intercourse and oral–anal sexual contact. He did not have any past histories, except for recurrent amebiasis. There were no reported outbreaks of gastrointestinal diseases for over 10 years in the couple’s residential area. Results of a blood examination showed no particular abnormalities (). Although a direct microscopic examination was negative for any protozoa, the patient’s stool tested positive for E. histolytica with polymerase chain reaction (PCR). Total colonoscopy showed white-coated ulcerative lesions at the cecum (). In a pathological examination, Entamoeba was identified on the surface mucosa in a biopsy sample (). We treated the patient with a lumen-active agent (paromomycin monotherapy) because (1) he had a past history of acute oral mucosal lesions owing to metronidazole, (2) tinidazole is not approved to treat amebiasis in Japan, and (3) his symptoms of E. histolytica were mild. Negative PCR results for E. histolytica were confirmed in stool samples taken at 1, 2, and 4 months after treatment. Follow-up colonoscopy showed that lesions of the cecum were completely resolved ().


Patient 2 was a 43-year-old female and the wife of patient 1. She was referred to our institute because of Entamoeba infection as confirmed on microscopic examination of a stool sample. One month before diagnosis, she had a positive fecal occult blood test result in an advanced health check. Dysentery, abdominal pain, and fever were not documented at referral. She denied extramarital sexual intercourse. She had a high anti–E. histolytica antibody titer (1:800). Direct microscopy of stool samples showed the cystic form of Entamoeba. E. histolytica was confirmed using PCR (). Total colonoscopy showed multiple, white-coated, ulcerative lesions from the cecum to the sigmoid colon (). The patient was treated with paromomycin monotherapy because her symptoms were mild, and she wished to avoid the potential adverse events of metronidazole experienced by her husband. PCR for E. histolytica in stool samples collected at 1, 2, and 4 months after treatment were negative. Follow-up colonoscopy showed that all lesions had completely resolved ().
We informed both patients about risk behavior for acquiring E. histolytica, such as oral–anal sexual contact or food and waterborne infections in poor sanitary settings. More than 2 years after treatment, neither patient has experienced recurrence of invasive E. histolytica infection.


The patient is a 29-year-old male with a past medical history of poorly controlled type II diabetes mellitus, hypertension, tobacco abuse (one pack-per-day), and leukemia at the age of nine months for which he received systemic chemotherapy, total body radiation, and bone marrow transplant. The patient had not seen a doctor or been on any medications for the past five years. The patient presented to a hospital after a syncopal episode. CT head showed a right sphenoid wing meningioma, which was 4.3 x 4.1 x 2.2 cm in size with mass effect. He was admitted to the hospital and treated with hypertonic saline and levetiracetam for increased intracranial pressure.
During the hospital stay, the patient reported intermittent episodes of chest pressure lasting for 15-20 minutes each. Given the typical nature of his chest pain, transthoracic echocardiography was performed, which revealed a left ventricular ejection fraction of 45%-50% with anteroseptal hypokinesis (Figure ). He then underwent cardiac catheterization, which revealed 95% stenosis of the proximal left anterior descending (LAD), 60%-70% stenosed ostial left circumflex, and mid-portion of the right coronary artery (RCA) with 95% stenosis. It also revealed an elevated left ventricular end-diastolic pressure. The patient was then transferred to our hospital for a higher level of care.
Cardiothoracic surgery was consulted given the patient's triple vessel disease, and the patient underwent CABG with LIMA graft to the LAD and reverse saphenous vein graft to the posterolateral circumflex and posterior descending artery (PDA). Within two hours postoperatively, the patient developed ischemic changes in the anterior and lateral leads (ST elevation in V2, I, and augmented vector right (AVL)). See Figure .
Given the EKG findings of STEMI, the patient was taken for emergent coronary angiography, which demonstrated diffuse vasospasm in the body of the grafted LIMA conduit (Figure ). Intracoronary infusion of nitrates successfully relieved the spasm (Figure ). The patient was then continued on a low-dose nitroglycerin infusion for a day after the angiography.
On the first postoperative day, the patient again experienced angina and persistent ST changes were noted in leads V2, V3, and V4 with a maximum troponin level of 3.46 ng/ml. He underwent a redo CABG emergently with anastomosis of the reverse saphenous vein graft to the distal LAD and aorta. The patient’s recovery was uneventful thereafter, and he was discharged home in a stable condition on the ninth postoperative day after the redo CABG.


The patient is a 69-year-old male with a past medical history of dyslipidemia, brain aneurysm status post repair, benign prostatic hyperplasia, thyroiditis who was seen inpatient as he presented with shortness of breath, difficulty swallowing, cough, fatigue, unintentional weight loss of 12 pounds in the last five weeks. Upon his first admission to the hospital, his labs were: WBC: 9.8x 103/uL , RBC of 4.42x106/uL, hemoglobin of 13.3 g/dl, hematocrit of 41.4%, MCV of 93.7fl, MCH: 30.1 pg, MCHC of 32.1 g/dl, RDW of 42.1 fl, and a differential showing increase in lymphocytes of 11.1%. His chemistries showed a sodium of 144 mmol/l, potassium of 4.1 mmol/l, magnesium of 2.3 mg/dl, phosphate of 3.3 mg/dl, AST of 20 U/l, ALT of 45 U/l, creatinine of 1 mg/dl, albumin of 3.4 g/dl, and CA 19-9 of <1.4. Initial imaging on CT chest was found to have a mild pericardial effusion with bilateral pleural effusions, multiple nonspecific small minimally prominent bilateral cervical lymph nodes with small-to-moderate confluent consolidation in the posterior central lingula extending to the hilum, with the impression that malignancy could not be excluded (figure ). He then proceeded to undergo a thoracentesis. He subsequently developed fairly rapid reaccumulation of fluid and was concerned about a potential underlying empyema and therefore, transferred to a facility with thoracic surgery specialty. At our facility, cardiothoracic surgery performed a pericardial window.
The final pathology of 600ccs of serosanguineous fluid was suggestive of poorly differentiated malignancy, with stains positive for CK7, TTF1 and NAPSIN A, suggestive of metastatic stage IV adenocarcinoma of the lung. Additional stains showed that he had negative PAX8 and CD68, and was also negative for ALK mutations. An additional gene study was done which showed positive BRAF V600E mutation. He eventually had to have placement of a pleural catheter on the left side, since there was continuous reaccumulation of pleural fluid.
He had another episode of pleural effusion on the right side, had a right side thoracocentesis, and developed a pneumothorax for which he had to have a chest tube inserted. His pneumothorax improved, and he was discharged home and instructed by his oncologist to start BRAF inhibitors and MEK inhibitors. While he was following outpatient with oncology, over time, his chemotherapy regimens included being started on palliative Carboplatin, Alimenta with Keytruda, then Tafinlar/Mekinist combination, and then finally on Vemurafenib. He stated the BRAF inhibitors did help him. He currently follows outpatient with oncology.


A 79-year-old man underwent open hepatectomy for a hepatocellular carcinoma 4.3 cm in diameter. One year later, abdominal computed tomography revealed a locally recurrent tumor 1.5 cm in diameter and a giant incisional hernia measuring approximately 15 × 6 cm on the supraumbilical midline. Consequently, the incisional hernia was repaired using a free fascia lata graft patch after repeat hepatectomy. During the surgery, the fascia lata was harvested from the left thigh. An incision approximately 12 cm long was made on the lateral side of the thigh to allow for the dissection of subcutaneous tissue and harvest a piece of fascia lata measuring 16 × 8 cm. After the hepatectomy, the skin and subcutaneous tissues were dissected down to the level of the anterior rectus sheath on both sides of the defect, exposing the native fascia along the edge of the defect. After suturing 2 cm of the top and bottom edges of the native fascia lata, the fascia lata graft was used to reconstruct the remaining defect, which measured 12 × 6 cm. For placement of the graft, the fascia lata was sutured superficial to the edge of the defect with minimal overlap (interpositional graft) () [].
Two years later, the patient was readmitted because of recurrent tumors measuring 3.0 and 1.0 cm in the S5 and S7 subsegments of the liver, respectively. Preoperative magnetic resonance imaging (MRI) revealed the implanted fascia lata graft above the sheath of the rectus abdominis as an interpositional graft (). Repeat hepatectomy was performed. A ventral midline incision was made and the skin and subcutaneous tissues were dissected down to the level of the fascia, which was white and hard, similar to an implanted fascia lata graft (). The fascia lata graft had survived well and become incorporated into the native fascia. We incised this fascia lata graft in the same way as for a normal laparotomy (). After limited hepatectomy had been performed, the midline abdominal fascia, including the fascia lata graft and the skin, was closed in layers with interrupted suture and skin staples, respectively. The patient was discharged on postoperative day 11 with no wound-related morbidity or hernia recurrence. Three months after surgery, MRI revealed that the resutured fascial graft had healed completely to the same state as that evident preoperatively, and the patient is doing well for 11 months after surgery without hernia recurrence.


A 5-year-old boy with a history of uncontrolled, moderate-persistent asthma presented to the pulmonology clinic for asthma management. The patient had a previously normal developmental history, up-to-date immunization record including Streptococcus pneumoniae and Haemophilus influenzae, and notable past medical history significant for recurrent otitis media due to poor compliance to oral antibiotics, which were subsequently successfully treated with parenteral antibiotics. Upon presentation, the patient recently had a left lower lobe community-acquired pneumonia that was treated with one dose of intramuscular ceftriaxone in the emergency department followed by a standard 10-day course of amoxicillin. The patient's family was instructed to open the capsule and mix the powder with food to improve compliance due to known history of poor palatability with previously prescribed antibiotics. The patient returned to the emergency department two weeks later with worsening of symptoms and was treated with oral cefdinir for unresolved pneumonia. He was subsequently seen in the pulmonology clinic four days later to establish care without acute complaint. He was started on fluticasone/salmeterol and a five-day course of oral prednisolone for management of an acute asthma exacerbation.
One month later, the patient presented to the emergency department with fever and increased cough and was diagnosed with right middle lobe pneumonia. He was treated with intramuscular ceftriaxone to be followed with a home course of oral antibiotics, which was reportedly completed. Two weeks later, the patient again presented to the emergency department with recurrent symptoms and diagnosed with right lower lobe pneumonia that failed outpatient treatment. The patient was subsequently admitted to the medical floor and managed with a single dose of intravenous ceftriaxone. He was discharged the following day in stable condition on cefdinir and azithromycin. The patient was to follow-up as an outpatient with his pulmonologist and primary care physician. Hospital laboratory work including CBC with differential, peripheral blood cultures, sweat chloride test, immunoglobulin assay, T cell count, and Aspergillus antibody was unremarkable. At the two-week follow-up, he had persistent fever, wheezing, daytime and nighttime cough, shortness of breath, exercise intolerance, and difficulty breathing. The patient was scheduled for further evaluation with high-resolution chest CT without contrast and flexible bronchoscopy with bronchoalveolar lavage and nasal cilia biopsy to evaluate for Primary Ciliary Dyskinesia (PCD). Repeat chest X-ray at that time revealed an improved right lower lobe consolidation without complete resolution, as expected. CT scan revealed a mild degree of bronchiectasis in the lower lobes, bilaterally, associated with consolidation on the right and linear atelectasis and scarring on the left with other nonspecific evidence of chronic inflammatory versus infectious process. Bronchoscopy identified multiple mucus plugs in the right bronchus intermedius (Figures –), right middle lobe (Figures and ), and right lower lobe (). Gross inspection of the bronchoscopy sample revealed a solid, rock-like mucus plug (). Airway clearance via a high-frequency chest wall oscillation vest along with nebulized therapies was initiated. The patient did not tolerate postoperative oral azithromycin and required mixing with grape juice.
One week following the procedure, he was readmitted for recurrent fever and a new rounded lucency on chest X-ray in the right lung base, suspicious for cavitation or pneumatocele. Chest CT demonstrated a 2.5 × 3.2 × 3.7 cm area of cavitation within a region of consolidation or bronchiectasis in the posterior aspect of the right lower lobe with mediastinal and right hilar adenopathy, suspicious for cavitary bacterial pneumonia or fungal disease (). Home treatment continued with the addition of intravenous ceftriaxone. Bronchoalveolar lavage culture from the previous week grew beta lactamase negative Haemophilus influenza and Moraxella catarrhalis. Fungal culture, mycobacterial culture, bronchial aspirate cell count, cytology, gastrin pepsin assay, and pathology report were all unremarkable. A PICC line was placed for 3 weeks of home antibiotics as a management for complicated cavitary pneumonia. Further workup included QuantiFERON-TB Gold, HIV assay, complement assay, tetanus antibody, diphtheria antibody, and pneumococcal antibody which were all unremarkable.
At 3-week follow-up post hospitalization, the patient was symptomatically improved without coughing or fever and he was reported to be back to his baseline. The mother reported adherence to antibiotic regimen through the PICC line. He continues maintenance therapy with fluticasone/salmeterol, montelukast, and vest therapy. He was started on oral azithromycin three times weekly. At the two-month follow-up, the patient remained asymptomatic, and repeat imaging at the nine-month follow-up revealed complete resolution of cavitating consolidation with residual bilateral mild bronchiectasis ().


A 37-year-old female underwent surgery for a thyroid nodule of the right lobe. The nodule presented as suspicious because of its hard consistency, its adhesion to surrounding tissues, and the presence of calcifications on ultrasound. The patient was euthyroid before surgery. She had a total thyroidectomy. Histological study showed a granulomatous thyroiditis. The granulomas were composed of lymphocytes and histiocytes grouped into clusters. After surgery, the patient was referred to our establishment for investigation of a polyuropolydipsic syndrome and a one-year history of amenorrhea-galactorrhea.
Physical examination was normal except for bilateral galactorrhea. Urine specific gravity before test was 1.005. The water deprivation test results were in favor of a central diabetes insipidus (DI) (). Hormonal tests revealed pan-hypopituitarism, with a very low follicle-stimulating hormone (FSH) level of 0.9 mIU/mL (reference range 3.9–8.9 mIU/mL) and a luteinizing hormone (LH) level below 0.1 mIU/mL (reference range 2.1–10.9 mIU/mL). Baseline serum cortisol was 240 nmol/L and did not respond to insulin hypoglycemia test. An inappropriate adrenocorticotropic hormone (ACTH) level of 12 pg/mL suggested secondary adrenal insufficiency. Likewise, baseline GH was 0.25 ng/mL and did not respond to insulin hypoglycemia test. Prolactin level was mildly elevated (2520 mIU/mL with a reference range of 71–567 mIU/mL), which was consistent with disconnection hyperprolactinemia. As for the thyroid function tests, they were noninterpretable because the patient had already been on therapy. However, it is important to note that thyroid-stimulating hormone (TSH) level responded to TRH stimulating test. MRI of the hypothalamic-pituitary axis showed an infiltrative process of the hypothalamus with a loss of the posterior pituitary bright spot. The patient was discharged; she received hormone replacement therapy including desmopressin acetate 20 µg intranasal solution per day, oral hydrocortisone 20 mg per day, and levothyroxine 150 μg per day.
Eight months later, she presented with a left retroauricular bone pain and preauricular and submandibular lymphadenopathies, along with dental pain and tooth mobility. The facial and temporal bones CT-scan revealed a lytic lesion of the horizontal branch of the right mandible and a significant lytic lesion of the left petrous bone, extended to the squama (). The diagnosis of LCH was then strongly suspected, which led to re-examination of the excised thyroid gland by the pathology department. The immunohistochemistry study was performed, and it revealed a strong positivity of histiocytes for the CD1 antigen and the S100 protein ().
The extension assessment of the disease included a thoracic CT-scan that revealed peripheral pulmonary micronodules, some of which were excavated. The treatment consisted of a chemotherapy combining vinblastine at a dose of 5 mg weekly and prednisone at a dose of 0.5 mg/kg daily. After four months of treatment, favorable evolution was noted, with a good tolerance of treatment, a clear relief of the headache, and disappearance of the left retroauricular pain. The control temporal bones CT-scan showed complete regression of the lytic lesion (). Nevertheless, the patient still required the same doses of pituitary hormones replacement therapy.


A 32-year-old male patient was diagnosed with chronic renal failure in December 2014, and his serum creatinine gradually increased later. He entered continuous ambulatory peritoneal dialysis in September 2016 (peritoneal dialysate 2000 ml qid, calcium concentration 1.5 mmol/l). More than 1 year ago, there were multiple gray-white granular nodules on the patient's skin, mainly distributed in the neck and back, without pain and itching. These nodules gradually progressed to the upper limbs and shoulders in the following six months (). After extruding, the nodules can be partially detached, leaving pigment spots of 0.2 to 1.5 cm in size. The patient was scheduled for a metastable Technetium-99 methyl diphosphonate (99m TcMDP) bone scan, and a significant positive result was obtained. Angiographic flow phase images showed asymmetric perfusion in the lower extremities with the left greater than the right (). Delayed images showed moderate-to-intense uptake related to superficial soft tissues of the left calf muscles (). Fusion images showed that the areas of abnormal radiotracer uptake were at the left lateral gastrocnemius muscle (). The patient took calcium carbonate 2-3 tablets everyday for 3 months in 2015 and Rocaltrol 0.25 g/d from September 2016 till now. He had previous history of hypertension and hepatitis B but no diabetes, without glucocorticoids, immunosuppressants, warfarin, and heparin application history. Bone scan results strongly suggest a diagnosis of calciphylaxis. For further diagnosis and treatment, skin biopsy (pea-sized tissue) was performed, and the results showed subcutaneous arteriole calcification and extensive calcium salt deposition confirming calciphylaxis (). Then, the patient was treated with sodium thiosulfate (6.4 g/d) for 21 days, and the skin lesions were diminished.


Patient 1 is a 69-year-old female whose primary cause of the liver disease was nonalcoholic steatohepatitis complicated by hepatocellular carcinoma (HCC). Her preoperative Model End-Stage Liver Disease (MELD) score was 9 and 28 after the addition of HCC points. She also had multiple medical comorbidities including hypertension, obstructive sleep apnea, and obesity and a past surgical history of Cesarean section and hysterectomy. She received a deceased donor orthotopic liver transplant, performed with piggyback technique of veno-veno bypass with an arterial reconstruction of donor celiac artery to recipient right replaced hepatic artery and choledocho-choledochostomy. Patient 1 had a height of 154.9 cm and a weight of 84.4 kg, while the donor had a height of 170.2 cm and a weight of 122.6 kg, giving a donor-weight-to-recipient-weight ratio of 1.45. The liver had a volume of 2049 cubic centimeters (cm3) (as estimated by donor preoperative CT volumetric studies) and was deemed to be large relative to the patient's abdomen. At the end of the transplant, the fascia could not be closed primarily without risking compression of the graft. A Vicryl knitted mesh was used as a bridge to close the fascia without tension. She did well postoperatively and was discharged on postoperative day 6. During the course of her follow up, she developed a right anterolateral ventral hernia. The repair has been delayed due to concerns of recurrence given her BMI of 33. Her transplanted liver is doing well, with normal liver function tests at 21 months postoperatively.


Patient 2 is a 54-year-old male with hepatitis C with subsequent hepatocellular carcinoma as a primary cause of liver disease, complicated by esophageal varices, portal hypertension, and ascites requiring paracentesis. His preoperative MELD score was 17. He received a deceased donor orthotopic liver transplant with a graft volume of 1670 cm3 (as estimated by donor preoperative CT volumetry.) His height was 165 cm and his weight was 66.9 kg, and his donor's height and weight were 172.2 cm and 70.7 kg, respectively. His donor-weight-to-recipient-weight ratio was 1.05. The transplant was performed using a piggyback technique of veno-venous bypass with a choledocho-choledochostomy and recipient ductoplasty. Primary fascial closure was not possible due to the large liver size, so a bridging Vicryl knitted mesh was inserted in the right subcostal region to prevent abdominal compartment syndrome. On postoperative day 4, the patient's liver enzymes were elevated, requiring a transjugular liver biopsy that showed mild acute cellular rejection. This was resolved with three days of pulse steroids. The patient recovered well and was discharged on postoperative day 11. MRI performed 4 months postoperatively demonstrated a hernia in the lateral abdominal wall. The patient remains asymptomatic and does not wish to pursue repair. Graft function remains excellent at 26 months post-op.


Patient 3 is a 59-year-old female whose primary cause of the liver disease was nonalcoholic steatohepatitis with a preoperative MELD score of 29. Her liver disease was complicated by hepatic encephalopathy and ascites requiring paracentesis. Her comorbidities included depression, anxiety, colitis, and tricuspid regurgitation, and history of roux-en-y gastric bypass. She received a deceased donor orthotopic liver transplant with a graft volume of 1977 cm3 (as estimated by donor preoperative CT volumetry). At the time of surgery, her height was 167.6 cm, and her weight was 78.1 kg. Her donor height and weight were 165.1 cm and 92 kg, respectively, giving a donor-weight-to-recipient-weight ratio of 1.18. The transplant was performed using the piggyback technique on veno-venous bypass with choledocho-choledochostomy and recipient ductoplasty. A large amount of ascites was evacuated intraoperatively. Due to large graft size, a bridging Vicryl knitted mesh was placed in the right lateral part of the chevron incision to avoid compartment syndrome. On postoperative day 4, the level of her liver enzymes was acutely elevated. Liver ultrasound showed normal resistive indices, and she was started on stress dose steroids for suspected acute cellular rejection. Her liver enzymes responded appropriately to the steroids. Her postoperative course was also complicated by hypotension requiring pressors for 48 hours, acute kidney injury, and ileus, all of which resolved by her discharge on postoperative day 10. She is currently five months postoperatively, and her liver enzymes remain within normal limits.


Patient 4 is a 61-year-old female whose cause of the liver disease was primary biliary cirrhosis complicated by ascites not requiring paracentesis and esophageal varices, with a preoperative MELD of 26. Her medical history also included diffuse alveolar lung disorder and fibromyalgia. She received a deceased donor orthotopic liver transplant with a graft volume of 1909 cm3 (as estimated by donor preoperative CT volumetry) using the piggyback technique of veno-venous bypass with a choledocho-choledochostomy and portal vein eversion thrombectomy. Her height was 154.9 cm, and her weight was 56.5 kg, while her donor's height and weight were 157.5 cm and 63.3 kg, respectively, giving a donor-weight-to-recipient-weight ratio of 1.12. Due to concerns of compartment syndrome because of large graft size, a bridging mesh was utilized to achieve a tension-free fascial closure. Her postoperative course was complicated by possible acute rejection evidenced by elevated alkaline phosphatase, which responded to 3 days of stress-dose steroids. She was discharged on postoperative day 8. She is currently 8 months postoperatively and doing well with good graft function.


A 27-year-old female presented with itching in her right eye four days ago. As personal records, she had severe head itching after she had been traveling in southern Italy five days earlier. The patient thought that it might be a head lice infection and was treated with pyrethrins shampoo getting the head itch to disappear. Visual acuity (VA) was 20/20 (Snellen) in both eyes. Slit-lamp examination revealed bilateral blepharitis and some mobile insects attached to the eyelashes (Figure 1 ). Looking carefully, translucent nits and eggs could be seen attached at the base of the eyelashes (Figure 2 ). A mechanically dislodging was carried out removing every insect and egg. A microbiological study was performed with a Phthirus pubis result. The patient was diagnosed of pediculosis ciliaris. A vaseline application twice daily was recommended to asphyxiate any nit or louse that could remain.


A 39-year-old male presented with the complaint of blurred vision, described as haziness, in his left eye of two months’ duration. He acknowledged that he had experienced blurring of vision of both eyes 12 years ago, and that his vision improved and stabilized after using unrecalled topical medications. He reported little or no difficulty with night vision. Aside from occasional tearing, the patient had no other associated ocular symptoms. He had no known comorbid conditions and no systemic complaints. He denied any of his immediate family experiencing similar symptoms. He also denied consanguinity of his parents.
Snellen uncorrected visual acuity in the right eye was 20/20, and the manifest refraction was plano. Snellen uncorrected visual acuity in the left eye was 20/100, which was best corrected to 20/20 with –1.25 D sphere =–0.75 D cylinder x90°. Color vision, gross eye examination findings, and intraocular pressures were within normal limits. Slit lamp examination showed a small and thin posterior subcapsular cataract in the left eye. Dilated fundus examination revealed in both eyes roundish gray patches of chorioretinal atrophy, some coalescing to form a gyrate shape, mostly located in the inferior retinal midperiphery and far periphery (Figure 1 ). No such lesions were seen in the macula. There was also peripapillary atrophy.
On fluorescein angiography, the patches appeared as hypofluorescent areas with faintly hyperfluorescent borders. Choroidal vessels were prominent within the lesions. Spectral-domain optical coherence tomography (OCT) of the macula revealed normal central foveal thickness in both eyes and perifoveal thinning, which was more pronounced in the left eye than in the right. Horizontal scans of the macula showed no abnormalities. Testing of the central 30 degrees of the visual field with the Octopus 300 perimeter (Haag-Streit AG, Köniz, Switzerland) revealed in each eye a superior arcuate scotoma and enlargement of the blind spot (Figure 2 ). There was inferior retinal nerve fiber layer (RNFL) thinning in the right eye on OCT of the optic nerve head and RNFL. Full-field electroretinogram (ERG) responses in light- and dark-adapted conditions showed A and B waveforms with amplitudes and implicit times that were generally within the normal range (Table 1 ). Waveform amplitudes in the left eye were mostly lower than those in the right eye. Multifocal ERG, which is more sensitive than full-field ERG in detecting local retinal dysfunction, was not available in our center and in other centers in the country and thus was not performed.
Quantitative plasma amino acid analysis done after an overnight fast revealed a normal plasma ornithine level at 61 µmol/L (reference interval: 48–195 µmol/L). Levels of the other amino acids, with the exception of taurine, were within normal limits. Plasma taurine concentration was below normal range at 36 µmol/L (reference interval: 54–210 µmol/L). The amino acid analysis was done using ultrahigh performance liquid chromatography – Waters MassTrak amino acid analysis system. Reference intervals were adapted from the Princess Margaret Hospital for Children, Perth, Australia. Testing for OAT activity and OAT genetic testing were unavailable in our center and in nearby institutions and thus were not done. Nerve conduction studies revealed motor potentials within normal limits and normal F-waves but with reduction of sensory potentials of the right median nerve, indicative of mild carpal tunnel syndrome. Monopolar needle studies on the muscles of the right paracervical and paralumbar areas showed no signs of acute denervation.
Based on the history, fundus findings, and results of ancillary tests, our patient was diagnosed as having a gyrate-atrophy-like phenotype. The patient was advised spectacle correction of his error of refraction, increased intake of taurine-rich foods, and once- or twice-yearly follow-up for monitoring of the lesions and possible repeat plasma amino acid analysis. Ophthalmologic examination of his parents and siblings was also recommended.


A previously well, 24-year-old Indo-Trinidadian female first presented two weeks prior to ICU admission with a short history of fever and vomiting. She received symptomatic treatment at her local general practitioner and was subsequently well enough to attend her own wedding three days later. Despite some persistent abdominal pain, she seemed her normal self during the event. The following day, she left for her honeymoon on a neighboring island. There, she experienced daily episodes of vomiting, intermittent fever, a pruritic rash, and occasional diarrhea. She was thus forced to return home three days after her departure. At home, she sought further medical attention. She was noted to be icteric and was admitted to a smaller hospital for investigation.
She had no previous blood transfusions, denied illicit drug use, consumed alcohol only occasionally, and had no tattoos. There was also no history of travel outside of the country prior to initial symptoms. Four years ago, she was hospitalized for dengue fever and pneumonia, which resolved without sequelae. No history was given of any behavioral or neurologic changes that may have indicated Wilson’s disease.
She spent five days being investigated and treated and was then transferred to our tertiary institution for further advanced management of her worsening liver function. Initial tests were negative for leptospirosis, dengue fever, hepatitis A, B, and C, and human immunodeficiency virus (HIV). Her autoimmune screen was also negative (anti-nuclear antibody (1/1000), anti-ds DNA, anti-smooth muscle antibody, and anti-mitochondrial antibody), along with her urinary pregnancy test.
An abdominal ultrasound showed coarse echotexture of the liver, biliary sludge, no gallstones, no gall bladder wall thickening, and a normal common bile duct. The main portal vein measured 13 mm with no thrombosis seen and hepatopetal flow. Moderate ascites with bilateral pleural effusions were also noted. Subsequent MRI revealed a nodular liver with hepatosplenomegaly (Figures -).
On examination, she was grossly icteric, mildly agitated, and had an ascitic abdomen. Her lab investigations at that time were significant for a raised white cell count - WCC (20.8 x 10^9/L), a marked rise in serum creatinine (1.5 mg/dl from 0.9 mg/dl), a transaminitis (aspartate aminotransferase (AST): 961U/L, alanine aminotransferase (ALT): 589U/L), hyperbilirubinemia (total: 41.2 mg/dl, direct: 26 mg/dl, indirect: 15 mg/dl), and her prothrombin time was reported as no coagulation. Her hemoglobin was 6.2 g/dl with no evidence of external bleeding and a negative direct and indirect Coombs test. Other significant investigations were alkaline phosphatase (ALP) of 84 U/L, gamma-glutamyl transferase (GGT) of 157 U/L, and lactate dehydrogenase (LDH) of 600 U/L.
The working diagnosis was an acute liver failure of an undetermined cause with grade I encephalopathy (West Haven). Based on her MRI and ultrasound findings (hepatosplenomegaly, nodular liver, and dilated portal vein), she was subsequently considered to also have a background of chronic liver disease. She was admitted to the ICU for further management of her liver failure. Treatment at that time consisted of intravenous (IV) piperacillin/tazobactam, oral lactulose, and fleet enemas, albumin (12.5 g IV bd), vitamin K, and IV esomeprazole in addition to general supportive ICU care. Hepatitis E serology was also requested, which showed a strongly positive IgM (4.1, Euroimmun, Lübeck, Germany).
The patient spent six days in the ICU. Persistent anuria, despite fluid resuscitation, metabolic acidosis and a rising creatinine (2.1 mg/dl), prompted the early initiation of continuous renal replacement therapy. A 50% dextrose infusion was started to treat persistent hypoglycemia. Her encephalopathy worsened over the first three days moving from grade I to grade III.
On day three of ICU, the patient had worsening oxygenation and agitation that warranted intubation, mechanical ventilation, and sedation using propofol and fentanyl infusions at 100 mg/hr and 100 ug/hr, respectively. Transaminitis and coagulation abnormalities persisted and this, together with her rapid decline and poor prospects for a liver transplant, prompted the use of N-acetyl cysteine as salvage therapy. After a brief improvement in oxygenation following intubation, further decline over the next two days required paralysis and the use of high positive end-expiratory pressure (PEEP) to improve her oxygenation. Pneumonia was suspected because of her rising white cell count (WCC). However, none was confirmed on microbiology.
On the sixth day, the patient became hypotensive and a noradrenaline infusion was started at .8 ug/kg/min. On examination, she had bounding pulses and warm peripheries suggestive of vasodilatory shock. The hypotension persisted and she was noted to have bilateral dilated pupils associated with bradycardia of 40 to 45 beats per minute as seen on a real-time cardiac monitor and a low mean arterial pressure (MAP; <50).
Using point-of-care ultrasound, cardiac contractility was noted to be preserved, with a full inferior vena cava with minimal respiratory variation, and lung sliding was seen bilaterally with B-lines noted over the lung fields. Her optic nerve sheath diameter measured 63 mm, using a high-frequency ultrasound probe (Figure ).
This together with her clinical state likely indicated raised intracranial pressure (ICP). The noradrenaline dose was increased to 1 ug/kg/min and an adrenaline infusion added in an attempt to increase her mean arterial pressure (MAP) to improve her cerebral perfusion pressure in the setting of a raised ICP. Respiratory rate was also increased to attempt to reduce her ICP while awaiting stabilization for CT brain. However, despite escalating inotropic and vasopressor requirements, her blood pressure and heart rate continued to fall, leading to an eventual fatal cardiac arrest shortly after her dilated pupils were noted.


A 54-year-old Thai female with locally advanced breast cancer (T4N2M0) stage III-B and hepatitis B carrier presented with a large right breast lump with skin involvement. She was a housewife, lived in Chonburi Province in the east of Thailand, and had no other medical conditions. The tumor's profile was grade 3 invasive ductal carcinoma, estrogen and progesterone receptors negative, and human epidermal receptor 2 positive. Physical exam showed right breast mass 10 × 10 cm with skin inflammation and right axillary lymph node 9 cm, and others were unremarkable. Computed tomography (CT) scan of the chest and abdomen was done for staging and showed no evidence of distant metastasis ().
She received chemotherapy consisting of Adriamycin (doxorubicin) and cyclophosphamide (AC) as neoadjuvant treatment and lamivudine for hepatitis B infection. Unfortunately, she had two episodes of febrile neutropenia (FN) after the chemotherapy although she received granulocyte-colony stimulating factor (G-CSF) subcutaneously for FN prophylaxis in the second cycle of the chemotherapy. The both episodes were successfully treated with intravenous (IV) ceftazidime together with G-CSF, and blood cultures were negative. She received AC a little bit behind the schedule (normally every 3 week); however, her breast tumor was shrunk after the second cycle.
In the third cycle, she received decreased dose AC plus G-CSF prophylaxis. Nonetheless, on day 10 after the chemotherapy, she presented with fatigue, diarrhea, and fever. Initial physical examination was as follows: blood pressure 88/51 mmHg, pulse rate 124/min, body temperature 39.5°C, respiratory rate 32/min, and O2 saturation 88% on room air. She looked ill, marked icteric sclerae. Cardiovascular exam revealed regular rhythm, tachycardia, and no murmur. Other systems were unremarkable. Sepsis and febrile neutropenia were the most likely diagnosis.
After blood cultures were done, she received IV piperacillin/tazobactam as empirical antibiotic, IV fluid, and G-CSF injection and was admitted to a hospital. Laboratory tests showed CBC: hemoglobin 6.4 g/dL (10.8–14.2), white blood cell count 450 cells/μL (3,700–10,000), absolute neutrophil count 230 cells/μL (1,600–6,900), platelet 26,000 cells/μL (150,000–360,000), Na 118 mmol/L (136–145), K 2.8 mmol/L (3.5–5.1), Cl 87 mmol/L (98–107), HCO3 15 mmol/L (22–29), BUN 48 mg/dL (6–20), Cr 4.2 mg/dL (0.5–1.0), total bilirubin 4.7 mg/dL (0.0–1.2), direct bilirubin 4.6 mg/dL (0.0–0.2), albumin 3.2 g/dL (3.5–5.2), globulin 3.7 g/dL, AST 368 U/L (0–32), ALT 387 U/L (0–33), and ALP 154 U/L (35–104); urine: SpGr 1.025 (1.003–1.030), protein 2+, WBC 2-3 cells/HPF, and bacteria-numerous. On the same day, 8 hours later, she deteriorated and needed to receive orotracheal tube intubation, mechanical ventilator support, and inotropic agent; moreover, the antibiotic was changed to imipenem and metronidazole. The patient was diagnosed with septic shock with multiorgan failure, febrile neutropenia with underlying breast cancer, and hepatitis B infection.
With intensive care, her symptoms were gradually improved. Dopamine and the orotracheal tube were off on day 4 and day 5 after admission, respectively. On day 6, two blood cultures and a urine culture showed positive for Burkholderia pseudomallei susceptible to amoxicillin-clavulanate, ceftazidime, imipenem, and trimetroprim/sulfamethoxazole (TMP/SMX), and stool exam for Clostridium difficile toxin revealed negative. Thus, the antibiotic was de-escalated to ceftazidime 2 gram IV every 8 hour, which was infused until 14 days. Fever disappeared on day 10 after injection of ceftazidime, and on the same day, ultrasound of upper abdomen showed multiple small hypoechoic nodules of the liver and spleen (). A repeated blood culture was negative. On day 20 after admission, CT scan of the chest and whole abdomen was done, showing small lung nodules and multiple small hepatosplenic abscesses, which was compatible with disseminated melioidosis, and breast tumor was decreased in size (). She was discharged on the same day and received TMP/SMX 3 tablets two times a day.
At the 3rd month after treatment with TMP/SMX for melioidosis and changing chemotherapy combined with trastuzumab for breast cancer, she had no symptom from infection, but breast tumor had progressed in size. CT scan was repeated to evaluate diseases and showed the breast tumor increased in size, but markedly decreased in the number of lung, liver, and splenic nodules (). As a consequence, she received TMP/SMX up to 20 weeks, and preoperative concurrent chemoradiation for breast tumor was performed. Finally, mastectomy was done successfully, and the pathological report showed 3.5 cm residual tumor, lymph node positive 11/12 for metastasis, and free surgical margin. CT scan was repeated again 3 months later, showing no evidence of recurrent tumor and infection.


A 27-year-old man with a history of CD and ITP lost to follow-up for two years presented with two to three days of dark, bloody diarrhea, nausea, and nonbloody, nonbilious vomiting. Physical exam was notable for mild periumbilical tenderness; no hepatosplenomegaly was present.
The patient had been diagnosed with Crohn's disease seven years prior to this admission and had known moderate to severe colonic disease. He was diagnosed with ITP during a Crohn's disease flare four years prior to this presentation, was treated with IV methylprednisolone, and discharged on a prednisone taper. The direct antiglobulin test (Coombs) was negative; d-dimer, fibrinogen, C3, and C4 levels were within normal limits. Serologic studies for ANA, ANCA, viral hepatitis panel, and HIV were also negative. He did not undergo a bone marrow biopsy at time of ITP diagnosis.
On admission, laboratory results were notable for platelets of 18,000 per microliter (normal 160,000–360,000 per microliter), ESR of 49 mm/hr (normal 0.00–12 mm/hr), and CRP of 8.04 mg/L (normal 0.00–0.74 mg/L). He was started on methylprednisolone 20 mg IV every 8 hours and given 2 units of platelets, with platelet count improvement to 51,000 per microliter. He subsequently received intravenous immunoglobulin (IVIG) 1 g/kg daily for two days. He continued to experience bloody diarrhea and underwent esophagogastroduodenoscopy and flexible sigmoidoscopy, which revealed significant body and fundus gastropathy as well as moderate to severe proctosigmoiditis (). He had a prior history of thrombocytopenia with adalimumab and infliximab, so he received a 390 mg IV loading dose of ustekinumab for his Crohn's disease flare. Antibody testing for anti-infliximab was negative during this hospitalization. His platelet count briefly responded to combined corticosteroids and IVIG but subsequently declined (). He received rituximab on days 10 and 17 but had persistent thrombocytopenia. On hospital day 24, CRP increased to 5.99 mm/hr (from 0.09 mm/hr on hospital day 15), and symptoms for colitis persisted. Given his transfusion-dependent thrombocytopenia refractory to medical therapy, the patient underwent splenectomy. Due to persistent symptoms of bloody diarrhea and cramping, the patient concurrently underwent total colectomy with end ileostomy.
Following surgery, the patient's platelet count improved without transfusion support to 571,000 per microliter by the date of discharge. Pathologic review of the resected colon demonstrated changes consistent with chronic inflammatory disease, with severe disease activity. Five months following discharge, he has been maintained on ustekinumab and 6-mercaptopurine and has not had a Crohn's disease flare. His platelets remained stable at 170,000 per microliter without additional medical management for his ITP.


A 79-year-old woman presented with a fast-growing, painless thyroid nodule and symptoms of dysphagia and dysphonia for six months. She reported a history of hypertension, no history of radiation to the neck, and no family history of any malignancy. She was admitted to hospital for a tentative thyroid radiofrequency ablation (RFA).
On physical examination, a very large, solid, and irregular thyroid nodule was detected (Figure ). No lymph node was found in the lateral neck. Thyroid function tests (FT4, thyrotropin) and other tests (CBC, liver, and renal function tests) were normal with serum FT4 1.18 ng/dL (normal range [NR] 0.93-2.22); thyrotropin 0.59 microU/mL (NR 0.27-4.2). Her chest X-ray showed no abnormal findings. The thyroid ultrasonography showed a 45 × 32 mm mixed echogenicity mass which occupied nearly the entire thyroid gland, with no calcification, no vascularity, and no lymph node involvement. Fine Needle Aspiration (FNA) of the thyroid mass yielded hemorrhagic aspirate (Figure A,B) with diagnosis of a large benign thyroid nodule. The patient refused a core needle biopsy. A total thyroidectomy instead of RFA was performed because of compressive symptoms and high risk of malignancy (Figure ).
Histopathologic examination (HE stain) revealed the tumor cells were spindle-shaped, round, or ovoid, and arranged in a whirlpool fashion with invasive growth, with high mitotic activity and areas of necrosis. It is consistent with a moderately differentiated thyroid sarcoma (Figure A,B). Immunohistochemical staining showed diffuse marked reactivity for vimentin and focal reactivity for CD68 and Ki-67 (positive 20%). No reactivity for AE1/3, thyroglobulin, S100, or smooth muscle actin was detected (Figure A-H). Histopathologic and immunohistochemical staining features were suggestive of UPS of the thyroid. No postoperative chemo- or radiotherapy was given because her daughter refused. The whole-body scan detected no metastasis to distant organs and lymph nodes. After 6 months of follow-up, local recurrence was detected and no further treatment was undertaken because of her poor performance status.


An 80-year-old Caucasian man was observed at our clinical center in January 2018, for unintentional weight loss of 5 kg, itching without any skin lesion [,], and stomachache. His blood cell count showed lymphocytosis. No noteworthy pathologies were reported in the anamnesis. He was still active in cultivating his soil, but recently he was less active. [18F] FDG-PET performed with co-registered CT in February 2017 () showed a significant area of increased metabolic activity in abdominal cavity loaded with solid tissue (23 × 14 × 4 cm) which occupies the whole of the mesentery and the retroperitoneal space by displacing the pancreas anteriorly and the intestinal loops laterally (SUV 4.85) and splenomegaly []. The bone marrow examination showed a 30% infiltration of phenotypically clonal lymphocytes k+, CD20 +, CD5 + CD23 + CD10−, with del (11q) in FISH test with evidence of an un-mutated immunoglobulin heavy-chain variable region (IGHV) gene. The patient was affected by CLL stage II according to Rai with symptomatic active bulky disease and he needed treatment. The patient was also tested for serum markers of HIV, HBV, HCV, and CMV and was found positive only for anti-HBc, as a sign of previous HBV infection [,,,,]. Lamivudine prophylaxis at the dose of 100 mg/day was administered 4 weeks before the start of chemotherapy, in order to prevent HBV reactivation. In March 2017, chlorambucil therapy was administered together with oral opioids for pain control []. In the following 2 months, the patient lost other 5 kg, because of the difficulty in feeding, and suffered of severe fatigue, which forced him to bed all day long; at this time, his body weight was 40 kg and his BMI 16.4 kg/m2. On 2 May 2018, he performed all blood chemistry tests that showed the following anomalies: Erythrocyte sedimentation rate (ESR) 71 mm; C-reactive protein (CRP) 54 mg/L; fibrinogen 637 ng/dL, lactate dehydrogenase (LDH) 498 U/L; albumin 2.6 g/dL; triglyceride, cholesterol and glicemy levels were at the lower limits of normal; a moderate anemia with hemoglobin 9.5 g/dL, a lymphocytosis (20,000/microliter). At cardiology consultation and echocardiogram he showed good functionality. Chemotherapy was changed and ibrutinib 420 mg/day was introduced in June 2017. In addition, a rehabilitation physio kinesitherapy program and parenteral nutrition were started associated with intravenous (i.v.) albumin every day and, erythropoiesis-stimulating agents (ESAs) subcutaneous (s.c.) every week. Peripheral parenteral nutrition included the intake of glucose, lipids, branched chain amino acids, B vitamins, and folate. The muscle exercise program was based on an attempt to bring the patient back to physical capacity 4 months before bedtime. After one month, the general conditions began to improve. He started walking with support and gradually he was able to walk and eat independently. His performance status improved progressively, and he was recommended to walk for 30-minutes a day at mild intensity in July 2018 []. His CT showed an important decrease of all lymphoadenomegaly, and his blood count become normal, with ESR 40 mm; CRP 20 mg/L. In August 2018, a 5-days-a-week training program favoring some hobbies of the patient was started: A physical activity for at least 20 min consisting of a fast walk in the open air three times a week and a moderate physical activity in the remaining two days of at least 20 consecutive minutes (cycling at a regular pace, carrying light weights) [,,]. The exercise program included also aerobic, upper and lower limb resistance training, core stability, and stretches [,,]. Appetite increased more and more along with his ability to feed and digest all foods. The aerobic training was first increased to 30–40 min walking or cycling stationary at moderate and progressive intensity and after to 60 min. The physical condition further improved and remained excellent throughout the follow-up period. All blood tests progressively improved; in October 2018, they showed only a slight increase in pancreatic amylase (77 U/L) and lipase (83 U/L). In December 2018 his clinical condition was quite normal, a CT showed a great decrease of all lymphoadenomegaly, FISH test did not show del(11q) and all blood tests are normal. Now, the patient is in good clinical condition and thanks to the physical training program, he has achieved an enviable physical condition. He continued to cultivate his land, and is still being treated with ibrutinib; his last CT performed in October 2019 showed good partial response ().


A 49-year-old Indian male with no known co-morbidities, presented with right iliac fossa pain for one day. The pain started gradually and progressively increased in severity. It was non-migratory and non-radiating without any aggravating factors.
The patient has a history of constipation for 5-6 years for which he was taking laxatives intermittently. There was no history of weight loss or bleeding per rectum, similar prior episodes, night sweats nor any contact with tuberculosis patients. His family history was non-contributory. His past medical history was significant for left inguinal hernia repair.
At presentation, his vitals were within normal range. His abdomen was non-tender, non-distended and soft to touch. He had mild fullness in right lower quadrant. However, no definite mass was palpable. Digital rectal exam was normal.
Investigations
His labs revealed WBC of 11,400 per microliters with neutrophil of 82%, hemoglobin was 15 gm/dl, platelets 273,000 per microliters, rest of the labs were within normal limits. Quantiferon test for tuberculosis was negative and Echinococcus antibody titer was normal. Tumor markers carcinoembryonic antigen (CEA) and Alfa fetoprotein (AFP) were done and were normal.
The patient underwent CT scan, which revealed a 7 x 6 cm cystic lesion with incomplete peripheral calcification in pelvis more to right side (Figure ). The cyst is lying anterior and separate from the right common iliac vessels, urinary bladder, rectum, and small bowel loops (Figure -). The lesion appears unenhanced on CT scan (Figure , ).
Differential diagnosis
The differentials of this lesion in the right lower quadrant were benign and malignant cystic lesions. We considered it a benign cyst, based on the history of no weight loss, normal tumor markers and radiological findings of a well-defined cyst on CT scan which does not appear to involve or invade any surrounding structures. One of the possibilities was an echinococcal cyst but it was ruled out by the absence of daughter cysts, non-enhancing wall or floating membrane, and normal echinococcal titers. The other differentials were neoplastic cystic lesions but it was less likely as the cystic mass had a well-defined wall without any features of invasion to surrounding structures.
It was really challenging to figure out the diagnosis based on history and imaging. That’s why we opted for laparoscopic excision of the mass to establish a definitive diagnosis.
Treatment
The patient underwent laparoscopic cyst excision (Video ).
The location of the cyst was clearly the retroperitoneum as there was a complete peritoneal covering over the lesion. The cyst was compressing the right ureter and in proximity with the right internal iliac vessels. It was dissected carefully from the right ureter and right iliac vessels using Harmonic scalpel and safe laparoscopic surgical principles. Identification of the ureter by gently stimulating peristalsis is clearly demonstrated in the video. Inadvertent opening of the cyst occurred during dissection of the posteromedial part of the cyst due to a very thin false capsule. The fluid content of the cyst was aspirated and tested for amylase and tumor markers (AFP and CEA) but was not detected, confirming the diagnosis of a non-pancreatic pseudocyst. The mass was excised completely and removed using a specimen retrieval bag after copious irrigation and ensuring good hemostasis.
Outcome & follow-up
Postoperatively, the patient recovered well with no complications. Oral fluids were started on postoperative day 1 which was progressed to full diet on the same day. On postoperative day 2, the patient was pain-free, had passed stool, had adequate urine output and was discharged home. After two weeks, he was followed up in the clinic with a normal abdominal examination and well-healing wounds.
His histopathology showed a benign fibrous cyst wall containing fibrin with cholesterol clefts. There was no epithelial or endothelial lining and it was negative for granulomas or malignancy (Figure and Figure ).


A 68-year-old man was admitted to our emergency department with left upper quadrant abdominal pain and hematemesis of 4 h duration. He had undergone Whipple’s procedure (duct-to-mucosa pancreaticojejunostomy and side-to-side gastrojejunostomy) with B-II reconstruction for duodenal papillary adenocarcinoma 5 years earlier. His vital signs were stable with a blood pressure of 163/93 mmHg, temperature of 37.0 °C; and heart and respiratory rates of 86 per/min and 20 per/min, respectively. Physical assessment showed mild tenderness in the left upper quadrant, only. A complete blood count showed white cell and platelet counts of 11.69 × 103/L and 196 × 103/L, respectively, and a hemoglobin level of 13.5 g/L. Abdominal computed tomography (CT) suggested a retrograde intussusception of the intestines into the stomach with dilatation of the remnant stomach (Figs. and ). The patient immediately underwent exploratory laparotomy, which revealed a 20-cm retrograde efferent limb at the remnant stomach that had travelled through the previous gastrojejunostomy (Fig. ). There was no evidence of malignancy. We manually reduced the intussuscepted loop using gentle traction, and the viability of the intestinal loop was preserved. The patient had an uneventful postoperative recovery.


A 54-year-old woman was admitted to hospital with recurrent stoma malfunction and prolapse requiring surgical revision, on a background of prior total colectomy and ileostomy formation for chronic pseudo-obstruction, and short gut syndrome. A long-term Hickman line for total parenteral nutrition (TPN) was last replaced 10 months prior to admission, with a history of previous Hickman line infections secondary to enteric and skin flora.
The patient had a fever on day +1 of admission and a set of blood cultures was collected from the patient's Hickman line. Budding yeasts were identified on microscopy of the anaerobic BacT/ALERT® (bioMérieux) blood culture bottle after 23 hours incubation. Empiric anidulafungin (200mg IV loading dose, followed by 100mg IV daily) was commenced. The subsequent isolate was identified on MALDI-TOF (Bruker) as C. parapsilosis (score = 2.1) after sub-culture. Yeasts were also identified in the aerobic bottle after 28.5 hours incubation. After 2 days incubation of sub-culture of the aerobic bottle, 2 morphologic colony types were noted on chocolate agar, subsequently identified on MALDI-TOF as C. parapsilosis (score 2.26) and L. elongisporus (score 2.04). L. elongisporus colonies were observed to have lighter pigmentation than C. parapsilosis. Sub-culture of the aerobic blood culture bottle revealed pink and turquoise colonies after overnight incubation on Chromogenic Candida Agar (ThermoFisher Scientific) (). MALDI-TOF of the green/blue colonies identified these as L. elongisporus (score 2.15). Antifungal susceptibility testing of both isolates was performed using the Sensititre™ YeastOne™ YO10 plate (ThermoFisher Scientific) ().
C. parapsilosis was also identified in the aerobic bottle of a second blood culture collected on day +2 of admission after 24 hours incubation. The Hickman line was removed on day +6 of admission, and culture of the tip grew >100 colony-forming units of C. parapsilosis (identified by MALDI-TOF) after 24 hours incubation. A PICC was inserted for ongoing TPN.
Anidulafungin was continued for 14 days following Hickman line removal (ceased on day +20). There was no growth in blood cultures collected day +10 and subsequently, and the patient had clinical improvement with resolution of fever following Hickman removal.


A 24-year-old woman received bilateral lung transplant for COPA syndrome. Manifestations of her COPA syndrome included arthritis, biopsy-confirmed immune-mediated kidney disease, antineutrophil cytoplasmic antibody (ANCA) positivity, interstitial lung disease, diffuse alveolar hemorrhage, and progressive respiratory failure leading to mechanical ventilator support. Chest computed tomography demonstrated extensive cystic changes in the lungs, diffuse coarse reticular interstitial thickening, and ground glass opacities (). Pulmonary function tests showed a restrictive pattern with decreased DLCO. She received rituximab and plasmapheresis in the perioperative period and induction immunosuppression with thymoglobulin. Histologic sections of the explanted lungs showed a relatively uniform fibrosing interstitial pneumonia (nonusual interstitial pneumonia pattern) with honeycomb change and emphysema (). Organizing lung injury and patchy alveolar hemorrhage with hemosiderin-laden macrophages were noted, but capillaritis was not seen.
The postoperative course was complicated by neutropenic fever, cytomegalovirus viremia, bilateral lymphocytic pleural effusions, and persisting leukopenia. A year after transplant, she had no respiratory symptoms, but testing confirmed severe gastroesophageal reflux disease (GERD). She was successfully treated with laparoscopic Nissen fundoplication. At 15 months posttransplant, her pulmonary function is stable, with no episodes of acute cellular- or antibody-mediated rejection and no evidence of disease recurrence. She is maintained on an immunosuppression regimen that includes prednisone, tacrolimus, and intravenous immunoglobulin.


A 56-year-old lady was presented to the emergency clinic with acute onset of headache since 3 days followed by sudden progressive loss of vision in both eyes. No associated history of redness, photophobia, and floaters was noted. There was no evidence of any associated systemic illness. There was no uveitis-related systemic history in the form of joint pains, exposure to tuberculosis, exposure to pets, or fever with rashes. No previous history of any ocular surgery or trauma was noted. On examination, her best corrected visual acuity was counting fingers closely in both eyes; her intra ocular pressures were within normal limits. There was no relative afferent pupillary defect. Slit lamp examination showed anterior chamber reaction with cells occasionally in both eyes, no keratic precipitates were seen, lens was clear, and anterior vitreous face showed cells 1+ retrolentally. Fundus showed bilateral exudative retinal detachment with pockets of subretinal fluid in the posterior pole mainly superiorly and inferotemporally, with dot and blot hemorrhages seen around fovea, temporal to macula, and superior to disc. No evidence of vessel sheathing was seen (Fig. a, b).
Optical coherence tomography (OCT) macula showed areas of serous detachment (Fig. a) and RPE undulations with pockets of subretinal fluid (Fig. b). Fundus fluorescein angiography showed early blocked fluorescence with multiple pin-point leakages in the middle phases with placoid areas of hyperfluoresence in late phases (Fig. a, b). Clinical findings, OCT, and fluorescein angiography features are all typical of acute uveitic phase of VKH disease except for the presence of retinal hemorrhages. Differentials that were considered in our index case included leukemia, lymphoma, and syphilis. Magnetic resonance imaging (MRI) brain showed scattered foci of T2/FLAIR hyper intensities in the bilateral centrum semiovale, bilateral corona radiata, and subcortical and deep white matter of both frontal and parietal lobes. Full blood count was normal, and peripheral blood smear showed no significant morphologic abnormalities suggestive of hematopoietic tumor. Serological screen for syphilis, tuberculosis, and hepatitis viruses were negative.
In view of the typical angiographic features of acute uveitic stage of VKH syndrome, systemic corticosteroids were started in the form of intravenous methylprednisolone 1 g/day for 3 days followed by oral prednisolone 1 mg/kg along with topical steroids. Patient responded well to the treatment with resolution of the exudative retinal detachments as well as the hemorrhages (Fig. ). On subsequent follow-up, her best corrected visual acuity improved to 6/9 in both eyes. She was on close follow-up.
We present this case the presence of retinal hemorrhages in the setting of acute VKH that is quite unusual in the absence of associated systemic diseases.


The patient was a 65-year-old woman with no relevant family history. She was born without any perinatal anomalies, although short stature was identified at around 3 years of age, but she never received effective treatments. Withdrawal bleeding occurred from around the age of 16 years after hormone-replacement therapy was started to address the absence of secondary sexual characteristics and primary amenorrhea. However, treatment was self-interrupted 1 year later, and she remained amenorrheic thereafter. The patient noticed hair loss in her late 30s. She visited a medical practitioner due to gout at 45 years of age and was subsequently treated for hyperuricemia, dyslipidemia, and hypertension at the clinic. In addition, the patient was diagnosed with hypothyroidism at 50 years of age, and thyroid hormone-replacement therapy was started (levothyroxine sodium hydrate, 50 μg/day). In 2013, at 58 years of age, the patient was referred to our department for endocrinological examination of short stature. The results of this examination showed impaired secretion of TSH on the thyrotropin-releasing hormone (TRH) stimulation test, impaired secretion of LH/FSH on the luteinizing hormone-releasing hormone (LH-RH) stimulation test, impaired secretion of GH on both arginine and GH-releasing peptide-2 (GHRP-2) stimulation tests (Fig. ), and an empty sella turcica with atrophy of the anterior pituitary gland on magnetic resonance imaging (MRI) (Fig. ), but secretion of ACTH was retained on the corticotropin-releasing hormone (CRH) stimulation test (Fig. a). For this reason, GH-replacement therapy (0.075 mg/day of somatropin; genetic recombinant) was started. In addition, chronic kidney disease of unknown cause was identified (estimated glomerular filtration rate [eGFR], 31.9 mL/min/1.73 m2) (Table ). At 63 years of age, hypoxia and hypercapnia were identified (partial pressure of carbon dioxide [pCO2], 59.6 mmHg; partial pressure of oxygen [pO2], 48.7 mmHg), and respiratory function testing confirmed a markedly restricted ventilation disorder, and both vital capacity (VC) and forced vital capacity (FVC) were significantly reduced (VC, 0.54 L; percentage predicted VC (%VC), 26.9%; FVC, 0.50 L), but percentage predicted forced expiratory volume in 1 s (%FEV1.0) was within normal limits (80.0%). Because the patient did not wish to receive home oxygen therapy (HOT) or respiratory rehabilitation, she was followed-up. However, HOT (0.5 L/min by nasal cannula) was started about 6 months later as her exertional dyspnea gradually worsened, and renal function also slowly decreased (eGFR, 25.0 mL/min/1.73 m2). Since there was no bronchiectasis or emphysema, pulmonary embolism and shunt disease were ruled out by the lung perfusion scan (technetium-99 m-labeled macro-aggregated albumin scintigraphy), and since there was weakness of proximal muscle without abnormal neurological findings, the patient was diagnosed with endocrine myopathy by both a respiratory physician and a neurologist. Furthermore, a nephrologist noted that there was no glomerulonephritis or hereditary kidney disease, and that there was unexplained renal atrophy. Around this time, frequent episodes of hypoglycemia occurred, and a low basal level of serum cortisol (6.38 μg/dL), a relatively low basal level of plasma ACTH (34.2 pg/dL), and a low 24-h urinary cortisol level (5.5 μg/day) were confirmed (Table ). Delayed overreaction of plasma ACTH and failure of serum cortisol on the CRH stimulation test were confirmed (Fig. a, b), and a failure of plasma ACTH on the insulin tolerance test was confirmed (Fig. c). Furthermore, the cortisol response on the rapid ACTH stimulation test was slightly delayed and attenuated (Fig. d), and the prolonged ACTH stimulation test showed a sufficient increase in urinary free-cortisol levels (Fig. e). The patient was therefore diagnosed with hypothalamic secondary hypoadrenocorticism, and replacement therapy was started (prednisolone at 2.5 mg/day). At 65 years of age, the patient developed CO2 narcosis (pCO2, 80.1 mmHg, pO2, 76.0 mmHg) during bronchopneumonia treatment, and ventilatory function was: VC, 0.48 L; %VC, 27.1%; and FVC, 0.49 L. Noninvasive positive-pressure ventilation (NPPV) (2.0 L/min by mask) was started and continued at home.
The patient was 129.4 cm tall and weighed 21.8 kg (body mass index, 13.2 kg/m2). Blood pressure was 110/57 mmHg, and heart rate was regular at 93 beats/min. She showed no mental retardation, and no pigmentation on the skin and oral mucosa. Her hair was thin, and no underarm or pubic hair was present. The Turner classification for the breasts was stage 1. Mild scoliosis was evident. Cardiopulmonary examination showed normal results, and no abnormal abdominal findings were identified.