A 17-year-old male patient was admitted to the hospital for “recurrent episodes of
right-side twitching for more than 6 years that was aggravated over 2 days”. More than 6
years prior, right facial convulsions with no obvious cause were noted. The main
manifestations were right-side twitching, salivation, and right-side face and right hand
numbness. He was clearly conscious when twitching but could not perceive verbal information,
and each instance lasted approximately 1 minute. After cranial magnetic resonance imaging
(MRI) (),
electroencephalography (EEG), and lumbar puncture in our hospital, the patient was diagnosed
with “viral encephalitis” and discharged after treatment with antiviral and antiepileptic
drugs. The patient used carbamazepine (100 mg twice/day orally) to control his epilepsy when
he was discharged from the hospital, but his symptoms were poorly controlled. Five years
prior, at Xinqiao Hospital affiliated with Chongqing Third Military Medical University, the
patient underwent epilepsy focus resection. A postoperative pathological biopsy of the brain
tissue indicated focal hemorrhage with softening and perivascular lymphocyte sheath
formation (because of the length of time and since the operation was performed in another
province, the biopsy image results could not be found). When the patient was discharged from
the hospital, he continued to use oral sodium valproate (VPA, 500 mg twice/day),
oxcarbazepine (450 mg twice/day), clonazepam (CZP, 1 mg twice/day), and levetiracetam (500
mg twice/day) to control his epilepsy. His convulsions were controlled for the first 3
months after discharge, but the effect was not satisfactory after 3 months.
Two days prior to his latest admission, because of a cold, the patient’s convulsions
appeared again. In our hospital, while in the intensive care unit, he had no seizures after
intravenous infusion of midazolam and propofol. After consultation with a neurologist,
immunoglobulin was added (12,500 mg/day for 5 days). At that time, detection of serum and
cerebrospinal fluid (CSF)-related antibodies and other CSF-related tests were performed. The
results showed that the patient was positive for serum anti-Hu antibodies. No abnormalities
were detected in other related CSF examinations. Cranial MRI suggested abnormalities (). The EEG results showed
background activity dominated by slow waves, paroxysmal delta waves in the left posterior
area, and epileptiform discharges in the left central parietal-occipital area. Chest
computed tomography (CT) and abdominal CT showed no abnormalities. After transfer to our
department, the patient was treated with daily administration of methylprednisolone sodium
succinate (1000 mg), which was slowly reduced after 5 days, followed by an intravenous drip
of cyclophosphamide (1000 mg) and subsequent administration of immunoglobulin (17,500 mg/day
for 5 days) and the oral antiepileptic drugs lamotrigine (75 mg twice/day), VPA (500 mg
twice/day), CZP (2 mg every night), and topiramate (75 mg twice/day). After this treatment,
the patient had no seizures.
A physical examination showed the following: temperature, 37.1°C; pulse, 130 beats/minute,
respiration 20 breaths/minute, blood pressure, 135/86 mmHg, intermittent convulsions of the
right upper limb and face, and thick double lung auscultation. Other parameters exhibited no
abnormalities. A neurologic examination revealed the following: the patient was conscious,
could not speak, and had right limb muscle strength of grade 4. Other neurological tests
showed no abnormalities. The patient participating in the study and their next-of-kin
provided verbal informed consent for the study.


A 17-year-old male presented to our neurosurgery clinic with progressive vision loss. He had initially been evaluated by a pediatric endocrinologist for delayed puberty as suggested by a high-pitched voice and feminine features. His hormonal work-up revealed panhypopituitarism. Preoperative Humphrey visual field testing demonstrated bitemporal visual field loss.
Further, magnetic resonance imaging (MRI) evaluation identified a 2.6 cm mass in the suprasellar region, noted to be hyperintense on T1 and T2 sequences with a nonenhancing, T1 hypointense nodule []. There was mass effect on the optic chiasm without extension into the cavernous sinus. The computed tomography scan did not show any calcifications [].
Differential diagnosis included proteinaceous CP, cystic degeneration of a pituitary macroadenoma, and less likely RCC.
Given the vision changes, the patient and family consented to resection of the lesion through a transsphenoidal endoscopic endonasal approach. Since the presumptive diagnosis was that of a CP, we discussed with the patient that there was a distinct possibility that the pituitary stalk would have to be sacrificed to obtain gross total resection of the lesion.
The sella was accessed through the standard transsphenoidal approach with stereotactic navigation. After dural opening, the tumor was found to be firm and with a thick capsule. Sharp dissection and radiofrequency ablation were required to debulk the tumor. The tumor center yielded classic motor oil-like fluid as well as large cholesterol granulations that were histopathologically evaluated. There were dense adhesions of the tumor capsule to bilateral optic nerves and chiasm [].
The capsule was sharply dissected away from the neural structures. We could now clearly visualize that the tumor capsule emanated from and had infiltrated the pituitary stalk. To achieve gross total resection, the stalk was truncated and the lesion was removed. Closure was performed using fat graft as inlay, followed by fascia lata wedged into the sellar opening using a MEDPOR (Stryker) graft, creating a gasket seal. This skull base reconstruction was then layered with a pedicled nasoseptal flap. The patient tolerated the procedure well.
Postoperative Humphrey visual field testing demonstrated visual improvement after resection of the lesion and decompression of the chiasm. In addition to his preoperative hormone replacement regimen, he continued to require desmopressin acetate tablets at his 1-year postoperative follow-up. His postoperative 3-month MRI shows gross total resection of the lesion with decompression of the optic chiasm. The residual fat graft as well as the MEDPOR (Stryker) skull base reconstruction can also be visualized [].
Pathology showed a dense fibrous walled cyst with a focal residual internal epithelial lining consisting of squamous cells, whose membrane stained positive on beta-catenin stain [] and occasional ciliated epithelial cells which stained positive for cytokeratin AE1 and AE3 [].
There were marked degenerative changes with lymphocytic and histiocytic inflammation, hemosiderin deposition, cholesterol clefts, and multinucleated giant cells. The histiocytes stained positive for CD68 and many contained a foamy cytoplasm that highlighted on periodic acid– Schiff diastase stain, which was consistent with them being muciphages. These features in summary were consistent with a RCC with marked XGC and squamous metaplasia.


Patient 1 is a >90-yr-old male with a history of smoking (20 pack years), significant sun exposure, and prior keratinocytic carcinomas. He presented with progressively worsening left-sided facial swelling and intermittent pain that was unresponsive to antibiotics. A computed tomography (CT) scan of the neck showed a left parotid superficial mass, and fine needle aspirate (FNA) revealed keratinizing SCC, thought to be metastasis from his prior skin cancer. Upon left parotidectomy and neck dissection, a specimen from the parotid was procured for this study. This specimen was assessed in pathology to be moderately differentiated SCC. Subsequent lymph node dissection identified one of six parotid nodes and zero of 15 neck nodes positive for disease. The patient passed away 45 months after initial diagnosis and 43 months after surgical removal and collection for our study.


Patient 2 is a 61-yr-old male with a family history of leukemia and breast, prostate, and bladder cancer and a prior right parietal scalp SCC (T2N0M0 (II) G2) that was treated with Mohs two-stage excision followed by 60 Gy of adjuvant radiation. Twenty-four months after his scalp SCC diagnosis and 12 months post-Mohs, the patient was seen at the ear, nose, and throat (ENT) clinic for a progressively enlarging right posterior neck mass. FNA demonstrated p16 negative metastatic cSCC. Subsequent neck dissection confirmed five of 25 positive lymph nodes from which a specimen was procured for our study. The patient presented 3 months postexcision with recurrent, right neck cSCC. Positron emission tomography (PET)/CT revealed multiple regional nodules with pulmonary and lymph node lesions compatible with metastatic disease. The patient then underwent 66 Gy radiation therapy and systemic treatment with cetuximab; however, he was unable to tolerate the chemotherapy. The patient passed away ∼22 months after resection of his neck cSCC.


Patient 3 is a 68-yr-old male with a history of smoking (50 pack years) and alcohol use. He initially presented with a 7-month history of poorly differentiated p16 positive cSCC of the left ear and left mastoid process. The patient underwent surgical resection, during which a tumor specimen from the ear lesion was procured for our study. Pathology findings of left ear specimen showed invasive cSCC, T2N3M0. Additionally, a floor of mouth specimen showed invasive SCC, T1N0M0. The patient declined adjuvant chemotherapy and radiation and elected surveillance. At 4 months post–initial surgery, the patient developed lung metastases, and biopsy of a left hilar node confirmed metastatic, p16-positive SCC. The patient was started on nivolumab but was taken off therapy because of development of bullous pemphigoid. Two years post–primary resection and 1 year after discontinuing treatment, the patient developed a superficially invasive, moderately differentiated SCC on the right floor of the mouth. The patient was last seen at the time of floor of mouth lesion removal, 34 months after the initial diagnosis of left ear SCC.


The patient is a 40-yr-old woman who initially presented to the emergency department with a partial large bowel obstruction. Colonoscopy revealed an obstructing mass in the transverse colon with biopsy-proven adenocarcinoma. She underwent partial colectomy, and her pathology showed a well to moderately differentiated invasive adenocarcinoma (pT4a N1a–AJCC eighth edition) with proficient mismatch repair status. She was treated with two cycles of capecitabine and oxaliplatin followed by eight cycles of leucovorin, fluorouracil, and oxaliplatin (switched because of poor tolerance), but CEA levels rose 7 mo later and imaging showed a new liver nodule. Then, she underwent partial hepatectomy for a 2-cm lesion consistent with metastatic adenocarcinoma by histopathology. DNA analysis of the liver lesion by a 324-gene next-generation sequencing (NGS) assay revealed BRAFV600E, TP53C176F, PIK3CAC420R, PTEN loss, and a SMAD4 exons 2-3 duplication (). CEA levels continued to rise over the next 2 mo and imaging revealed new peritoneal, abdominal wall, portacaval lymph node, and ovarian metastases. Patient transferred care to Stanford and was started on irinotecan and cetuximab initially in early 2018 with vemurafenib added several weeks later given the BRAF mutation detected in the liver specimen. The patient had rapid clinical improvement with notable shrinkage in palpable abdominal wall nodules and improvement in pain. Surveillance imaging showed a partial response until 6 mo later when enlarging abdominal wall masses were identified. Computed tomography (CT)-guided biopsy of one of the masses confirmed metastasis, and a new BRAF exon 2–10 deletion and CUL4A amplification was detected by DNA analysis using the same 324-gene NGS assay (). Irinotecan, cetuximab, and vemurafenib treatment was continued while the patient received palliative radiation therapy to the abdominal wall. Following recommendation from the molecular tumor board, cobimetinib, a MEK inhibitor, was added to her treatment 2 mo later through compassionate use. However, given disease progression, she was transitioned to hospice care soon after.


Five patients with brain tumors harboring the BRAF aberration () were prospectively enrolled in a phase I trial of the BRAF inhibitor vemurafenib in combination with mTOR inhibitor everolimus (NCT01596140).
A 10-yr-old boy with World Health Organization (WHO) grade II pleomorphic xanthoastrocytoma (PXA) of the left inferior parietal lobe previously treated by two surgical resections and 50.4 Gy equivalent focal proton therapy since age 4 was found to have a BRAF alteration (with no other co-occurring alterations) and subsequently treated on trial with 480 mg of vemurafenib twice a day and 2.5 mg of everolimus oral daily. The tumor decreased by 32% after two cycles (). He had a grade 3 skin rash that required 10 d cessation of medications but improved with supportive measures, and the patient remains in partial remission now in cycle 41 with no neurological deficits, excellent performance score, and good quality of life.
A 12-yr-old girl with a right optic pathway glioma (without neurofibromatosis type 1) had disease progression after multiple chemotherapy regimens (carboplatin and vincristine, vinblastine monotherapy, vinblastine, and carboplatin) since age 1 yr old. BRAF was confirmed with no other co-occurring alterations and she therefore chose to go on trial with 480 mg of vemurafenib twice a day and 2.5 mg of everolimus daily. The tumor decreased by 36% after two cycles. After five cycles, she was taken off the study as a result of noncompliance with monitoring visits.
A 37-yr-old man with a right parietal WHO grade III anaplastic astrocytoma and neurofibromatosis type 1 underwent surgical resection and 59.4 Gy focal radiation therapy (RT) with concurrent temozolomide, followed by adjuvant temozolomide. After eight cycles, an MRI showed progressive disease. Molecular profiling was obtained and revealed a BRAF alteration as well as mutations in PTPN11, EPHB4, and LRP1B (). Temozolomide was switched to 150 mg of dabrafenib twice a day. One month later the patient discontinued dabrafenib as a result of disease progression and enrolled on trial and received 720 mg of vemurafenib twice a day and 5 mg of everolimus daily. After cycle 6, the tumor size was reduced by 13.7%. His disease remained stable for 12 mo on therapy.
A 22-yr-old man was initially diagnosed with a right temporal WHO grade III anaplastic astrocytoma. He underwent a subtotal resection and focal RT with concurrent temozolomide, followed by adjuvant temozolomide. Local progression with WHO grade IV glioblastoma was found soon after; he underwent subtotal resection followed by a combination of temozolomide and veliparib, a poly(ADP-ribose) polymerase inhibitor. He was additionally treated with carmustine and 13-cis-retinoic acids. BRAF mutation was identified on molecular profiling with no other co-occurring alterations, and he was subsequently treated on trial with vemurafenib twice a day, 960 mg initially, reduced to 720 mg because of severe otalgia and erythematous rash. He maintained stable for 13 mo on this regimen prior to disease progression, at which time he was treated with 720 mg of vemurafenib twice a day and 5 mg of everolimus daily for 4 mo until experiencing disease progression.
A 13-yr-old boy was initially diagnosed with a right thalamic WHO grade II diffuse astrocytoma and treated with subtotal resection and 54 Gy focal RT. Local recurrence with higher-grade glioblastoma developed 2 yr later and was treated by subtotal resection, 45 Gy re-RT with concurrent temozolomide, followed by adjuvant temozolomide. A BRAF mutation with no co-occurring alterations was confirmed on molecular profiling, and the patient was treated on a different trial with vemurafenib monotherapy with stable disease for 9 mo. The patient then went on trial with a cyclin-dependent kinase 4/6 inhibitor and a combination of temozolomide and poly(ADP-ribose) polymerase inhibitor, but quickly progressed on this therapy. He subsequently elected to go on trial with 720 mg of vemurafenib twice a day and 5 mg of everolimus daily. He remained on trial for 3 mo, initially with symptom improvement in his visual disturbance, weakness, and expressive aphasia, but thereafter came off study for disease progression and unfortunately passed away at age 16.


A 39-year-old lady presented to our facility with a 20-year history of the right arm swelling which only progressively increased in size, to involve the entire arm, in the past 3 months (). There was a non-progressive swelling in the opposite distal arm as well. It was painless and there were no associated skin lesions. There was a history of open reduction and internal fixation of the right arm, following a swelling and subsequent distal humerus fracture.
Radiological studies showed extensive lucent, ill-identified, “ground-glass” appearing lesions involving the entire right humerus ( and ). There were incidental findings of similar lesions in both scapulae, right ulna, and left second metacarpal. Positron emission tomography scan showed avid lytic, expansile lesions involving the entire length of the right humerus, bilateral scapula, right ulna, and left second metacarpal. There were also lung nodules in the left upper lobe (≈8mm) and lower lobe (≈5mm) as well as in the right middle lobe (≈7mm).
Her alkaline phosphatase was 573. The parathyroid hormone was normal.
Histopathological examination of the biopsy samples from the bones showed woven bone fragments exhibiting calcification with focal cellular fibroblastic proliferation, including occasional osteoclast-like giant cells. On immunohistochemistry, the cells were negative for beta-catenin and p53, KI67 labeling was<1%. These features were concluded to be consistent with a fibrous-osseous lesion ().
CT-guided biopsy of the lung lesions showed features compatible with atypical adenomatous hyperplasia of the lungs with no evidence of malignancy.
After clinical, radiological, and histopathologic review at our joint clinic meeting, a diagnosis of polyostotic FD was made and the patient was worked up for a forequarter amputation ( and ).
The patient, after a follow-up of 2 years, is asymptomatic for her other lesions ().


A 12-year-old female child presented with gradual enlargement of the right breast for the past 2 months. There was no history of breast cancer in the family, and the menarche had not been attained yet. On examination, the breasts were asymmetrically enlarged, and there was no nipple discharge, ulceration, cutaneous edema, or nipple retraction. On palpation, a deep-seated, slightly tender, hard lump with restricted mobility was felt within the soft superficial breast tissue, measuring approximately 10 cm × 9 cm. Ultrasonogram of the right breast and axilla showed an oval, well-circumscribed, homogenously isoechoic mass lesion with smooth margins, measuring 9 cm × 7 cm, occupying all the four quadrants and normal breast tissue being seen superficial to it. Fine-needle aspiration cytology showed staghorn clusters of benign ductal epithelial cells and scattered bipolar nuclei in fibromyxoid background suggestive of fibroadenoma without significant nuclear atypia, necrosis, and mitosis.
On surgery, the mass was found behind the pectoral muscle separately from the orthotopic breast tissue and was excised by submammary incision []. Histopathological examination revealed a well-encapsulated biphasic lesion comprising both epithelial and stromal components. The stromal component was composed of proliferating spindle cells compressing the epithelial component at places. The stromal component showed a leaf-like overgrowth and perinodal condensation of the stromal cells, whereas the epithelial component had acini-like structures. No atypia or mitosis was seen. The overall features were suggestive of a phyllodes tumor [].


The second case had hypertrophied right and left ventricles with multifocal fibrosis.
The initial clinical diagnosis of this >40 years old man was cardiac amyloidosis. Echocardiography showed a borderline increase in the left ventricular wall thickness with apical regional wall motion abnormalities. Cardiac MRI showed patchy, irregular, and delayed enhancement sparing subendocardium suggesting non-ischemic cardiomyopathy (). PET with 2-deoxy-2-[18F]fluoro- D-glucose (FDG-PET) did not show hot uptake in the myocardium. Holter monitoring showed non-sustained ventricular tachycardia of three beats on two occasions.
Endomyocardial biopsy was performed twice at ages 49 and 51. One biopsy at age 51 showed focal patchy fibrosis with a single micro-granuloma ().
The resected heart after transplantation at age 51 weighed 560 g. Both right and left ventricular walls were hypertrophied. There were multiple white scars in the myocardium, particularly in the left ventricle, the ventricular septum, and papillary muscles (). Histologically, the scars were dense fibrous scars and had fatty replacement. At least five micro-granulomas and histiocytes were scattered ().


On 22 January 2020, a 42-year-old woman with no history of disease presented to Ningbo First Hospital (Zhejiang, China). She suffered from upper abdominal discomfort, including belching, which had persisted for 5 days. She did not report the common COVID-19 symptoms and had not traveled to Wuhan or other endemic areas of COVID-19. She was otherwise healthy, with no history of thoracic, cardiovascular, or abdominal diseases. Endoscopy showed chronic superficial gastritis with erosion and esophagitis. An abdominal computed tomography (CT) scan showed intrahepatic nodules and no lung infection on 23 January (). She was diagnosed with gastritis, dyspepsia, and liver tumors, and was treated with omeprazole, pancreatin, and rebamipide for 3 days.
Enhanced magnetic resonance imaging of the abdomen performed on 26 January showed a lesion in the right lower lobe of the lungs (). CT scans performed on the same day showed multiple ground-glass opacities in the lungs (). She was diagnosed as having pneumonia and was referred to the respiratory clinic for treatment.
On 23 January, she began to experience fever and a runny nose. In consideration of the new scan results and the fever, she was transferred for the second time to the fever clinic on 26 January. Her body temperature was 37.4°C. A complete blood count showed a white blood cell count of 5300 cells/mm3, with 69.4% neutrophils, 22.5% lymphocytes, and 7.9% monocytes. The C-reactive protein level was 5.08 mg/L. Liver function, and creatine kinaselactate dehydrogenase levels were within normal limits.
To treat pneumonia, we empirically started ceftriaxone 2.0 g once daily and levofloxacin 0.4 g once daily. Reverse transcription- polymerase chain reaction (RT-PCR) using a throat swab sample was performed on 27 January, but the result was negative for SARS-CoV-2. The Ningbo First Hospital response team decided that a second RT-PCR assay was required to exclude COVID-19. The patient was persuaded to return to the hospital and tested positive for SARS-CoV-2 with a throat swab specimen by RT-PCR on 29 January. Therefore, her diagnosis was confirmed as COVID-19 in accordance with the Diagnosis and Treatment Guideline of the National Health Committee of China.
The patient was treated with Lianhua Qingwen capsules 16.8 g three times daily, umifenovir 200 mg three times daily, and lopinavir and ritonavir tablets 600 mg twice daily for 2 days. She was transferred to Ningbo No. 2 Hospital on 30 January. She was then treated with moxifloxacin, umifenovir, lopinavir, and ritonavir tablets, interferon-2α, and thymosin. After several days of symptomatic treatment, cough and suffocation in the chest gradually subsided. A chest CT scan showed major improvement. RT-PCR for SARS-CoV-2 was negative twice from throat swab specimens on 5 and 6 February 2020 and once with a rectal swab specimen on 6 February 2020. In accordance with the guidelines, the patient was clear of SARS-CoV-2 and was discharged from the hospital on 6 February 2020.


A 60-year-old lady developed right flank bulge shortly after open nephrectomy for a chronically infected right kidney due to renal calculi. The bulge had been managed conservatively due to perceived technical difficulties and high risk of failure with flank hernia repair. In addition, there was a component of postdenervation myopathy. Surgery was considered in light of worsening pain, inability to perform daily activities and increasing size of the bulge. Significant medical history included morbid obesity (BMI > 35) and previous open cholecystectomy through a Kochers incision. On examination, she had a large non-tender right flank bulge () with unconfirmed reducibility. Computed tomography revealed a large necked right abdominal wall hernia containing multiple loops of small bowel, hepatic flexure, distal appendix, fat and lateral right lobe of the liver.
After evaluation, she underwent a hybrid laparoscopic and open incisional flank hernia repair with concomitant limited flank abdominoplasty. The hernia was marked to delineate skin flap limits for abdominoplasty. A diagnostic laparoscopy was performed (). The hernia defect extended from the right subcostal region to the right iliac crest, medially close to the midline and posteriorly up to the lumbar muscles. Laparoscopic adhesiolysis was performed and hernia sac reduced. The previous nephrectomy scar and large redundant abdominal lateral wall (25 × 15 cm) were excised. The plane between residual external and internal oblique muscles and the transverse abdominis muscle was identified and developed. The fascia transversalis and transversus abdominis muscles were plicated together to create a new preperitoneal layer. A large 30 × 30 cm Ultrapro (Ethicon) mesh was placed in this interstitial position and secured with 2–0 polydioxanone sutures (PDS) and fixed with ProTack (Covidien) fixation device to the lower ribs and iliac crest (). The external and internal oblique muscle flaps were approximated with 2–0 PDS and a further 10 × 15 cm Ultrapro mesh was placed onlay covering the medial aspect of the defect and secured with ProTack and PDS sutures. Two 15Fr Blake drains were placed in under fat plane. Scarpa fascia was sutured with 2–0 Vicryl. Skin closed with staples and 3–0 Nylon sutures (). Abdominal binder was used over the dressing.
Clinical recovery was uneventful. One drain was removed prior to discharge on the fourth postoperative day, and the other on day 9 while draining 44 ml over 24 h. She had a good result postoperatively with resolution of the bulge and preoperative symptoms. There was no evidence of recurrence at 6 months’ follow-up ().


A 30-year-old man, previously fit and well, was brought to the emergency department with four episodes of witnessed generalized tonic clinical seizures. He had been well prior to the event, with no neurological or respiratory symptoms, and there was no history of similar episodes in the past.
On examination the patient was conscious and oriented, temperature was 36.6°C, blood pressure was 139/81 mm Hg, respiratory rate was 18 per minute, and oxygen saturation was 99% on room air. He had no neurological focal deficit. Laboratory results showed lymphocytopenia 0.61 × 109/L (normal 1.0–4.5 × 109/L), raised prothrombin time 15.1 s (normal 9–12 s), raised D-dimer 0.75 mg/L FEU (normal <0.45 mg/L FEU) and slightly raised AST 44 U/L (normal 0–40 U/L). The rest of liver function test, renal profile, and C-reactive protein were within normal limits. Ferritin was 91.0 μg/L (normal 48–420 μg/L). Serology for hepatitis B surface antigen and hepatitis C antibody was negative. HIV test was also negative. Protein C and S activity, lupus anticoagulants, and anticardiolipin antibodies were all negative. A nasopharyngeal COVID-19 swab taken as per hospital screening protocol was positive. Chest X-ray was normal, with no evidence of ground glass opacities or consolidation.
A CT scan of the head showed a small hypodense lesion in the right temporal lobe anteriorly with mild surrounding edema and mass effect. Tiny foci of hyperdense hemorrhage were seen within the lesion and in the adjacent Sylvian fissure (Fig. ). MRI showed that the lesion was a hematoma with susceptibility weighted imaging showing blooming hypointense blood products in the lesion and subarachnoid hemorrhage in the Sylvian fissure (Fig. ). Postcontrast thin 3D T1 MPRAGE images showed nonocclusive filling defects suggestive of clots within the right sphenoparietal venous sinus at the anterior temporal pole (Fig. ). A postcontrast MR venogram of the head showed nonocclusive venous thrombosis of the torcula, left transverse sinus, and sigmoid sinus, extending to occlude the proximal part of the left internal jugular vein (Fig. ).
Seizures were controlled with levetiracetam 500 mg twice daily. Following the MRI results, the patient was commenced on subcutaneous low-molecular-weight heparin 1.5 mg/kg body weight for the first 48 h and subsequently changed to rivaroxaban 15 mg twice daily for the first 3 weeks, then 20 mg daily for a minimum of 3 months. He did not require any treatment for COVID-19 as he remained asymptomatic. He remained stable through the course of admission and was discharged to a quarantine center.


A 43-year-old lady presented to the lower GI clinic secondary to rectal bleeding. She reported her bowels open approximately four times a day and stools were described to be small and loose with occasional clots and ribbon-like characteristics. She also denied having any unintentional weight loss. No previous surgical or medical history.
Initial examination showed soft, non-tender abdomen. Rectal examination revealed a suspicious anterior rectal mass. An urgent colonoscopy was therefore arranged and biopsies returned to show evidence of ulcerative colitis (UC) but no masses/neoplasia. Despite establishing the diagnosis and initiating appropriate Asacol and mesalazine treatment, she subsequently became significantly unwell and presented through the emergency department with severe abdominal pain, distention and vomiting. Physical examination revealed a blood pressure of 101/67 mmHg, HR of 123 beats/min, respiration rate of 24 breaths/min and saturations of 90% in room air. Computed tomography (CT) abdomen and pelvis not only showed descending colon inflammation and severe terminal ileitis but also moderate volumes of free fluid with extensive intra-abdominal inflammation. Initial laboratory blood tests were Hb 132, WBC 8.23, Plts 44, lactate 1.2, CRP 72, K+ 3.3, AST 46, Albumin 37. PR bleeding continued and haemoglobin levels began to drop out of proportion, reaching a low of 70 g/l as well as derangement of her coagulation studies. She was then escalated to critical care given her persistent hypotension and DIC, but ultimately she underwent emergency laparotomy. In theatre, we identified a massive retroperitoneal haematoma and approximately 30 cm of gangrenous terminal ileum. Patchy gangrene tissue of the ascending and descending colon was noted with preservation of the sigmoid colon. A subtotal colectomy was performed with end ileostomy and the cross-stapled sigmoid stump was left in situ. Additionally, two large (3 cm) abnormal mesenteric lymph nodes were also identified adjacent to the DJ flexure. These were resected for histological analysis. Histology of the lymph node surprisingly showed evidence of metastatic neuro-endocrine carcinoma with characteristics of solid trabecular growth and moderate nuclear pleomorphism ( and ). Post-operatively, she underwent a number of investigations in hope to identify the primary, as well as tests to identify the cause of the thrombosis.
Initial basic laboratory investigations on presentation are mentioned above; however given the complexity of this case, further investigation was required. An octreotide single-photon emission computed tomography (SPECT/CT) imaging was performed in hope to identify any additional active tumour cells (). An avid ~15 mm node was noted in the proximal mesenteric jejunum (), as seen intra-operatively, with no additional pancreatic, small bowel or colonic uptake. Other specialized laboratory investigations included chromogranin A&B, which were within normal values of 20 and 88 pmol/L, respectively. 24-h urine 5-HIAA test was also normal of 14 μmol/24 h. The overall image and appearance closely points to a well-differentiated grade 2 neuro-endocrine carcinoma.
The patient continues to experience passing blood per rectum secondary to the ulcerative proctitis however she is making a good recovery post-surgery.


A 22-year-old Caucasian lady presented in 2014 with features of pulmonary fibrosis, chronic cutaneous problems, and symmetrical polyarthritis. She had previously been well without any comorbidities. A high-resolution computed tomography (CT) chest and lung biopsy were performed, with a diagnosis of hypersensitivity pneumonitis being made, in the context of significant mould exposure. Skin biopsies demonstrated spongiotic dermatitis (eczema). Echocardiography revealed right ventricular dilatation with mild tricuspid regurgitation. Left ventricular size and function was normal.
She was discharged and attended as an outpatient to a specialist centre for pulmonary hypertension, as it was assumed at the time that the right ventricular dilatation was a consequence of undiagnosed pulmonary hypertension. Here, right heart catheterization indicated normal pulmonary arterial pressure. Subsequently, no cardiology follow-up was arranged. The patient was treated with methotrexate, sulfasalazine, and intermittent prednisone over the next 4 years, although a single unifying diagnosis to explain her all of her symptoms was not identified.
In 2018, the patient attended a rheumatology clinic. The rheumatologist was concerned that there were clinical signs of pulmonary hypertension detected on physical examination, and thus a routine echocardiogram was performed. This study demonstrated severe right ventricular dilatation (). There was now severe tricuspid valve regurgitation (). The left ventricular size was normal, as was systolic and diastolic left ventricular function. There was no significant left-sided valvular disease. The patient was experiencing dyspnoea on walking 50 m on a flat surface, or at the top of a single flight of stairs. Additionally, she described four episodes of unheralded syncope, all occurring whilst seated. On physical examination, the blood pressure was 130/70 mmHg, the heart rate 88 b.p.m., and regular, with oxygen saturation of 99% on room air. The jugular venous pressure was elevated with prominent V waves. There was a right ventricular heave and a loud pan-systolic murmur audible at the left sternal edge. Pulsatile hepatomegaly was present. Fine crackles were present in both lung bases. Electrocardiogram demonstrated atrial fibrillation with a right bundle branch block ().
Due to the concerning features on echocardiography and the symptoms of dyspnoea and syncope, the patient was admitted to a monitored cardiac ward for further investigation. Telemetry demonstrated non-sustained ventricular tachycardia (VT) at a rate of 150 b.p.m. (), of which the patient was symptomatic with palpitations but there was no haemodynamic compromise.
Right heart catheterization revealed normal pulmonary arterial pressures (22/10 mmHg), but elevated right atrial pressures (16/14 mmHg). Cardiac output (5.4 L/min) and pulmonary vascular resistance (88 dynes.s/cm5) were normal.
The patient underwent transoesophageal echocardiography (TOE) and successful electrical cardioversion, restoring sinus rhythm. The TOE excluded atrial thrombus, any intracardiac left-to-right shunt, and all four pulmonary veins communicated with the left atrium.
Cardiac magnetic resonance imaging (MRI) did not show any late gadolinium enhancement in either ventricle, and congenital anatomical anomalies were excluded. Cardiac positron emission tomography ( PET) did not reveal increased metabolic activity to indicate inflammation or features of active cardiac sarcoidosis. A CT chest demonstrated unchanged features of pulmonary fibrosis.
Myocardial biopsy was performed under fluoroscopic guidance. Histopathology revealed a predominantly lymphocytic chronic inflammatory infiltrate (). A single well-formed small granuloma as well as a second focus of scattered giant cells was noted. Moderate to severe myopathic features were seen, including myocyte necrosis with features of myocytolysis and anisonucleosis, and patchy regenerative interstitial fibrosis. The diagnosis was consistent with lymphocytic myocarditis. The morphological pattern that comprised myocyte necrosis and a widespread lymphocytic infiltrate without predominating giant cells and granulomas was less likely to be consistent with cardiac sarcoidosis or giant cell myocarditis. There was a paucity of eosinophils, making the diagnosis of hypersensitivity myocarditis highly unlikely. The classic features of arrhythmogenic cardiomyopathy (AC) were also not seen, i.e. patchy to diffuse irregular replacement of myocytes by adipose tissue, with associated variable amounts of fibrosis, lack of significant lymphocytic infiltrate, and scattered enlarged degenerate vacuolated myocytes. A broad range of serological investigations had also been conducted, with no significant positive results.
Given the documented symptomatic VT, and the history of syncope, the patient was referred for a cardiac electrophysiology study, which demonstrated inducible VT originating from the RV apex that lasted for over 30 s. Following a multidisciplinary discussion, the patient consented to the implantation of an automated implantable cardioverter-defibrillator (AICD).
The patient was commenced on prednisone 25 mg daily, as well as sotalol 80 mg twice daily and apixaban 5 mg twice daily, prior to discharge. Since discharge, her prednisone has been ceased after being systematically weaned following commencement of azathioprine 100 mg daily. She has been stable thereafter, with no further arrhythmias and has returned to work. Occasional episodes of decompensated mild right heart failure have been treated successfully with oral diuretics, without the need for intravenous therapy or inpatient admission. An echocardiogram performed 12 months after discharge revealed a marginal improvement in right ventricular function, with residual severe tricuspid regurgitation. As the overall clinical condition of the patient has remained stable, repeat myocardial biopsy was not performed.
The process is considered chronic based on the first detection of right ventricular abnormalities 4 years prior. In addition, the chronic inflammatory infiltrate and interstitial fibrosis supports this conclusion.
Thus, we describe a case of an immunosuppressed young lady who was suffering from a chronic low-grade lymphocytic myocarditis of the RV resulting in dilatation of the chamber, severe functional tricuspid regurgitation, and arrhythmic complications including atrial fibrillation and VT. All cardiac imaging modalities demonstrated normal left ventricular size and function, suggesting this process demonstrated a preponderance to the RV.


A 65-year-old man underwent a pace-and-ablate strategy for drug-refractory, symptomatic atrial arrhythmias. Since LV systolic function was impaired, a biventricular pacemaker was implanted with a coronary venous LV lead. Initially, LV thresholds were stable and he obtained a good haemodynamic response to LV pacing. However, insurmountable positional phrenic nerve stimulation required LV lead deactivation—subsequently heart failure recurred. As the coronary venous anatomy was considered to be unfavourable, an LV endocardial lead was placed transatrially via a patent foramen ovale. Despite initial clinical improvement, he presented with worsening breathlessness after 9 months. Transoesophageal echocardiography (TOE) demonstrated severe mitral regurgitation with tethering of the posterior mitral valve leaflet to the LV lead (Figure , left-hand panel). A decision was made by the multi disciplinary team (MDT) to undertake percutaneous removal with surgical standby, and the LV lead was extracted using a firm, LV length non-locking stylet and simple traction. The lead detached from the LV endocardium without any acute consequences and was successfully removed ( – endo lead removal case 1). An LV lead was reimplanted via the coronary sinus and deployed without phrenic nerve capture. The patient had a good clinical response at 6 months follow-up.


A 53-year-old male with non-ischaemic cardiomyopathy, heart block, and bronchiectasis in the context of an auto-inflammatory syndrome, underwent implantation of a primary prevention biventricular defibrillator. Despite long-term antibiotic therapy, recurrent device infections occurred, requiring repeated extractions and re-implantations. A lack of viable coronary venous options indicated the implantation of a transatrial septal LV endocardial lead in a second procedure. Thirty months following this, a device infection developed and a full system extraction was undertaken.
The right atrial (RA) and right ventricular (RV) leads were extracted in their entirety with lead locking device stylets (LLD, Spectranetics) and 11 and 13 Fr mechanical sheaths (Tightrail, Spectranetics). The LV endocardial lead (Medtronic 5076 CapsureFix, 85 cm) was successfully removed using gentle traction and an LLD stylet. Following recovery from active infection, a surgical epicardial system was implanted.


A 77-year-old male with ischaemic cardiomyopathy underwent dual-chamber pacing for high-degree atrioventricular (AV) block. He had repeated device infections with subsequent extractions and re-implantations. He was referred for upgrade to a biventricular defibrillator on the basis of impaired LV function, drug-refractory symptoms of heart failure, and high-degree AV block. Conventional LV lead placement failed due to unfavourable coronary venous anatomy and a transatrial LV endocardial lead was implanted as part of the Alternate Site Cardiac Resynchronization (ALSYNC) study. During this implant procedure, he suffered a thromboembolic stroke which left him with permanent loss of vision in his left eye—he responded favourably to CRT therapy. However, an early device infection developed necessitating system extraction. Given his excellent response to CRT, a transventricular LV endocardial lead with a new cardiac resynchronization therapy defibrillator (CRTD) system was implanted successfully. After 3 years he presented once again with device-related infection necessitating complete system extraction. Transoesophageal echocardiography images of the LV lead traversing the ventricular septum can be seen in Figure (middle- and right-hand panels)( – transventricular lead on 4ch echo case 3). Lead locking device stylets were deployed to all three leads (LLD, Spectranetics). The RA lead was removed with gentle traction alone. The RV lead required the addition of a 13 Fr mechanical sheath (Tightrail, Spectranetics). The endocardial LV lead was easily removed with simple traction. A small residual ventricular septal defect (VSD) was evident on intraoperative TOE. However, there was no clear shunt evident at repeat transthoracic echocardiography 48 h later. The patient underwent a dual-chamber internal cardioverter-defibrillator (ICD) implant on the contralateral side and was subsequently implanted with a wireless LV endocardial electrode (WiSE CRT, EBR systems, USA). After 6 months of follow-up he remains well, free from infection with improved exercise tolerance.


A 23-year-old male with hypertrophic cardiomyopathy underwent primary prevention dual-chamber ICD implantation in 2009. The original implant procedure was complicated by a blood-stained pericardial and pleural effusion requiring readmission and drainage, the aetiology of which was unclear. In 2016, the original RV lead developed noise and was removed and replaced uneventfully. In 2018, he presented with a grand-mal seizure, on a background of non-specific neurological (predominantly visual) symptoms over the preceding 2 years. Subsequent echocardiography and cardiac computed tomography demonstrated the ventricular lead had inadvertently traversed a (previously unknown) superior vena cava (SVC) to right pulmonary vein connection and was in fact implanted in the LV endocardium. Embolism of material attached to the ventricular lead was thought to be the cause of his neurological symptoms. Following review in the multi disciplinary team (MDT) meeting, a full system extraction was scheduled in a hybrid theatre and cardiac catheter laboratory with cardiothoracic surgical standby. Both leads required simple traction alone for successful removal using stiff non-locking lead stylets and this was performed without complication ( - lv icd lead removal). The patient was subsequently implanted with a subcutaneous ICD. He also underwent angiography confirming congenital anomalous connection between the right upper pulmonary vein and the SVC.


A 46-year-old gentleman presented to our emergency department with a 7-day history of generalized abdominal pain associated with abdominal distension, in addition to multiple attacks of vomiting and loose bowel motion. He denied any history of weight loss, chronic diarrhea, night sweats, or any past medical illness.
Upon examination, generalized abdominal distension was noticed, associated with generalized guarding and tenderness all over his abdomen, without any detected impulse at the hernial orifices.
Computerized tomography (CT) scanning of the abdomen and pelvis with double oral and IV contrast revealed a segmental mural wall thickening with increased enhancement involving distal ileal loops with a suspected soft tissue mass (Fig. ), resulting in back dilatation of the jejunal and ileal loops in addition to multiple air-fluid levels within it. Moreover, intraperitoneal fluid collection and multiple mesenteric lymphadenopathy were noticed.
On laboratory work-up, the results showed leukocytosis of 26 × 103 and high serum creatinine (1.3 mg/dL) and LDH (>1,995 U/L) levels. As a result, the patient was prepared for an emergent abdominal exploration as a case of small bowel obstruction, after anesthesia assessment and the patient's consent for surgery. Exploratory laparotomy was conducted via mid-line incision with identification of 1.5 L of intraperitoneal pus collection and a mass 5 × 5 cm in size at the terminal ileum with a dirty omentum and multiple hard mesenteric lymph nodes. As a consequence, ileocecal resection was done, followed by primary ileocolic anastomosis. In addition, omentectomy was performed with biopsy of the peritoneum (Fig. ).
The surgery was finalized after appropriate hemostasis and insertion of two intra-abdominal drains. Postoperatively, the patient was shifted to the intensive care unit for 4 days, and then shifted to a regular room in stable condition.
Histopathological assessment of the excised specimens revealed a 9 × 3 cm high-grade B-cell lymphoma (Burkitt's lymphoma) in the ileocecal region with involvement of all mesenteric lymph nodes, the omentum, and the peritoneal biopsy.
During the postoperative follow-up, the patient developed right-sided pleural effusion, which was managed by US-guided drainage with the insertion of a pig-tail catheter. Cytological analysis of the drained fluid revealed malignant cells. The patient was transferred to a tertiary care center for further management by adjuvant therapy.


A 6-month-old girl presented with mild development delay and infantile spasms since 3 months of age. The frequency was 2–3 spasms/cluster and 2–3 clusters/day. The prenatal and perinatal period was uneventful. No significant family history was observed. Examination revealed multiple ash leaf spots over left arm, right leg, and buttocks []. She also had a pink-red blotchy stain over right upper limb extending to chest and back []. Also, there was visible hypertrophy of the right upper and lower limbs []. Rest of the systemic and neurological examination was normal. Magnetic resonance imaging (MRI) of brain revealed multiple cortical tubers and subependymal nodules (SEN) with calcification [Figure and ]. Echocardiography and abdominal ultrasound were normal. EEG was suggestive of hypsarrhythmia. In view of multiple tubers, SEN, and ash leaf spots (three major criteria for TSC), and right hemihypertrophy and port-wine stain (two of the classic triad of KTS), a diagnosis of TSC with KTS overlap was made. She was initiated on vigabatrin with which the spasms became passive.


A variant SN was found during the routine dissection of a male Caucasian cadaver aged 72-years-old at death. The right SN was split by the PM into two roots, a superior CFN part and an inferior TN part (). The superior root was found to pierce the piriformis and the inferior root traveled inferior to the inferior border of the piriformis (infrapiriformis foramen). Distal (approximately 3 cm) to the piriformis, the CFN and TN were united by a communicating branch (Cb) descending superomedial to inferolateral in an oblique manner. The Cb was approximately 1 cm in width by 3 cm in length. Distal to this Cb, the CFN and TN continued toward the popliteal fossa and were distributed in normal fashion to the anterior/lateral and posterior compartments of the leg, respectively. No atrophy or other pathology was noted in any muscles of the leg and no other anatomical variations of the left or right lower limbs (including gluteal region) were noted.


A 66-year-old female presented to our clinic with the mixed diagnosis of essential tremor and Parkinson’s disease, as she had both resting and action components of tremor in bilateral upper extremities with bradykinesia and rigidity that were somewhat improved on levodopa. The tremor was largely refractory to medication and interfered with her quality of life. She underwent bilateral DBS lead electrode implantation targeting the dentatorubrothalamic tract, specifically, the ventral intermediate nucleus (Vim), in the thalamus using the standard stereotactic protocol.[] A trajectory through the ventricle was avoided. Normally, we start by implanting the microelectrodes on the more symptomatic side and then proceed to the other side. In this case, three microelectrodes were simultaneously descended to target the left Vim (as her symptoms were worse in her right hand) first, followed by another three microelectrodes to target the right Vim. Their cannulas were used for macrostimulation to assess for improvement and to choose the best trajectory. Electrode placement (Medtronic 3387 model, Minneapolis, MN, USA) then occurred after confirmed improvement in tremor. The rostral ends of the electrodes were left in a subgaleal pocket to be accessed during a subsequent staged procedure for extension and pulse generator placement. The lead placement was verified in the operating room theater with computed tomography (CT) imaging before closure. Surgery was uncomplicated, and the patient remained interactive and conversant throughout. She was admitted to our neurosurgical ICU as per routine. Head CT performed on early postoperative day (POD) 1 was unremarkable []. Physical examination revealed no deficit; the patient complained of headache with some nausea/vomiting. She desired to stay overnight. An examination later on the evening of POD 1 found her to be sleepy, and ultimately lethargic. Stat head CT performed revealed marked left-sided peri-lead edema extending into the centrum semiovale with cystic cavitation and trace right-sided edema []. Physical examination on the morning of POD 2 revealed the patient to be alert but with global aphasia (not following commands and not speaking), right-sided neglect, and plegic right upper extremity. Corticosteroids (IV dexamethasone) were begun early on POD 2. She later became increasingly lethargic, and over concerns for airway protection was intubated. Repeat head CT revealed increased edema.
The critical care team was concerned for fulminant gas- producing bacterial infection as suggested by neuroradiology interpretation of cavitation surrounding one lead and strongly pushed for lead removal, which was resisted. Vancomycin and meropenem were empirically begun. Systemic tests for infection, including C-reactive protein, erythrocyte sedimentation rate, and white blood cell counts, were normal, as well as blood cultures, which were ultimately negative at 24, 48, and 72 h. Such negative infectious workup and lack of change on serial repeat imaging disproved this idea. Magnetic resonance imaging could not be performed due to safety concerns at our institution with an incomplete DBS circuit. Acute venous infarction was also considered a possibility, but the radiological appearance of a cortical- subcortical typically wedge-shaped ischemic pattern was not present.[] This patient ultimately underwent tracheostomy and percutaneous endoscopic gastrostomy placement 6 days later. She was transferred to a rehabilitation facility on a steroid taper and subsequently discharged home on POD 40. She returned to the clinic 3 months after surgery fully recovered and ready for lead extension and pulse generator placement. Follow-up CT scans at the time showed significant resolution of the peri-lead edema and cystic cavitation [].


A 5-year-old boy with a more than 2 months history of otogenic CVST of the right sigmoid sinus was referred by pediatrician consultants. Despite adequate antibiotics and anticoagulant therapy, the child developed progressive bilateral papilledema and visual loss. An MR imaging (MRI) – MR venography (MRV) of the head showed a thrombotic occlusion of the right transverse-sigmoid sinus associated with a tight stenosis of the left transverse sinus [ and b] and bilateral enlargement of the optic nerve sheath with bulging of the optic discs []. Serial consecutive measurements of the visual acuity revealed a progressive and dramatic visual loss over 1 week. The case was discussed in a multidisciplinary meeting, and a decision was made to offer an endovascular reopening of the occluded right side, transverse-sigmoid sinus.
An informed consent signed by both parents was obtained, and the procedure was performed under general anesthetics on a biplanar angiography system (Philips Allura). A 7F guider was placed in the right jugular bulb and a diagnostic 4F catheter in the right internal carotid artery (ICA) through a femoral (venous 7F and arterial 4F) approach. After several attempts, we managed to cross the occluded segment of the sinus with a 016” guide and a 021” microcatheter. A 6 × 30 mm Solitaire (Medtronic) stent- retriever was then delivered across the thrombosed sinus, unsheathed and, after a few minutes, wait, trashed out. A second pass with the stent-retriever was repeated: each time only small and scanty clot fragments were retrieved. While attempting to cross the occlusion for the third time, the child presented a transient tachyarrhythmia (200 bpm) and hypotension requiring the anesthesiologist’s assistance with a quick resolution of the event. Once the child was hemodynamically back to normal, a dynaCT was obtained, and it resulted in normal appearances. Subsequent check angiograms showed an unsatisfactory partial reopening of the occluded sinus []. A decision was made to stop the procedure after placement of two 0.21” microcatheters in the right transverse and sigmoid sinuses, respectively, their distal tips 2–3 cm apart. Then, a local infusion of rt- PA through the two microcatheters was started. The total dose of the rt-PA was titrated according to the child body weight and evenly split between the two microcatheters at a rate allowing a total 6 h infusion. The boy was transferred to the Intensive Care Unit (ICU), tubed, and sedated over the night. The following morning, a single pass with two unsheathed, and side-to-side 6 × 30 mm Solitaire stent- retrievers (“double stent-retrievers” technique), followed by aspiration through the 6F guider, resulted in a satisfactory reopening of the right sigmoid sinus [ and c]. Final angiograms demonstrated an improved cerebral venous outflow []. At the end of the procedure, a therapeutic dose of heparin was prescribed and the child was woken up in ICU. In the following days, the child vision progressively improved and he could play cards with his mother (recognizing the colors and numbers on the cards). A week later, the bilateral papilledema resolved with further visual acuity improvement. A follow-up MRI three weeks later showed a stable, though partial, reopening of the right sigmoid sinus with a resolution of the bilateral optic nerve sheath enlargement and disc bulging. The boy was discharged home on a full dose of fractionated heparin.


A 53-year-old female presented with dyspnoea and atypical chest pain on a background of hypertension, impaired glucose tolerance and asthma. The patient was normotensive, normal saturation on room air, with a normal heart rate and rhythm. There were no significant clinical findings on examination. Electrocardiogram was significant for a left bundle branch block. Transthoracic echocardiogram showed normal left ventricular (LV) size with mildly reduced ejection fraction. Coronary angiography demonstrated unobstructed coronary arteries. She was referred for CMR to further assess her LV dimensions and function..
The patient underwent standard comprehensive screening for contraindications to CMR. This was negative. The CMR demonstrated a small LV cavity with mildly impaired ejection fraction, dyssynchronous contraction and mild concentric LV hypertrophy. Right ventricular volumes and function were normal. There was no myocardial infarction or infiltration. Unexpectedly the apical segments were partially contaminated by signal loss and distorted by off-resonance artefact consistent with a potentially ferromagnetic body (FB). This artefact was particularly prominent on balanced steady-state free precession (bSSFP) sequences (). The patient had no history of previous surgery, other intervention, penetrating injury, and no recollection of an event that would have resulted in a FB within the thorax. She was not on iron supplementation. The patient was well and had no ill effect at clinical follow-up.
Further investigation with chest X-ray revealed a thin needle-shaped structure near the cardiac apex (). A three-dimensional volume-rendered image on multi-detector computer tomography (Somatom flash, Siemens Medical Solutions, Erlangen, Germany) showed a thin needle-shaped metallic density in the right ventricular side of the apical septal wall ().
Previous transthoracic echocardiogram images were reviewed. The FB was not visible. This patient’s coronary angiogram report was requested from the referring institution and reviewed. The coronary angiogram had made incidental note of a slim line of radio-opacity that moved in time with the heart, but the exact location was unclear.
Controversy was raised regarding patient safety in undergoing further CMR examination vs. inappropriate refusal to arrange follow-up imaging for a patient with cardiomyopathy. Following a review, the patient was treated for a non-ischaemic cardiomyopathy and follow-up CMR imaging deemed safe. Finally, protocol adjustments were required to optimize images and minimize the artefact. Informed consent was obtained for this publication.


A 47-year-old male visited an otorhinolaryngologist with a chief complaint of bilateral hearing loss since July 2019, which was gradual in onset. In November 2019, the treating otorhinolaryngologist after managing conservatively and reporting no relief in symptoms referred the case to the medical oncology department of a private hospital in New Delhi. On examination by a medical oncologist, the patient reported the loss of hearing and pain. He complained of pain over the preauricular region, which was described as dull, aching, and bilateral. Pain which used to be on and off and started as mild but gradually increased on rare occasions which got relieved by taking tablet tramadol 50 mg. At the clinic, the patient got investigated, and one small growth was found at the posterior nasopharyngeal wall which was confirmed to be NPC on biopsy. PET scan report shows that the disease is confined to its anatomical site of origin and is not metastasized yet. He was given six cycles of chemotherapy (CT) over a span of 9 weeks. He responded to the treatment and planned for further therapies. Then, the patient was given concurrent chemoradiation. The patient got the last radiotherapy on April 10 and chemotherapy on April 8.
One week later, he developed a low-grade fever which was considered as a complication of chemotherapy. However, when fever continued, he was suspected of corona infection, so he was sent to the COVID-19 testing center where he came negative. Then, the patient developed urinary retention the next day and found to have some growth in urinary bladder and bladder was overflowing according to CT scan. Urinary catheterization tried but failed, so the patient was planned for suprapubic catheterization. Before the procedure, he was again tested for COVID-19, and the report came out to be positive. Plan for suprapubic catheterization was changed and urinary catheterization tried which was successful this time with smaller size catheter (10 F). Then, the patient was sent to RML Hospital, New Delhi, on April 28, and from there, he was referred to the COVID care facility at NCI-AIIMS Jhajjar.
The patient is asymptomatic given COVID-19 infection and being managed conservatively. His vitals are stable; oxygen saturation is well maintained above 98% on room air. He has been tested thrice for COVID-19. He is still getting reverse transcription polymerase chain reaction (RT-PCR) positive for a month. As the patient got COVID-19 infection just after his therapies for NPC, immunosuppression can be the reason. This shows that cancer patients with ongoing treatments have a higher risk.
He is not able to communicate properly with health-care workers and his family members on the phone as he has hearing difficulty. Due to a lack of communication, he feels lonely and unattended. His father is the main caregiver, and his family has been quarantined so he is unable to get a few items which he needs from home. As the father is unable to come to meet his son, he is also feeling guilty for not being able to fulfill his needs.
His first report was negative then subsequent 3 RT-PCR test reports came positive. He is regularly asking about his report and doubting the results. He is worried and in doubt that why others are getting negative after 2 tests, but he is not. He does not trust the report. His father is also repeatedly calling to ask why his report is coming positive.
He has this fear that his condition will deteriorate as he is getting repeated positive reports. He is afraid that his cancer treatment plans will get delayed and will affect his outcome.
Due to the overwhelming number of people seeking medical care and the burden that COVID-19 is placing on health-care providers, it has become more difficult to access regular cancer treatments during this time. Further treatment of cancer and follow-up is getting interrupted due to COVID-19 infection. Delay in the treatment is making the patient more anxious and irritable. His father believes that his cancer will get cured if he gets further treatment. But due to COVID-19, his treatment is getting affected.
The role of a caregiver during this time is to provide support and stability. Recommend patient or family members to avoid watching the news if it causes them anxiety or concern. Reassure patient that you will always be available by phone or video call. Continue to remain in contact with your patient. As a caregiver, he should be the pillar of security and comfort. Preparing a safe environment and providing the right resources will ensure the patient.


A 52-year-old female who recently recovered from a myocardial infarction following a coronary angiography and stenting was not fortunate enough to be in good health thereafter. She developed symptoms such as pain upper abdomen, feeling of fullness even with small meals, and loss of appetite just 1 month after her stenting. Perplexed by her symptoms, she visited the hospital in January 2020. She underwent a battery of investigations including an upper gastrointestinal endoscopy which suggested a growth at the lower end of the stomach compromising the gastric outlet. After having a tissue diagnosis of adenocarcinoma stomach, the patient was devastated and worried about the outcome the disease will have for her. The patient developed a ray of hope when she was planned for surgery considering the localized nature of the disease. She was informed that an early diagnosis and treatment will have favorable outcomes for her. She was scared by the outcomes and extensive nature of the surgery but still hoped to get through it and be better.
However, when her surgery time neared, the country had been hit by the pandemic. Because of the few people being diagnosed of coronavirus, the hospital where her surgery was planned decided to put on hold major elective surgeries to avoid the risk of coronavirus to patients. She was informed that her surgery was canceled to avoid any risk to her health considering her high risk for complications because of the pandemic. She recalled that she felt like the floor slipped off from under her. Her caregiver stated that they understood that the decision of postponing of surgery was in their favor, but they were distressed by the fact that what if it progressed. Their mind kept wondering if there was some way that they could get the surgery done and still did not get the infection. They were advised certain home-based medications for the disease to tide over some time.
After 1½ months of this, the patient presented in the emergency department of our hospital with progressively increasing dysphagia, multiple episodes of vomiting, and significant weight loss. On initial stabilizing, a scan was done which suggested complete gastric outlet obstruction along with metastatic spread to the liver. It was informed that all that could be done for her in the present situation is a feeding jejunostomy to help her intake of food. Because in the present cachexic situation, it will take her some time to build up before she can tolerate a chemotherapy. This left the patient and her relative startled and they could not believe their ears. On explaining about the advanced nature of the disease, they were heart wrenched. The patient was annoyed at the entire situation and said I do not know whom to blame for my current situation. She said that ever since I was diagnosed with this disease, the only good thing I thought was its early stage. But now, because of this pandemic, although I did not get infected, should I be happy or be furious that I am in an even worse situation nearing death. It was a difficult conversation which led us wondering what went wrong that she is in such a situation.


A 22-year-old Indian male is presented with chief complaints of increased frequency of micturition, burning micturition, graveluria, and passage of hair in the urine. There was no history of abdominal pain or hematuria. There was no significant history or any family history of malignancy. Physical examination was essentially normal. Clinical, routine blood analysis and urine analysis, as well as all other laboratory workups, were within the normal limits. Computed tomography (CT) scan showed a mass lesion in the bladder with calcification measuring 2.5 cm × 2 cm × 1.5 cm. The patient was hospitalized and planned for a cystoscopy. Cystoscopy showed a 3 cm × 3 cm sized whitish flat lesion with scattered areas of calcification and hair follicles on the right anterolateral wall []. Complete transurethral resection of the mass was done. The histological report confirmed it to be mature teratoma, a benign mature teratoma with ectodermal, mesodermal, and endodermal elements and confined to the bladder wall, with no extension outside []. The patient was discharged and planned for surgical excision. Right now, the patient is symptom-free and waiting to decide about the surgical excision.


A 62-year-old male presented to the retina clinic with a GRT detachment in his left eye (his right eye had been blind for several years), reducing vision to counting fingers at 1 meter. Following the repair of the retinal detachment (using a combined vitrectomy and encirclement band) in December 2017, his vision improved to 6/36. In the early postoperative period, a large bubble of sub-macular PFCL was noted supero-temporal to the fovea. He was reluctant to having this PFCL bubble removed, since he did not wish to risk a reduction in his gained vision.
After 4 months, he had an uneventful phacoemulsification of cataract, with implantation of a posterior chamber intraocular lens and silicone oil removal (SOR). His left eye vision improved to 6/18, N10 (aided). His vision was stable for over 18 months despite the presence of the parafoveal PFCL. His macula status and the PFCL bubble were monitored using OCT and OCTA, and he was quite satisfied with his vision. At his clinic visit in October 2019 (22 months post-primary retinal surgery), the parafoveal PFCL had migrated to the subfoveal position and this was associated with a reduction in vision to 6/36.
As a result of the decrease in vision, he consented to the removal of the subfoveal PFCL. He also had an ERM at this time and the surgical plan was to remove both subfoveal PFCL and ERM.
Surgical steps for the intended surgery was, first detach the foveo-macula, permitting the displacement (using intravitreal injection of PFCL) of the subfoveal PFCL away from the fovea to a peripheral retina position where it can be safely and easily removed via a retinotomy. Peel the ERM and perform air tamponade. It was the thinking that the injection of subretinal fluid would guarantee a more complete removal of all subfoveal PFCL compared to a trans foveal drainage approach.
Surgery was performed in October 2019. As in previous surgeries, he gave written informed consent. During surgery, a 39-gauge flexible cannula was used to inject subretinal balanced salt solution (BSS) superior to the fovea (), detaching the entire foveo-macula area (). A thick ERM was peeled from the detached macula ( and ). This was complicated by the formation of an iatrogenic macular hole (MH). Intra-vitreal injection of PFCL displaced the subretinal fluid with the bubble of PFCL to the retina periphery. An inverted internal limiting membrane (ILM) flap was created and used to close the MH. A superior drainage retinotomy enabled drainage of all subretinal fluid, which included the displaced PFCL bubble. Air fluid exchange was performed, and the vitreous cavity was filled with air. He was advised to have 3 days of face-down position.
Since this was a single case report, the institutional review board (IRB) waived the need for a formal review process. Institutional approval was required to publish this case report and a permission to report the case was given by the IRB. A verbal informed consent for publication of his case report and all accompanying images was given by the patient. He was in a remote locality, with limited access to electronic media and was unable to give a written informed consent. This verbal informed consent was approved by the IRB.


A 55-year-old right-handed diabetic male presents with skin necrosis and infection on the left thumb and thenar eminence. The infection started from a 1-cm laceration obtained 6 weeks prior to his admission while working construction. At admission, the patient’s history included poorly controlled diabetes and peripheral neuropathy. The patient was started on broad spectrum antibiotics as soon as he arrived. Extensive debridement was also undertaken at admission. Cultures obtained during surgery of the initial debridement were positive for group B Streptococcus.
No improvement was observed in the next 12 hours, with expansion of the area of necrosis in the superficial and deep fascia, expanding necrosis of the left thenar eminence and medial side of the hand. The lack of improvement prompted two consecutive debridements with wound vac application in the following 48–72 hours. Between debridements, intravenous antibiotics were utilized but with poor response. As the infection worsened, the patient’s health deteriorated with increased fever, leukocytosis, septicemia and renal failure. Distal left forearm amputation was undertaken as necrosis worsened.
After amputation, the patient improved and was evetually discharged. Two years after surgery, the patient continued rehabilitation training with an occupational therapist to adjust to the myoelectric prosthesis. The patient described the training as difficult and had gain some function in moving the prosthetic arm. The patient still had goals of improving his function with the prosthesis ( and ).


A 52-year-old man presented to our hospital complaining of dull pain and fullness in the upper abdomen for 2 mo, especially after eating. He reported no fever, diarrhea, hematemesis, black stool, or other discomfort.
The patient’s symptoms started 2 mo ago. He took domperidone but had no relief.
The patient had tuberculosis 20 years ago, and he had habits of drinking alcohol 150 mL per day and smoking 20 cigarettes per day for over 30 years.
Upon admission, the patient’s temperature was 37.0 °C, heart rate was 80 bpm, respiratory rate was 18 breaths per min, and blood pressure was 120/80 mmHg. Physical examination showed tenderness in the upper abdomen.
Routine blood examination showed mild erythrocytopenia (4.19 × 1012/L). Serum carcinoembryonic antigen (CEA) level was 9.9 ng/mL (reference range, < 4.7 ng/mL), and other serum tumor markers were normal, including CA19-9, CA724, SCCA, and α-fetoprotein.
Endoscopy showed a congestive mucosa in the gastric fundus with edema and an irregular ulcer approximately 2.5 cm × 2.5 cm in dimension at the antrum-body junction (Figure ). Abdominal contrast-enhanced computed tomography further revealed irregular thickening and enhancement of the wall of the gastric antrum near the pylorus (Figure ). No abnormalities were found in the esophagus, duodenum, liver, pancreas, kidney, or bladder.


A 27-year-old male was admitted to our hospital with edema of lower extremities and fatigue. Before admission, he had received the hydrochlorothiazide treatment for one week (25mg, qd) at a local hospital. After hydrochlorothiazide administration, he still felt weak, although the swelling was slightly relieved. Consequently, he discontinued the treatment because of its poor therapeutic effect. The patient acknowledged that he had never taken any exogenous hormone.
The patient’s blood pressure and body mass index were 127/88 mmHg and 35kg/m2, respectively. Moreover, his weight had increased by about 25kg during the previous 6 years. The patient’s secondary sex characteristics appeared when he was 15 years old, and although, he had normal external genital organs, but he had never experienced erection and ejaculation. Physical examinations revealed that he presented centripetal obesity, moon face, buffalo hump, and purple striae (). Mild swelling was found in his lower extremities.
Laboratory tests revealed the following data: adrenocorticotropin (ACTH)<0.220 pmol/L (1.1–13.2 pmol/L); 24-hour urinary 17-hydroxycorticosteroids and 17-ketosteroid was 1.142mg (2–10 mg/24h) and 6.286mg (6–25 mg/24 h), respectively; and 24-hour urinary-free cortisol was 11.95nmol (100–379 nmol/24h). Other data obtained are shown in . Pituitary magnetic resonance imaging showed normal pituitary anatomy (), while the adrenal computed tomography scan showed bilateral adrenal atrophy and increased content of intra-abdominal fat and abdominal subcutaneous fat (). Dual-energy X-ray absorptiometry revealed that the body mineral density at the levels of lumbar 1-lumbar 4 was 0.685, Z score was −3.7, and the body fat was 48.7%. The patient’s chromosome examination revealed a normal 46, XY chromosome.
The patient’s father had been abandoned as a child. He received inguinal herniorrhaphy at the ages of 10 and 53. He suffered from hypertension at age 45 and took nifedipine sustained release tablets (20 mg/d) and indapamide (1.5 mg/d) to control his blood pressure. His body mass index was 21.5 kg/m2 and he had a few purple striae on the abdomen. His ACTH was also less than 0.22 pmol/L and 24h urinary-free cortisol was 23.62 nmol. The patient’s mother was healthy and had normal serum ACTH and cortisol levels.
The patient and his father were recommended the insulin tolerance test by intravenous injection of insulin (0.15 U/kg). Serum samples were taken at 0, 15, 30, 60, 90, and 120 minutes. During the test, the patient experienced dizziness, sweating, and weakness 3min after the insulin injection and the blood glucose measured by glucose meter was 1.6 mmol/L. Subsequently, he was intravenously administered 50% glucose (60mL). After 15 min, the blood glucose increased to 12.2 mmol/L. Furthermore, his cortisol level was increased by 49% during the test. The plasma cortisol of the patient’s father increased by 105.9% during the test ().
The function of renin-angiotensin-aldosterone system was also assessed. The blood samples were drawn by venipuncture after 30 minutes of rest in a supine position in the morning, and 2 hours after staying in an upright position with 20mg furosemide intramuscular injection. The aldosterone-to-renin ratio was calculated. The patient’s plasma renin activity was above normal and serum aldosterone was close to the lower limit (). The patient appeared weak and his potassium decreased from 3.5 to 2.9 mmol/L after the injection of furosemide.
RT-PCR was performed to evaluate the mRNA levels of GRα, GRβ, mineralocorticoid receptor (MR), 11β-1-hydroxysteroid dehydrogenase (11β-1-HSD), 11β-2-HSD, hexose-6-phosphate dehydrogenase (H6PD), and 3β-2-HSD in the peripheral blood lymphocytes of the patient and two normal, age-matched men at three different time points. The mRNA levels of MR were found to be lower in our patient. However, the difference of the mRNA levels of other indices between the patient and the normal, age-matched males was not significant ().
The patient was treated with mifepristone 200mg/d for one month, and then 400mg/d for the next eleven months, during which time the patient was instructed to maintain a similar level of physical activity and receive the same calorie intake (25 kcal/kg/day). After one year of treatment with mifepristone (, ), the patient’s body weight decreased from 95 kg to 93 kg. His total body fat decreased from 48.7% to 39.9%. The body mineral density at the levels of lumbar 1-lumbar 4 was increased from 0.685 to 0.702. During the treatment, the patient’s potassium level was always maintained within the normal range. The ACTH and cortisol level remained lower than normal.


A 30-year-old male was hospitalized for massive hematemesis and lightheadedness. He had a history of chronic alcohol-induced pancreatitis. Three weeks before the presentation, he underwent a cystogastrostomy with LAMS (AXIOS; Boston Scientific) placement to treat a 10-cm walled-off necrosis (WON). He presented to an outside facility with hemodynamic instability. Computed tomography (CT) scan did not reveal any acute finding suggestive of bleeding. Esophagogastroduodenoscopy (EGD) showed a large blood clot protruding from the LAMS in the stomach. After multiple attempts to control bleeding were unsuccessful, the patient was transferred to our center for a higher level of care.
On arrival, the patient was tachycardic and hypotensive. Laboratory data showed hemoglobin was 8.2 mg/dL, platelets were 177 000/µL, and international normalized ratio was 1.3. The patient received a blood transfusion. Subsequently, he underwent CT angiography (CTA) of the abdomen. CTA showed a ruptured PA arising from the distal splenic artery. The PA was directly posterior to the LAMS, considered a complication of LAMS (). The patient was sent for mesenteric angiography, and multiple coils were placed in the splenic artery near the PA ( and ). The patient continued to improve without a further drop in hemoglobin and was eventually discharged from the hospital. Subsequently, the LAMS was removed after the near resolution of the WON.


A 3-month-old male Chinchilla cat weighing 0.8 kg was admitted for pain in the right hindlimb. Radiographs revealed an oblique diaphyseal fracture of the right femur. After closed reduction, two Kirschner (K)-wires of 1.5 mm diameter were inserted in a normograde fashion. The inserted K-wires were removed 1 month after the surgery. Several days after removing the implants, the patient was presented with a fracture of the distal metaphysis of the right tibia after falling from a low height. Surgical repair was performed in the same way using two K-wires 1.6 mm in diameter. After the second operation, as additional fractures occurred owing to unknown reasons or suspected slipping events, several additional surgeries were carried out inserting K-wires into intact long bones to aid stress distribution on those bones. K-wires with a diameter of 1.2 to 2.0 mm were used and applied in a normograde fashion after closed reduction ().Blood tests were performed to identify other diseases that could cause pathological fractures. Complete blood count revealed no particular abnormality. Serum total calcium, serum total phosphorus, ionised calcium, parathyroid hormone and 25-hydroxy vitamin D levels were all normal (). As blood urea nitrogen and creatinine levels were within the reference intervals and renal ultrasonography confirmed no specific findings, secondary renal hyperparathyroidism was deemed improbable. Trabecular bone mineral density was measured from the 12th thoracic vertebra () and the fourth lumbar vertebra () using CT (Hi Speed QX/I; GE Medical). Relatively low trabecular bone mineral density was confirmed in the patient (219.8 Hounsfield units [HU] at the 12th thoracic vertebra and 169.46 HU at the fourth lumbar vertebra), compared with normal cats of a similar age. Bone biopsy was performed from the patient’s humerus during surgery and the sample was submitted for histopathological examination. Irregular and thin cortical bone with increased diameter of Haversian canals and replicated cement lines was identified (). Based on these results, OI was tentatively diagnosed.Radiography was performed for follow-up or when the patient showed clinical signs. Follow-up radiographs were taken for 2 years. The patient showed spinous process fractures on several thoracic vertebrae 3 months after the first operation. After 5 months, the patient showed fractures on the ribs. As alignment was unchanged, no additional surgery was performed. Seven months later, a pelvic fracture not disrupting the pelvic cavity was revealed. The patient showed a fracture of the proximal ulna which had the K-wire inserted, luxation of thoracic vertebrae 4–5, changes in the morphology of the thoracic cavity and a fracture of the canine tooth of the right mandible, implying dentinogenesis imperfecta 1 year after surgery. Loosening of an inserted pin often occurred and irritated the adjacent joint and soft tissues, which then required cutting of the pin and/or re-insertion. The last radiographs taken 2 years postoperatively suggested repeated fractures and bone unions (). Follow-up complete blood count and serum chemistry, except parathyroid hormone and 25-hydroxy vitamin D, were performed regularly but failed to reveal any abnormalities. During the 3-year follow-up period, although the patient often showed lameness or ataxia due to fractures and required medical management using tramadol (Tridol 2 mg/kg q12h; Yuhan Corporation) and/or meloxicam (Metacam 0.05 mg/kg q24h; Boehringer Ingelheim), it was able to walk until it died due to undetermined reasons.


A 55-year-old Hispanic lady presented for an opinion regarding decreased visual acuity in her right eye (OD) for 6 months. She had no relevant background and was of Spanish descent. A previous ophthalmic consult had resulted in a presumptive diagnosis of choroidal melanoma and enucleation was suggested. On our examination, best-corrected visual acuity (BCVA) was 20/100 OD and 20/20 in her left eye (OS). Anterior segment examination of OD was normal; however, the fundus examination revealed a 5 × 5 mm subretinal lightly pigmented lesion under the fovea and extending inferiorly. Overlying orange pigment and subretinal fluid were clinically visualized, and later confirmed by fundus photography [] and fundus autofluorescence (FAF) []. The OS examination was unremarkable. Ancillary studies were ordered [Fig. and ]
Enucleation and histopathological analysis Diagnostic incisional biopsy via transvitreal approach Plaque brachytherapy Photodynamic therapy (PDT) with verteporfin.
Fundus photography OD showed the presence of orange pigment over the lesion [, white arrow] with moderate overlying hyperautofluorescence and marked surrounding hyperautofluorescence on FAF []. Optical coherence tomography (OCT) evidenced a dome-shaped mass with intralesional expanded vascular pattern within the choroid and overlying subretinal fluid [, black arrow]. Ocular ultrasound (US) showed an echodense, dome-shaped choroidal mass measuring 2.67 mm in thickness []. Additional workup with indocyanine green (ICG) angiography showed early hypercyanescence of the mass in the pre-arterial phase and diffuse late staining of the lesion. A diagnosis of circumscribed choroidal hemangioma (CCH) was confirmed and PDT was advised.


A 12-year-old girl was referred to the dental department for the assessment of a long-standing nonhealing ulcer on the labial mucosa of the lower lip. The lesion was first observed by the patient 2 years ago; however, the lesion was brought to the notice of the parents 6 months back which prompted them to seek the treatment. The patient had no symptoms otherwise. The size of the ulcer has remained constant over the past 2 years. There was no history of cough, fever, hemoptysis, and weight loss. She did not have any systemic condition, had no history of allergy, and was not on any medications.
She had consulted two dental practitioners in her village and was treated conservatively with the help of topical medications over the past 6 months. The older prescriptions revealed the use of mucopain gel (benzocaine 1.P. 20%), zytee gel (benzalkonium chloride 0.02%, choline salicylate 9%), and candid mouth paint (clotrimazole 1%). However, there was no change (increase or decrease in the size of the ulcer) or improvement in her clinical condition for the past 6 months.
General physical examination revealed that she weighed 33 kg and was 150 cm tall. The calculated body mass index of 14.7 kg/m2 indicated that she was underweight. Vital signs (heart rate – 82 beats/min, respiratory rate – 16 breaths/min, blood pressure – 120/80 mmHg, body temperature – 98.6°F) were within the normal range. Submandibular and submental groups of lymph nodes were examined and they were nonpalpable and nontender.
Oral examination revealed a round nonhealing ulcer on the labial mucosa of the lower lip on the left side measuring 4 cm × 4 cm. The ulcer was grayish-white in color with irregular borders. The edges were thin and undermined with slight induration at the base []. There was no tenderness on palpation. An incisional biopsy was performed under local anesthesia. The microscopy section from the lower lip showed the hyperplastic squamous epithelium, multiple granulomas composed of epithelioid cells and Langhan's giant cells, surrounded by lymphocytes and plasma cells []. No caseous necrosis was seen in the biopsy sample obtained. A preliminary diagnosis of TB/sarcoidosis was made.
Blood tests revealed a normal erythrocyte sedimentation rate (18 mm in the 1st h). The serum angiotensin-converting enzyme level was 58.2 U/L. Interferon-gamma release assay (IGRA) (Quantiferon-TB [QFT]) was positive (3.43 IU/mL). Results of Mantoux test interpreted 48 h after tuberculin injection (10 T.U) revealed erythema of 16 mm and induration of 18 mm. The chest radiograph revealed the absence of foci of infection indicating no pulmonary involvement. Thus, the diagnosis of primary TB was confirmed.
Treatment with anti-tubercular drugs was initiated. The patient was prescribed isoniazid 150 mg, rifampin 300 mg, pyrazinamide 750 mg, and ethambutol 400 mg daily for 2 months. The second phase of the treatment consisted of isoniazid 150 mg and rifampin 300 mg daily for 4 months. Lesion almost resolved within 3 weeks of initiation of the treatment [].


A 14-month-old girl, who had neuroblastoma with bone metastasis, had been treated with neoadjuvant chemotherapy with the Vincristine, Prednisolone, Etoposide and Chlorambucil (OPEC) protocol for two courses. She then underwent complete excision of an abdominal mass and left kidney surgery. Then chemotherapy followed (Topotecan/Endoxan) for 5 cycles. Because iodine-131-meta-iodobenzylguanidine (MIBG) was normal, she was candidate for haematopoietic stem cell transplantation (HSCT) at the age of 24 months. In 2017, she underwent autologous stem cell transplantation. Having good conditions, she was discharged on day 13. On day 28, the patient returned to hospital with symptoms of abdominal distention () and pain, malaise, fever, weight gain, vomiting and low urine output. During the examination, she had abdominal ascites and tender hepatomegaly. She was admitted to the hospital with a possible diagnosis of VOD. Laboratory studies indicated that she had high serum aminotransferases, bilirubin and thrombocytopenia (). Abdominal sonography showed marked hepatomegaly and mild to moderate ascites. Serologic analyses for hepatitis and cytomegalovirus (CMV) yielded negative results. During hospitalization on day 29, the patient developed respiratory distress, low oxygen saturation, tachypnea, and tachycardia. She was transferred to the intensive care unit. She was treated with antibiotics, heparin, Nacetylcysteine, low dose dopamine, albumin and a diuretic. According to serial laboratory tests, the patient's bilirubin was rising. On day 31, because of the severe VOD, treatment with Defibrotide (25mg/kg/day) was commenced. Defibrotide treatment for 21 days was administered. She responded well to treatment and was discharged in a good general condition.


In July 2018, a 45-year-old male patient was admitted the Department of Haematology, Lanzhou University Second Hospital, Lanzhou, Gansu Province, China for further evaluation of acute hepatic failure of unknown origin and hepatosplenomegaly. The patient was treated for acute hepatic failure for 1 month in another hospital, until jaundice, joint pain and pancytopenia developed. There was no history of recent acute infection or rash, no history of traveling, pet exposure and past medical history. Furthermore, the family history was negative for blood disorders and cancer, and there was no consanguinity in his family. Physical examination was notable for jaundice and hepatosplenomegaly, no lymphadenopathy, no spider telangiectasia and no palmar erythema were noted. In addition, serology for hepatitis A, B and C, Epstein-Barr virus, cytomegalovirus and human immunodeficiency virus were negative, and no leukaemia cells were found in the two bone marrow smears prior to referral. The clinical and laboratory tests implemented in the other hospital and in our hospital are shown in .
To determine whether it was HPS and to find out the cause, further examinations were undertaken. Natural killer cell activity was 11.31% (normal value: ≥15.11%) and sCD25 was 7441 pg/ml (normal value: <6400 pg/ml), which were accompanied by fever, hepatosplenomegaly, pancytopenia, hypofibrinogenaemia, hypertriglyceridaemia and hyperferritinaemia, which confirmed the diagnosis of HPS. Bone marrow aspirate and biopsy showed no haemophagocytosis, but hypercellularity with 87% lymphoblasts. Immunophenotyping showed the blasts were positive for CD10, CD19, CD20, HLA-DR and TdT, consistent with B-cell ALL. In addition, because of the high level of anti-nuclear antibody (ANA:1:320) detected in another hospital, a labial gland biopsy was performed to determine whether the patient had Sjogren's syndrome. Histopathological examination combined with immunohistochemical staining also confirmed B-cell-derived lymphoma or leukaemia: positive for CD20, CD10, CD79a and the Ki-67 proliferation index was 80%. The findings suggested that HPS was associated with ALL. Cytogenetic analysis showed t(9;22)(p24; q11.2) translocation in nine of 11 metaphases (), which has only been reported in a few cases. However, neither the janus kinase 2-breakpoint cluster region (JAK2-BCR) fusion transcript nor the BCR-JAK2 fusion transcript was detected.
The patient was treated according to the Oncology Protocol of China (2016) for adults, which included induction chemotherapy with 10 mg dexamethasone intravenous (i.v.) (days 1–28), 1200 mg cyclophosphamide i.v. (day 1 and day 15), 15 mg idarubicin i.v. (days 1–3), 2 mg vincristine i.v. (day 1, day 8, day 15, day 22) and 2500 IU PEG-asparaginase intramuscular (day 3); and intrathecal 15 mg methotrexate, 35 mg cytarabine and 5 mg dexamethasone (day 1, day 15). A bone marrow aspirate on day 15 of induction showed remission of ALL with 2% blasts as well as a low level of minimal residual disease by flow cytometry (0.18%). Clinical and laboratory signs of HPS transiently improved. The laboratory tests after chemotherapy are also shown in . Unfortunately, pancytopenia developed on day 22 after initial diagnosis and the patient died on day 35 because of severe sepsis due to multiple bacterial infections, including pseudomonas aeruginosa, staphylococcus aureus and candida albicans.
This report was approved by the Ethics Committee of Lanzhou University Second Hospital (no. 2019A-223).


A 62-year-old woman complained of persistent nasal discharge with nose bleeding accompanied by right tinnitus for 2 years. A nasopharyngeal mass was found on a nasopharyngeal endoscopic examination at another hospital. Histopathology of the biopsy specimen showed nasopharyngeal malignant melanoma. After taking six cycles of temozolomide at another hospital, the nasopharyngeal mass did not decrease in size. Therefore, conventional segmentation radiotherapy was performed 20 times.
Finally, the patient was admitted to our hospital. A specialist examination showed a mass in the nasopharyngeal cavity with pigmentation on the surface and growth into the right nasal cavity. Enhanced magnetic resonance imaging (MRI) () showed masses in the right lateral wall of the nasopharyngeal cavity, which involved the nasal cavity and was consistent with melanoma. The masses showed high-signal intensity on T1-weighted images and low-signal intensity on T2-weighted images. Non-enhanced computed tomography (CT) () showed an irregular mass in the nasopharyngeal cavity with an unclear boundary with the lateral nasopharynx wall. After excluding distant metastases, such as to the chest and abdomen, the operation plan of partial resection was selected. The operation process was divided into two parts. First, surgery was performed to open the whole group of bilateral sinuses. The tumour was found at the centre of the eustachian tube, and extended forward to the nasal cavity and sinuses, and invaded the ethmoid roof and meningeal tissue. Second, the diseased meningeal tissue was removed and cerebrospinal fluid rhinorrhoea was repaired.
Two weeks later, subtotal resection of the right temporal bone with the Fisch type B posterior auricular approach and of the lateral cranial base lesions was performed in the second stage. Intraoperatively, the lesion was found to be located inferomedially to the cartilaginous portion of the eustachian tube. The cartilage and lesion were removed together up to the foramen lacerum and down to the pharyngeal orifice of the eustachian tube. The ICA was exposed and properly protected. Three days after the second operation, the patient developed sudden massive oral and nasal bleeding with a volume of approximately 300 mL. Therefore, nasal endoscopic probing was performed after endotracheal intubation under general anaesthesia at the site of haemorrhage on the right side of the nasopharynx. Because of continuous bleeding, we performed emergency cerebrovascular angiography and balloon (Cordis 6 × 20 mm; Johnson & Johnson, New Brunswick, NJ, USA) embolization of the ICA. The specific process was as follows. Super selective arteriography of the bilateral common carotid arteries, internal carotid artery, and left vertebral artery was performed, and contrast media leakage was observed in the temporal segment of the right ICA (). Cerebral angiography showed that the anterior communicating artery was open and the bilateral anterior cerebral arteries, middle cerebral arteries, and their branches were well developed. The balloon was placed at the distal and proximal ends of the orifice of the fistula to block the blood vessel. Postoperative angiographic results are shown in . The patient developed transient myocardial ischaemia postoperatively, and 4 days after the treatment, the patient returned to the local hospital for medical treatment. No abnormalities were found on brain CT at discharge. After discharge, the patient did not receive any other comprehensive treatment. At the 6-month follow-up, the patient showed multiple bone and kidney metastases. She died of systemic metastases 1.5 years later.