A 52-year-old-male, with a prior history of non-insulin-dependent diabetes mellitus, underwent sigmoidectomy for a moderately differentiated adenocarcinoma of the sigmoid colon. Pathologic staging revealed a Duke's stage C tumor or C1 according to the Astler-Coller classification. A decision was made to administer adjuvant chemotherapy using the FOLFOX-4 regimen (5-FU, 400 mg/m2 bolus infusion on days 1, 2 followed by 5-FU, 600 mg/m2 as a continuous infusion for 22 hours on days 1, 2, leucovorin 200 mg/m2 as a 2 hour infusion prior to 5-FU on days 1, 2 and L-OHP 85 mg/m2 on day 1, every 2 weeks). He was programmed to receive a total of 6 monthly cycles. Treatment was well tolerated until the 4th cycle, at the beginning of which the patient's hematologic profile and renal function were normal (Hb: 13,8 g/dl, HCT: 43%, WBC: 4,9 × 109cells/L, platelets: 162 × 103 cells/μg, creatinine: 1,1 mg/dl, BUN: 35 mg/dl).
On the first day of therapy, and after L-OHP infusion was completed, the patient noticed urine discoloration and immediate urine analysis demonstrated hematuria with hemoglobinuria. During the next day, the patient's complete blood count exhibited findings consistent with acute hemolysis and thrombocytopenia (Hb: 12 g/dl, Hct: 36%, WBC: 4,78 × 109 cells/L and platelets: 50 × 103 cells/μl). A hemolytic reaction was indicated by elevated LDH and indirect bilirubin indices (1383 U/l and 3,34 mg%, respectively). The patient also exhibited signs of acute anuric renal failure (urea: 77 mg%, serum creatinine: 3,1 mg% and uric acid: 9,5 mg%). Peripheral smear analysis demonstrated fragmented RBCs.
Chemotherapy was immediately discontinued and the patient was managed conservatively with monitored IV hydration and loop diuretics, high dose corticosteroids and fresh frozen plasma (FFP) infusions on a daily basis.
The development of hemolysis was further substantiated with next day's laboratory results. LDH remained elevated at 819 U/l. Coagulation parameters were normal, partial thromboplastin time, fibrinogen and INR were 31,1 sec, 412 mg/dl and 1.05, respectively. D-dimmers were 9,1 μg/ml. The patient was not G6-PD deficient (G6-PD: 11.0 U/g Hb). Direct and indirect agglutination reactions remained negative providing no indication of immune mediated hemolysis. Hemoglobin and hematocrit values reached a minimum of 11.8 g/dl and 34.8% respectively, four days after the infusion of L-OHP. At that point, LDH was 499 U/l and BUN and creatinine were 149 mg/dl and 5,6 mg/dl, respectively.
The patient gradually recovered and the results of successive hematological and biochemical tests confirmed the improvement of his condition. Fifteen days after the onset of hemolysis and thrombocytopenia laboratory values were returning to normal: Hb: 12,1 g/dl, Hct: 38,2%, platelets: 151 × 103 cells/μl, LDH: 255 U/l, creatinine: 1,5 mg/dl, BUN: 78 mg/dl. A month later, all hematologic values and the urine analysis had returned to normal. The patient refused the continuation of chemotherapy. On follow-up, one year after the hemolytic episode, the patient remained disease free and asymptomatic, with no evidence of recurrence of hemolysis or thrombocytopenia. BUN and creatinine were 42 mg/dl and 1,2 mg/dl, respectively, indicating the absence of chronic renal sequelae.


A 32-year-old man presented with a 6-month history of intermittent dysphagia. Barium swallow showed an extrinsic compression of the lower thoracic esophagus. An upper gastrointestinal endoscopy was performed, which revealed a large submucosal mass in the lower thoracic esophagus without mucosal irregularity. Endoscopic ultrasonography (EUS) and computed tomography (CT) of the thorax showed a large homogeneous tumor in the esophageal muscular layer, measuring about 5 × 6 cm, without paraesophageal lymph nodes and projecting into the right pleural cavity (Figure ). Routine laboratory and clinical findings were normal.
The patient was intubated with a double lumen tube for one-lung ventilation and was positioned in the left lateral decubitus position. Three thoracic trocars were introduced (Figure ). The camera port (10-mm) was placed at the ninth intercostal space, mid-axillary line. Two 5-mm trocars were introduced at the fifth intercostal space, anterior axillary line and the seventh intercostal space, posterior axillary line for using a "grasper" or coagulating device. The left lung was retracted to expose the lower thoracic esophagus. A simple hook was used to divide the mediastinal pleura overlying the esophagus. The lesion was then enucleated by careful dissection.
Intraoperative endoscopy with air insufflation was performed to confirm esophageal integrity (Figure ). The esophageal muscle was re-approximated with interrupted 3/0 Dexon. The specimen was removed within an endobag through the camera port. A 28 Fr chest tube was inserted through the camera port for postoperative drainage. The operative time was 2 hours and intraoperative blood loss was minimal.
Barium swallow at day 3 after surgery revealed no leakage and the patient was started on a liquid diet on day 4. The pathological report showed a leiomyoma with mitotic figure 0–1 per10 high power fields (HPF). Immunoperoxidase stainings were positive for smooth mucle actin, and negative for S-100, CD34 and C-kit. The patient was discharged on postoperative day 6. The patient is currently asymptomatic three months after surgery.


The patient is a 63-year-old man who initially presented to an outside hospital for workup of hematuria. The patient was found to have a mass in his left kidney and radiological workup had shown lesions within his liver. The patient came to our hospital seeking treatment from one of our urologists. In order to rule out a urinary bladder carcinoma, a urinary cytology was performed and showed no malignant cells. As part of his preoperative workup, the patient had CT scans of his thorax, abdomen and pelvis. The scans once again showed a large lesion on his kidney (Figure ) and also defined multiple lesions within his liver. All but one appeared to be small simple cysts. A lesion found in segment 4a of the liver showed a circumscribed 1.1 × 1.6 cm hyperattenuating lesion pre-contrast (Figure ) that did not enhance post-contrast. The report indicated suspicion for metastasis or hepatocellular carcinoma. Because of the concern for metastatic disease, the lesion was removed and frozen section was performed intraoperatively. A 1.5 × 1.5 × 0.5 cm tan-pink cyst was examined and diagnosed as an epithelium lined cyst. Surgery was then continued and left nephrectomy was performed for removal of the renal mass. The surgery was without complications and postoperative hospital course was uneventful. On permanent sections, the cyst was microscopically described as composed of ciliated pseudostratified columnar epithelium, subepithelial connective tissue, smooth muscle layer and outer fibrous capsule (Figure and ). A diagnosis of CHFC was given. Grossly, the renal mass consisted of a well-circumscribed nodule with a solid, golden yellow cut surface. Histologically, it was composed of malignant clear cells with a rounded to polygonal shape and abundant clear cytoplasm forming a trabecular and nesting pattern with a prominent delicate vascular network. The final diagnosis was renal cell carcinoma (RCC), clear cell type. Two years after the surgery the patient is free of disease.


A 63-year old woman presented in 1993 with a short history of right iliac fossa pain, localised peritonitis, and an elevated white cell count. She reported a two-year history of intermittent colicky abdominal pain. At operation, a 5 cm ileal tumour was found, situated 10 cm proximal from the ileocaecal valve. This was resected. Histopathological assessment confirmed a carcinoid tumour with locally involved nodes (three positive lymph nodes). Nine years later, she re-presented with right-sided abdominal pain, a two-week history of diarrhoea, and a two month history of episodes of her face getting "hot and blushing". A computed tomography (CT) scan revealed a liver nodule (30 mm diameter) and a mass (60 × 40 mm) in the right iliac fossa, which was in contact with a small intestinal loop (Figure ). Octreotide scanning (indium-111 labelled pentreotide scintigraphy) demonstrated that the liver, the ascending colon, the right iliac fossa, and an area in the superior half abdomen had positive foci (Figure ). In addition, the tumour marker CA-125 was raised (40.5 U/ml)) and so were the urine levels of 5-hydroxyindoleacetic acid (5-HIAA) (41.2 mg/day). Percutaneous biopsy of the liver lesion confirmed a carcinoid tumour.
At operation in January 2003, peritoneal carcinomatosis was discovered with tumoural implants over the greater omentum, the parietal peritoneum, the left ovary, the terminal ileum (with stenotic and fibrous lesion), and a liver metastasis in segment IV. The peritoneal deposits, left ovary and fallopian tube, the involved distal ileum, and the liver metastasis were surgically removed. All deposits were histologically metastases from the original carcinoid tumour, although the distal ileal tumour metastasis only affected the serosal and muscular layers. Following an uneventful postoperative recovery, she commenced six cycles of chemotherapy with Streptozotocine and 5-fluorouracil.
In October 2004, she was admitted with abdominal cramps and vomiting. A CT scan revealed dilated intestinal loops that appeared to be attached to each other and with the anterior abdominal wall. In view of the previous surgery and the history of carcinomatosis, she underwent a further laparotomy. However, at operation, the cause was adhesional obstruction, although some foci were found in the parietal peritoneum and in the Pouch of Douglas. These implants were less than 4 mm in length and were excised. All the biopsies were positive for carcinoid tumour and after an uneventful recovery she was discharged. Further therapy with Somatostatin with the aim to control the progression of the disease and because she reported occasional flushing, achieved a good quality of life. At review in March of 2006, the patient reported some abdominal pain and infrequent flushing, but is otherwise well. A further Octreotide scan revealed no disease activity.


A 20-year-old male medical student presented with a 12-month history of right knee pain of insidious onset. He had been a regular amateur rugby union player for 12 years. No episodes of trauma and no previous knee problems were reported. The problem began as stiffness and pain and had worsened in the last 3 months to the extent that he had to stop playing. The patient described the pain as an intermittent deep ache. The pain was exacerbated by end of range flexion and extension.
Physical examination revealed right-sided posterior knee pain exacerbated by end range flexion and extension and mild effusion. Active range of movement was -15°–95° and passive range of movement -10°–100° (Fig and ). Atrophy of the right quadriceps was present. There was no joint-line tenderness on palpation. Anterior and posterior drawer and Lachmann tests were negative whilst compression rotation test was positive.
Physiotherapy was initially undertaken in order to improve range of movement. At review no improvement at all was seen and compression rotation test was still positive. MRI scans showed an abnormal anterior cruciate ligament (ACL). Its fibres were intact but displaced by material containing high signal on the fat sat sequence, reported as appearing mucoid in nature. There were also areas of focally high signal in relation to its tibial attachment. These areas had the appearance of tiny ganglia. The report presumed that these ganglia extended into and thickened the ACL. The findings were in keeping with mucinous degeneration. There was evidence of intra-osseous cyst formation as shown by high signal at the attachment sites of the ligament (Fig. – arrows). X-ray was unremarkable. Based on these findings an arthroscopy with biopsies was undertaken.
Arthroscopy revealed several small ganglia in the ACL around the tibial insertion. Both menisci were intact and cartilage was normal. The posterior cruciate ligament (PCL) was normal. The histology report from biopsies described ligament with multilocular foci of mucoid degeneration. There was no evidence of neoplasia.
As much as possible of the macroscopically abnormal tissue was excised arthroscopically (Fig , , ). Examination under anaesthesia showed a range of movement of -15°–110° pre-operatively and -5 – 140 degrees post surgery.
Lachmann's test and anterior drawer tests were negative after extensive debridement. Post-operatively the range of movement was improved to 5°–140+° (Fig , , , ). Following surgery the patient was referred for early mobilisation and physiotherapy. At 6 week follow up range of movement was increased to full passive flexion and full passive extension.
The patient remained pain free and returned to rugby 14 weeks after operation. No major trauma occurred during this time. However, instability was described following several games and positive anterior drawer and Lachmann's tests were present on examination. Arthroscopy confirmed the presence of an ACL tear (Fig ) and also showed cartilage and meniscal damage. The patient later had a patellar tendon graft performed.


A 28-year old man presented with a three month history of an asymptomatic scrotal swelling. He had previously undergone orchidopexy in childhood for a mal-descended right testis. There is otherwise no other significant medical or family history. The mid-scrotally located mass measured 4 × 7 cm and was nodular, hard and clinically separate from the testes. It was not attached to the scrotal skin or other underlying structures. Lymphadenopathy was not detected.
Initial ultrasonography (Fig ) showed a large extra-testicular and multinodular mass in the midline of the scrotum. The largest nodule, located behind the right testis was cystic. Both testes appeared normal. Subsequent staging Computer Tomography (CT) of the scrotum, abdomen and chest was performed, which identified a similarly localised heterogeneous mass of 6.9 × 3.7 cm, separate from the testes (Fig ) and did not extend into the tunica vaginalis. There was no evidence of distant or nodal metastases.
An initial diagnostic wedge biopsy indicated the lesion could be an ancient schwannoma. Curative surgical excision with partial scrotectomy was undertaken for removal of the mass and for definitive diagnosis. At resection, the tumour appeared to be superficial to the tunica vaginalis, testes and corpus spongiosum. Inferiorly, the tumour was attached to the bulbospongiosus requiring partial resection of the superficial muscle fibres.
The resected specimen (Fig ) was white and multinodular, with areas of cystic change and haemorrhage. Microscopically (Fig ), proliferation of spindled shaped cells with fibrillary cytoplasm was seen, with dense fibrous bands arranging the cells into nodules. In some cells, marked nuclear hyperchromaticism and atypia were seen. Mitoses were not present. Within the lesion, cellular areas were interspersed with looser myxoid and cystic areas. Blood vessels with thickened hyalinised walls were noted. Staining for S100 protein was positive in the tumour cells. The histological features are that of an ancient schwannoma. The margins of this specimen were ragged and the completeness of excision was difficult to assess.


Patient 1 is a 29-year-old Iranian-Jewish woman diagnosed at age 24 years. She presented at age 17 with inability to abduct her legs against any resistance on an exercise machine; other muscle groups were strong. Following her first pregnancy at age 20, she noticed weakness, difficulty climbing stairs, and tripping and falling that progressed in frequency over the next 3 years from once a month to once a week. A muscle biopsy and genetic testing confirmed the diagnosis of HIBM with homozygous M712T mutations in GNE. At age 25, she noted difficulty fastening snaps and could not raise her arms to pull back her hair. Progression was gradual, with more significant decline following illnesses. Treatments included solumederol, 1 g intravenously for 3 days, which resulted in improved strength but was followed by relapse. On one occasion, IVIG (dose unknown) was given over 3 days, providing no improvement but complicated by aseptic meningitis requiring hospitalization. For the 6 months prior to admission, the patient used a wheelchair and required assistance walking and with transfers. Medications included sertraline for depression and anxiety, and vitamins.
At the NIH Clinical Center, the patient was wheelchair dependent, but could go from sit to stand and ambulate a few feet with support. She had bilateral foot drop and significant weakness of upper and lower extremities, with some sparing of the quadriceps. Muscle atrophy was universally apparent. Cranial nerves and sensation were intact. MRI of the calf showed pronounced fatty involution of the musculature bilaterally, worse proximally. The lateral soleus and posterior tibial muscles were somewhat spared distally. A quadriceps muscle biopsy revealed moderate type II fiber predominance. The muscles were atrophic (type II worse than type I) and demonstrated necrosis, active degeneration, and red rimmed vacuoles, consistent with severe chronic myopathy without inflammation. Imunohistochemical staining for NCAM, a surface glycoprotein reflecting muscle regeneration [,], showed a significant number of positive fibers.


Patient 2 is the 42-year-old brother of patient 1. He was diagnosed with HIBM at 37 years of age, following the diagnosis of his sister. Weakness upon raising his arms overhead first appeared at age 27. At 34, he began tripping and, in the subsequent year, had difficulty climbing stairs and lifting his son. The upper extremities were most affected, but leg weakness was evident by decreased endurance, speed of walking, and getting in and out of a car. He occasionally used his hands to lift his legs. Symptoms were largely stable, but exacerbated by emotional events. He did not receive any treatment for his myopathy, and medications included only multivitamins.
At the NIH Clinical Center, there was wasting of the triceps and the interosseus and thenar muscles of the hand. Contractures of approximately 5–10 degrees were present in the elbows, with decreased range of motion upon supination of the forearm. The patient had no foot drop. Muscles of the shoulder girdle, pinch, grip, and proximal lower extremities exhibited weakness. The gait was normal, and cranial nerves and sensation were intact. MRI of the thigh revealed advanced atrophy with fatty involution in the proximal posterior compartment and adductor magnus muscles bilaterally. The sartorius and rectus femoris muscles were also involved. There was relative sparing of the quadriceps with the exception of the rectus femoris muscles. On biopsy, the quadriceps muscle showed several small vacuolated fibers with a few degenerating fibers, but no inflammation. Immunohistochemical staining was negative for NCAM, indicating negligible damage to individual muscle fibers.


Patient 3 is a 39-year-old woman of Bohemian/Czech/French Canadian ancestry diagnosed with HIBM at the age of 31 years. Following a year of progressive tripping and falling, she awoke one day with numbness from the waist down that spread to her entire body. She was diagnosed with mild multiple sclerosis, based on the presence of oligoclonal bands in her cerebrospinal fluid and a brain MRI showing a possible "spot." Muscle biopsy also provided evidence of inclusion body myopathy. Currently, she has difficulty arising from a supine position due to weak abdominal muscles, weakness in her hands, arms, and shoulders, and inability to climb stairs. She wears ankle braces to walk. She uses bronchodilators for asthma and sertraline for depression. Confirmatory mutational analysis demonstrated compound heterozygous GNE mutations, i.e., R246Q and A631V, affecting the epimerase and kinase domains, respectively.
At the NIH Clinical Center, the patient had bilateral foot drop with a compensated gait. She was able to transfer independently from sit to stand and from stand to sit. There was proximal muscle and neck weakness and limited endurance. Cranial nerves and sensation were intact. MRI of the calf showed end-stage myopathy with advanced fatty involution, but with preservation of muscle volume. Signal alteration was present in areas of non-atrophic muscle. The popliteal muscles in the upper calves and the distal posterior tibial muscles were relatively spared. On biopsy, the quadriceps muscle showed significant necrosis with replacement of muscle by fat and connective tissue, but without inflammation. NCAM staining was minimal.


Patient 4 is the 46-year-old brother of patient 3. He was diagnosed with HIBM at the age of 33 years following a one-year history of progressive tripping and falling, decreased muscle strength and inability to lift his foot. As a cabinetmaker, he relied on this action to support the cabinets as he made them. A muscle biopsy was initially read as consistent with either polymyositis or inclusion body myopathy, but intravenous steroids provided no relief, and HIBM was diagnosed. Plateaus were interspersed with declines, which occurred with lack of exercise but not with illnesses. The lower extremity weakness extended throughout the body. The patient currently wears ankle braces to walk, uses an electric wheelchair for distances, and has assistive devices to drive. He has difficulty holding a pen. Medications include acyclovir, Metamucil, multivitamins, tramadol and diazepam.
At the NIH Clinical Center, there was muscle wasting of the triceps and the interosseus muscles of the hands, along with tightness of the heel cords, hips, and hamstrings. The patient was partially independent when going from sit to stand, and when executing transfers to and from his wheelchair. He could ambulate a few steps using ankle-foot orthotics and support from the wall. He had significant weakness in the proximal upper extremities, triceps, wrist and hand, and decreased strength on hip flexion, hip extension, ankle dorsiflexion, and plantar flexion. Deep tendon reflexes could not be elicited in the upper or lower extremities. Sensation and cranial nerve functions were intact. MRI of the calf showed end-stage myopathy with advanced fatty involution but preservation of muscle contours. Signal alteration occurred in areas of the remaining muscle. There was relative sparing of the popliteus, the tibialis posterior, and the flexor digitorum longus muscles. Quadriceps muscle histology revealed vacuolated and moth-eaten muscle fibers, with degeneration and regeneration. Connective tissue was slightly increased with no signs of inflammation. Immunohistochemical staining with NCAM was negative.


In August 2003, a 20-year-old 16-week pregnant mother of one, presented to the orthopaedic outpatient clinic with a 2-month history of a slowly growing painless mass located in the right hypothenar eminence. She was right-hand dominant and a housewife. There was no history of trauma, insect bites or drainage from the area. There was no significant past medical or surgical history. She was not on any prescribed medication. There was no family history of tumours. She denied smoking, alcohol and drug abuse. On examination, a non-tender, firm to palpation, well-demarcated and tethered to the subcutaneous tissues mass, surrounded by mild erythema, and measuring approximately 2.5 cm × 3.0 cm was revealed, in the absence of palpable lymph nodes at the ipsilateral elbow or axilla. There were no other abnormal signs.
Haematological and biochemical investigations were within the normal range. A plain X-ray of the right hand and forearm was unremarkable. Magnetic resonance imaging showed the mass to have infiltrated all skin layers and to have encircled the ulnar artery without involvement of the underlying bones (Figure ). The absence of a signal void from within the mass excluded a diagnosis of haemangioma. Ultrasound-guided needle biopsy showed lesional areas consistent with collagenous fibrous tissue of varying cellularity, with little evidence of mitotic activity. Immunohistochemistry showed focal positivity for smooth muscle actin (SMA), suggesting myofibroblastic differentiation. Histological appearances were in keeping with fibromatosis.
Three weeks after initial presentation, the lesion was excised preserving the ulnar nerve and artery. Histology showed a multi-nodular, well-circumscribed uniform spindle cell lesion with varying cellularity and fibrosis. In one section, central cyst formation was observed. Occasional mitoses of normal morphology were seen. Necrosis was not a feature. In several areas, the lesion appeared to be intravascular, the vessel was lined by an apparent endothelium (Figure ). Scattered osteoclast-like multi-nucleate giant cells were also observed. Immunohistochemistry replicated diffuse positivity for SMA, but not for desmin, CD34 or S100 protein. Appearances were consistent with a myofibroblastic proliferation, confirming a diagnosis of intravascular fasciitis (IVF).
The patient had an uneventful recovery and required no further therapy. She continues to be evaluated for local recurrence every 6 months, with no sign of recurrence at the 2-year follow-up.


A 57 year old male presented with unstable angina. Risk factors included hypertension and hypercholesterolaemia. Significant past history included multiple episodes of deep vein thrombosis and pulmonary embolism. Coronary angiography demonstrated a single coronary artery arising from left coronary sinus (fig ) which divided into a normal sized left anterior descending artery (LAD) and a circumflex (Cx) artery. LAD had a 99% stenotic lesion beyond the first septal and was a good sized vessel going just beyond the left ventricular apex (Fig ). From its proximal segment, beyond the origin of first diagonal and prior to the origin of first septal artery, arose 3 right ventricular branches, the largest of which crossed the right ventricular outflow tract (RVOT), 2 centimetres below the pulmonary valve, to gain the anterior right atrioventricular groove (Fig , ), descended in the groove to anastomose with the distal right coronary artery (Fig ), which arose as a continuity of the circumflex artery (Fig ), as described below. The second right ventricular branch crossed the RVOT below the first but petered out well before it could gain the anterior right AV groove. The third branch supplied the right ventricle and followed a course close to the LAD (fig ). The diagonal artery was a large bifurcating artery with significant proximal stenosis. The circumflex artery had severe stenosis proximally after which it gave a large obtuse marginal branch and then the PDA in the posterior interventricular groove and thereafter continued in the AV groove as the right coronary artery (Fig ). This right coronary artery then gave off a ventricular branch to the inferior surface of the right ventricle and thereafter anastomosed with the proximal RCA arising anomalously from the LAD as described above (Fig ). There was no stenotic lesion in this composite, anomalously arising proximal and distal right coronary arterial system.
Patient was taken to theatre for urgent CABG. Operative findings confirmed the following: Proximal RCA was arising as a branch from the proximal LAD after the first diagonal, crossed the RVOT and gained the anterior aspect of the right AV groove to anastomose with the distal RCA which arose as a continuity of circumflex artery. LAD gave off two further branches to supply the right ventricle (Fig ). The distal circumflex continued to the crux of the heart, gave off PDA in the posterior interventricular groove (Fig ) and thereafter continued as RCA till just above the acute margin of right ventricle. This distal RCA also gave off a right ventricular branch to the inferior right ventricular surface below the acute margin of the heart (Fig ). Employing cardiopulmonary bypass, with antegrade cold blood cardioplegic arrest, quadruple coronary artery bypass grafting was performed. Left internal mammary artery (LIMA) graft was anastomosed to LAD and separate saphenous vein bypass grafts were constructed to Dx, OM Cx and PDA from Cx. Bypass was discontinued easily in sinus rhythm, without ionotropes and patient transferred to ICU in a satisfactory haemodynamic condition. He was transferred to ward on first postoperative day and home 8 days after surgery.


A non-smoking 77-year old gentleman of Indian origin was admitted with a 4-month history of intermittent night sweats, haemoptysis and 6 kg of weight loss. He had no past history of Mycobacterium tuberculosis infection but suffered from significant ischaemic heart disease (IHD). There was no history of diabetes mellitus and random blood glucose levels were normal. Testing for human immunodeficiency virus (HIV) was not undertaken as the patient was not felt to be at risk for HIV infection. Clinical examination was unremarkable apart from low grade pyrexia. He was retired and had recently arrived in the United Kingdom from India.
Routine laboratory investigations were normal apart from an elevated CRP at 22.1 mg/L. Chest x-ray revealed bilateral hilar lymphadenopathy and a mass in the right lower zone. CT scan of thorax demonstrated a 2.5 cm mass in the right middle lobe with multiple small nodules within the right lung and confirmed the presence of mediastinal and hilar lymph nodes.
Fibreoptic bronchoscopy demonstrated a distorted right main bronchus, anterior shift of the right upper lobe and occlusion of the right middle lobe bronchus with black necrotic material (See Figure ). Acid and alcohol fast bacilli (AAFB) were visible on microscopy in the bronchoalveolar lavage (BAL) fluid and were subsequently identified as M. tuberculosis. Histological examination of endobronchial biopsies taken from the necrotic material showed numerous fungal hyphae with a morphological appearance of zygomycetes within necrotic areas of tissue. Fungal cultures were negative; therefore anti-fungal sensitivity testing could not be performed.
Medical management was instigated as the patient's pre-existing IHD did not permit surgical intervention. Intravenous liposomal amphotericin (Ambisome, Gilead) at a dose of 3 mg/kg and standard four drug anti-mycobacterial regimen consisting of rifampicin, isoniazid, pyrazinamide and ethambutol was commenced. Following three weeks of therapy the intravenous liposomal amphotericin was changed to oral itraconazole (Sporanox, Janssen-Cilag) 200 mg once daily, which was increased to 200 mg twice daily following low therapeutic monitoring. Subsequent itraconazole levels were within the therapeutic range.
The patient completed 6 months of oral anti-fungal treatment. Due to concerns on a follow-up CT scan regarding lack of resolution of the multiple nodules 18 months of anti- mycobacterial chemotherapy was administered. Subsequently CT imaging following completion of anti-mycobacterial chemotherapy demonstrated improvement of the mass and a resolution of the associated nodules. The patient has been followed for 6 months to date and there has been no recurrence of symptoms. Recent BAL cultures have been negative for M. tuberculosis and zygomycetes.


The patient was a 76-year-old man. In November 1999, he underwent three- vessel CABG. A postero-lateral branch of the coronary artery was grafted by a pedicled RGEA. In July 2004, he presented to a private hospital because of tarry stool 5 years after CABG. By gastric fiberscopic examination, he was diagnosed to have an early gastric cancer (IIc) located in the lower anterior area of the stomach and was transferred to our hospital for gastrectomy in October. Preoperative angiography showed that the RGEA graft remained well patent (Figure ).
After an arterial catheter was inserted in the right femoral artery for an artificial circulation in preparation for a possible graft injury, an epigastric median incision was made. The RGEA was easily recognized on the left lobe of the liver accompanied by a vein and covered with soft tissues (Figure ). Papaverine hydrochloride was sprinkled around the RGEA to prevent vessel spasm. Its adhesion to the posterior aspect of the lesser curvature of the stomach was dissected. We dissected around the No.6 lymph node station as far as we could do safely and easily. Intraoperative frozen section examination showed no lymph node metastasis around the graft; therefore, complete dissection of lymph nodes at the base of the graft was not undertaken. A distal gastrectomy with lymph node dissection was performed. A Roux-en-Y procedure was used for reconstruction. The pathological diagnosis was poorly differentiated adenocarcinoma (por1>tub2), (18 × 11 mm), sm2, n0 and clinical stage was determined as Stage Ia according to the General Rules for the Gastric Cancer Study []. No complications occurred during the operation. The patient is currently alive without any signs of recurrence 18 months after the operation.


The patient was a 64-year-old man. In November 2005, he underwent three-vessel CABG. The posterior descending branch of the coronary artery was grafted by a skeletonized RGEA. In February 2006, he presented to a private hospital because of epigastric pain. By gastric fiberscopic examination, he was diagnosed to have an early gastric cancer (IIa + IIb) located at the angle of the stomach and was transferred to our hospital for gastrectomy in April. Preoperative angiography confirmed the patency of the RGEA graft (Figure ).
After an arterial catheter was inserted in the right femoral artery for an artificial circulation to be used in case of a graft injury, an epigastric median incision starting below the umbilicus was made with care because the RGEA took an anterior route. The RGEA was covered with the surrounding tissues involving the wall of the stomach and the liver, making it difficult to be found (Figure ). Papaverine hydrochloride was sprinkled around the RGEA to prevent vessel spasm. Eventually, it was recognized by its pulsation on the duodenum. Dissection around No.6 lymph node was carried out to the extent that was deemed safe. Since intraoperative frozen section examination revealed no lymph node metastasis around the graft, complete dissection of lymph nodes at the base of the graft was not undertaken. A distal gastrectomy with lymph node dissection was performed. A Roux-en-Y procedure was used for reconstruction. The pathological diagnosis was signet ring cell carcinoma (23 × 20 mm), m, n0 and clinical stage was determined as Stage Ia according to the General Rules for the Gastric Cancer Study []. There was no intraoperative complication. The patient is currently alive without any signs of recurrence 4 months after the operation.


A healthy 17-year-old Caucasian male weighing 70 Kilograms was infected with Absidia Corymbifera after suffering major abdominal injuries in an accident with agricultural machinery. The patient was admitted to hospital with multiple abdominal muscle lacerations and soil contamination of the abdominal wall, haemorrhage from the right internal iliac artery, multiple vein lacerations, contusions and obstruction of the right external iliac artery at the iliac-femoral junction, superficial femoral vein laceration, ruptured intestine and colon, fractured pelvis with bone detachment, right retroperitoneal hematoma and haemorrhagic shock.
In an 8-hour emergency operation surgeons constructed an external iliac-right common femoral artery bypass, inserted ligature of the right hypogastric artery, sutured iliac-femoral vein lacerations, resectioned the small intestine and left colon and sutured lacerations in the rectum, right abdomen and psoas. During surgery the patient received transfusions of 11 units of compacted red blood cells, 9 units of plasma, 10 units of platelets and 9 litres of hemagel.
After surgery the patient was admitted to the Intensive Care Unit (ICU) because of marked hypothermia and low blood pressure. Blood tests showed: white blood cells 10 × 109/L; haemoglobin12.4 g/dl; platelets: 124 × 109/L; prothrombin: 55%; partial thromboplastin time: 39.7 sec; International Normalized Ratio = 2; fibrinogen 195 mg/dl. Blood chemistry indicated nitrogen 33 mg/dl; creatinine 11 mg/L; creatinine clearance 100 ml/min; proteins 3.9 g/dl; albumin 1.7 g/dl; aspartate aminotransferase 101 UI/L; alamine aminotransferase 36 UI/L; creatine phosphokinase (CPK) 8436 UI/L; lactic dehydrogenase (LDH) 930 UI/L; myoglobin >500 ng/ml; normal electrolyte balance; glucose 90 mg/dl; lactate 43.1 mg/dl (normal range 5.7–22). The patient was HIV negative. As drainage fluid was positive for Enterococcus Faecium, Providentia Rettgeri, Hafnia Alvei and Candida Albicans, tecoplanin (6 mg/Kg i.v. once daily), metronidazole (7.5 mg/Kg i.v. daily 4 times daily) imipenem (10 mg/Kg i.v. thrice daily) and flucanozole (8 mg/Kg i.v. once daily) were administered. Twelve hours after admission to the ICU he developed a crush syndrome with acute renal failure requiring immediate extra-corporeal dialysis. Dosages of all drugs were adjusted to renal function.
Severe acute renal failure was partially resolved. No urination; creatinine 57 mg/L; clearance 4.30 ml/min; CPK 95700 IU/L (normal value < 190 UI/L); myoglobulin >500 UI/L; LDH 4733 IU/L. Potassium 7.1 mEq/L. Four days later extracorporeal dialysis was stopped.
The patient underwent splenectomy because of spleen hematoma with peritoneal bleeding. 800 ml blood were removed.
Surgical wound secretion resulted positive for A corymbifera, E. faecium, P Rettgeri, H. alvei and C. albicans. Wound secretion and CVC blood cultures were positive for C. albicans. A. corymbifera positivity was considered accidental contamination due to residual soil traces.
Although the CVC had been changed very high fever persisted. The patient was affected by vomiting and confusion. Discharge continued from the large, hyperemic, foul-smelling, abdominal wound. Surgeons drained abcess fluid from the right abdominal wall. Cultures of abcess fluid and drainage, as well as wound secretion smears, were positive for A. corymbifera (figs , and ). Growth was observed in mycetes medium and confirmed in Saburaud agar where greyish-white, woolly, flock-like colonies were observed 48 hours later. An antimicrogrammme indicated resistance to 5-fluorocytosine, flucanozole and voriconazole and response to amphotericin B with a low antimicrogramme index (0.016 μg/ml). Posaconazole was not tested as it was not available the hospital pharmacy. Flucanozole was suspended and liposomal amphotericin (Ambisome) started (5 mg/Kg i.v. for 40 days). Therapy was well tolerated.
The patient started to improve and fever gradually dropped.
Tampons and wound secretion smears were negative. Blood cultures for C. albicans were also negative. No other fungal infections developed.
Drainage fluid was positive for E. faecium and Pseudomonas Aeroginosa. As E. faecium was resistant to tecoplanin and vancomycin, linezolid (10 mg/Kg i.v. twice daily) and rifampycin (15 mg/Kg i. v. twice daily) were administered. P. Aeroginosa responded only to meropenem (13 mg/Kg i.v. thrice daily) and ciprofloxacin (6.5 mg/Kg i.v. thrice daily). This combined antibiotic therapy was continued until discharge from the ICU.
Abdominal computerized tomography visualized an area of 12 × 5.5 cm fluid accumulation around the iliac-femoral bypass. Abcess was ruled out when scintigraphy of autologous radio-labelled leucocytes showed no tracer uptake.
Given the risks of further surgery, the relatively complex surgical technique that would be required, the patient's 25% loss of body weight despite parenteral nutrition with 2,500 calories daily and negative scintigraphy, it was decided only to drain the lesion. Drainage fluid cultures were negative for bacteria and fungi, confirming the validity of therapy. Fluid accumulation gradually disappeared with prolonged antibiotic and antifungal therapy. (Figure shows the patient's clinical course). After 75 days in the ICU the patient was transferred to the Infectious Diseases Unit where he continued anti-infectious treatment with liposomal amphotericin B, linezolid and ciprofloxacin for another two months. One year after the accident he is in good health, with normal quality of life.


An eighteen year-old Asian female presented to the ophthalmology clinic of the McGill University Health Center with a three-month history of fluctuating swelling and ptosis of the left upper eyelid. Mild discomfort was felt whenever the swelling was more intense. A well-defined, soft lesion in the left upper eyelid could be palpated, just below the superior orbital rim, without associated inflammatory signs. No decrease in visual acuity or alterations of extraocular movements was found. Intraocular pressure was 17 mmHg OD and 20 mmHg OS. Computed tomography disclosed a distinct homogeneous lesion in the left superior orbit, molding to the globe and other orbital structures (Fig. ). There was no bone erosion. The findings favored the diagnosis of a lymphoid lesion and a transpalpebral biopsy was indicated and performed.
Histopathological evaluation revealed the presence of structures resembling lymphoid follicles surrounded by loose connective tissue (Fig. ). At higher magnification, those structures were composed of numerous blood vessels lined by plump endothelial cells with oval nuclei protruding into the lumen (Fig. ). Surrounding the vessels, there was a chronic inflammatory infiltrate composed of lymphocytes, plasma cells and a large proportion of eosinophils. Immunohistochemical studies, performed on paraffin-embedded tissue, showed the following: Factor VIII underscored the marked vascularity of the lesion (Fig. ), highlighting atypical vascular lining with "epithelioid" or "histiocytoid" cells (Fig. ). Whole body gallium scan failed to reveal lymph node involvement elsewhere. Blood counts and urinalysis were normal. Based on clinical and histopathological findings, the diagnosis of EH was made.


A 41-year-old non-smoking woman presented at a district general hospital with generalised weakness, breathlessness and non-productive cough for 5–7 weeks. Chest x-ray revealed a moderate right-sided pleural effusion, which was drained with a pigtail catheter. The drained fluid was hemorrhagic and fluid cytology was negative for malignant cells. The patient then became generally unwell, pyrexial and anaemic with increasing effusion on serial chest films. A C.T. scan of the chest [Figure ] showed right-sided pleural effusion along with compressive atelectasis in the right lower lobe without thickening of visceral or parietal pleura. Serial pleural fluid cultures were negative. Due to continuing diagnostic uncertainty and falling haemoglobin levels, the patient was referred to our thoracic surgery unit. On admission to our unit laboratory analysis revealed haemoglobin 7.0 g/dl, WBC 7000/mm3, and ESR 15 mm/hr. A diagnosis of chronic haemothorax was made and standard right thoracotomy was performed. After mobilising the right lower lobe from diaphragm a vaguely defined mass was identified lying between the diaphragmatic surface of the right lower lobe and right hemi diaphragm. This mass was approximately 10 × 10 cms in size, poorly circumscribed and contained blood clots, altered blood, fibrous tissue, and gelatinous debris. After removal of the mass the right lower lobe expanded well, and no other lesions were found in the right lung and parietal pleura. The patient recovered well from the operation with no recurrence of pleural effusion.
However, 13 months later, the patient presented again with an increasing right-sided pleural effusion. A CT scan revealed a soft tissue mass in right costophrenic recess. This was thought to be due to recurrence of the initial tumour and right thoracotomy was performed. A poorly circumscribed gelatinous tumour measuring 5 × 7 cms was present in the right costophrenic angle involving adjacent diaphragm, pericardium and visceral pleura of the right lower lobe. A right lower lobectomy was performed along with resection of right hemi diaphragm and involved pericardium. The defects were repaired with a patch. One year later, the patient was asymptomatic and CT-scan revealed no tumour recurrence.


The patient is a 60 year old white male who had Crohn's disease diagnosed in the 1960's. He had small bowel resections in 1965 and 1969. He developed recurrent stone disease in the early 1980's. In 1999 24 hour urine studies showed an elevated oxalate excretion of 1.29 mmol (normal <0.50 mmol) and low magnesium, citrate, and calcium excretion. His serum creatinine was 2.0 mg% in 1995 and 2.5 mg% in Nov. 2001. In June, 2002 he presented with acute renal failure secondary to obstructive stone disease with a serum creatinine of 12.7 mg%. With correction of the obstruction his renal function only modestly improved and he was placed on hemodialysis on 7/29/02. His dialysis course was complicated by multiple blood access problems and several episodes of life-threatening sepsis. As a result he underwent deceased donor renal transplant on 3/9/04. Induction therapy consisted of basiliximab, mycophenolate mofetil, and steroids and then maintenance therapy with tacrolimus, sirolimus, and steroids was instituted. The patient did not undergo intensive hemodialysis either prior to or after transplantation. He did have an acute rejection episode. The biopsy showed Banff 1b acute rejection, but no evidence of oxalate deposition. 24 hour urinary oxalate excretion was elevated at 113.3 mg (normal = 3.6–38 mg/24 hr). His rejection episode was treated with antithymocyte globulin (rabbit) with an excellent response. He was discharged on calcium with meals, Urocit-K, a low oxalate diet, and pyridoxine. His post-hospital course has been uneventful and his serum creatinine is 1.5 mg% almost three years later.


A 65-year-old man was referred to our hospital in October 2006 with a huge abdominal tumor. A firm mass was palpated extending from the epigastrium to the left hypogastrium. There were no laboratory abnormalities, except a slight elevation of the total bilirubin (1.3 mg/dl) and lactate dehydrogenase (LDH: 217 IU/L) levels in the serum. The tumor markers carcinoembryonic antigen (CEA) and carbohydrate antigen (CA19-9) were within the normal range. Ultrasonography showed that the mass occupied almost the entire upper abdomen anterior to the bowel loops. On computed tomography (CT), a mass behind the left hepatic lobe showed heterogeneous low density with faint enhancement (Fig. ). Abdominal angiography revealed that the tumor was vascularized mainly from the right epigastric artery (Fig. ). We suspected liposarcoma, leiomyosarcoma, mesothelioma, or gastric GIST. At laparotomy on October 2006, a well-encapsulated tumor was found in the greater omentum. There was no adhesion to adjacent organs and structures but a pinpoint adhesion to the stomach. The right gastroepiploic artery and vein were prominent and stretched by the tumor, and a major supply vessel diverged from it in one stalk (Fig. ). There was no evidence of metastasis in the abdominal cavity. Grossly, a well-demarcated reddish-gray solid tumor, 20 × 17 × 6 cm in size, showed irregular modularity (Fig ). The cut surfaces were tan-colored and contained focally necrotic areas and a cystic nodule. Histopathologically, the tumor was composed of proliferating epithelioid cells and myxoid cells with an interlacing bundle pattern (Fig. &). The cellularity was relatively high and the frequency of mitotic figures was 2 of 50 high power fields (HPF). The MIB-1 index was 4.4% (Fig. ). The tumor cells were diffusely immunoreactive for myeloid stem cell antigen (CD34), weakly or focally positive for c-kit proto-oncogene protein product (CD117) (Fig. ) and slightly positive for neuron-specific enolase (NSE). However, there was no staining for cytokeratin (CK), alpha-smooth muscle actin (SMA) or S-100 protein. Direct sequencing demonstrated mutations in the platelet-derived growth factor alpha (PDGFRA) gene exon 12, codon 561, encoding a thymine to adenine substitution (Fig. ). These findings were consistent with a myxoid epithelioid GIST lacking myogenic features and neural attributes. The patient had a complete tumor resection and an uneventful postoperative course. He was treated by per os administration of Glevec® 300 mg/day as an adjuvant postoperative molecular targeting chemotherapy and has been living disease-free for 6 months.


An 80 year old female patient was admitted as an emergency with abdominal pain, distension and constipation. Her past medical history included mild hypertension. On admission she was afebrile, with a BP of 146/79, and pulse of 110. On examination of the abdomen she had distension with lower abdominal tenderness. Abdominal XR showed dilated small and large bowel loops. The WCC (White Cell Count) was raised at 12.6, with CRP of 54 & bilirubin of 34. A CT scan of the abdomen showed large bowel obstruction thought to be secondary to a sigmoid carcinoma. A solid mass in the right adnexa was also noted and thought to be either a primary ovarian tumour or metastatic deposit. No distant disease was seen.
After a period of resuscitation, a laparotomy was performed. The large bowel obstruction was found to be secondary to a "sigmoid tumour". The uterus and right ovary were abnormal and were "fixed" to the upper rectum distal to the tumour. The appendix was noted to be abnormal with palpable ileocaecal lymph nodes. A low Hartman's was performed with en-bloc total hysterectomy and bilateral salpigo-oophorectomy. A separate Ileocaecal resection with end ileostomy was also performed. Postoperatively the patient made an uneventful recovery and she was discharged home two weeks later.
Macroscopic examination of the specimen showed an appendix measuring 40 mm in length and up to 15 mm in diameter. Microscopic examination showed invasive high grade adenocarcinoma of appendiceal origin showing diffuse infiltration of the wall of the appendix and the caecum with serosal extension. Metastatic tumour was seen in 2 out of 9 ileocaecal lymph nodes. Distant metastatic carcinoma was also shown to involve the wall of the distal sigmoid colon with stricture formation and infiltration of the adherent uterus, cervix, both ovaries and right fallopian tube.
Immunohistochemistry showed that in all areas of tumour infiltration identical staining was identified. There was strong CK20 positivity, patchy chromogranin positivity and negative staining for CK7. There was similarity in appearance to a malignant goblet cell carcinoid although the tumour was classified as a high-grade aggressive adenocarcinoma of the appendix with neuroendocrine differentiation (see figure ).
The patient was started on Chemotherapy, but 2 weeks into her treatment she declined any further Chemotherapy as she felt very unwell with it
Two years postoperatively the patient remains well with a clear surveillance CT scan.
In our review of the literature, some other unusual presentations of appendiceal tumours have also been reported, they included an appendiceal adenocarcinoma presenting as a vesical fistula [], neck mass and vaginal bleeding [], spontaneous skin fistula [], caeco-colic intussusception [], disseminated ovarian carcinoma []. There was also a reported case of adenocarcinoma of the appendix masquerading as a bladder tumour [], a uterine tumour [], and a case presenting as an inguinal hernia [].


A 36 year-old pre-menopausal Asian woman previously in excellent health, presented with a three-month history of a progressively worsening tender right breast lump, with associated ulceration, induration, and erythema, draining serosanguinous fluid. She denied systemic symptoms, as well as any known recent fungal or tuberculosis exposure. The patient is a G2 P2002 who nursed her children without difficulty. She has had no history of breast disease, and her past medical and surgical histories are otherwise unremarkable. She denied tobacco use, any family history of breast diseases, use of any medications including oral contraceptives, or any known drug allergies. Physical examination was otherwise unremarkable with no palpable lymphadenopathy.
During initial presentation to an outside hospital, mammography and breast ultrasound revealed an increased density and ductal dilatation of the right breast with associated edematous changes. All laboratory and other radiological studies were otherwise normal. Although malignancy had not been excluded, the patient received a presumptive diagnosis of mastitis and was treated with a 7-day course of amoxicillin and cefadroxil without improvement. This was followed by a course of cefdinir and gatifloxacin, which was complicated by an antibiotic-induced erythema nodosum. As a result, antibiotics were discontinued and the patient was scheduled for an open breast biopsy. Interestingly, the patient's symptoms spontaneously resolved before tissue sampling was performed.
Two months after her initial presentation, a second similar lump appeared in the right breast (Figure ). A repeat ultrasound of this area again demonstrated dilated ducts and debris. Bilateral breast magnetic resonance imaging (MRI) revealed regionally dilated ducts with prominent regional ductal and parenchymal plateau enhancement. Given these findings were not typical for mastitis, and clinical and radiological data could not exclude breast carcinoma, tissue sampling was performed. Microscopic examination revealed chronic inflammation and macrophage, giant histiocyte and epithelioid-like cellular infiltration, with cytologic features suggestive of a granulomatous process (Figure ). Further histopathological analysis showed no evidence of carcinoma or abscess formation, and all cultures and stains for infectious organisms remained negative. Secondary to exclusion of malignancy, infection, and other causes, the patient was given the presumptive diagnosis of idiopathic granulomatous mastitis (IGM). She was treated with a 6-month tapered course of prednisone with an excellent response, and has since remained free from recurrence.


A 62-year-old woman was referred to the Department of Surgical Sciences, University of Insubria, Varese, for left flank pain. Physical examination was unremarkable. Complete blood cell count, electrolytes, eosinophil count, serum biochemistry and urinalysis were within normal limits. Plain radiographs of the abdomen showed a small round opacity, about 3-cm in diameter, in the left upper region of the abdomen, with evidence of calcifications. Chest X-ray was normal. The patient underwent ultrasound examination (US) that showed a calcified heterogeneous cystic mass between the superior left kidney pole and spleen in the retroperitoneal area. CT scanning demonstrated the presence of a solitary cystic mass with a calcified wall within the left adrenal gland with no enhancement after iv contrast media injection. No other intra-abdominal or intra-thoracic masses were found, either in the liver, peritoneum or lung. MRI T2-weighted imaging showed a hypointensive lesion with a calcified wall, and a peripheral hyperintensive nodule (Figure , ). CT and MRI findings suggested adrenal echinococcosis. The specific serology for immunoglobulin anti-E. granulosus resulted positive 1:61 (n.v. < 50). Urinary catecholamines and metanephrine levels were within normal limits. The patient confirmed that she had had animal contact. The albendazole preoperative therapy resulted in the disappareance of pain. The diagnosis was confirmed by surgery. The cyst was removed by a transabdominal laparoscopic approach. The exploration of the rest of the peritoneal cavity did not reveal any other lesions. The area around the cyst was carefully packed with gauze soaked in 20% hypertonic saline solution. The cyst was not drained nor aspirated during the procedure. An appropriate dissection plane between the cyst and the adrenal gland could not be found as it was hard and adhesive, so both the cyst and the left adrenal gland were endoscopically removed. Pathological examination of the specimen confirmed that it was a hydatid cyst of the adrenal gland. Macroscopic pathologic examination showed a circumscribed unilocular cystic lesion 3-cm in diameter with a thick fibrous wall of 2-mm. It weighed about 57 grams. On palpation there were stone hard areas of calcification within the cyst wall. Examination of the contents of the cyst showed the typical clear fluid of a hydatid cyst with sand pasty material and calcified bodies. Moreover necrotic material with protoscolices was present (Figure ). No protein was detected and the glucose level was 72 mg/dl. The patient's postoperative course was uneventful. The patient was discharged on the third postoperative day. Therapy with albendazole was administrated for the 4 weeks following surgery (30 mg/kg/day orally). Six months later, the patient is well and in good condition with no further symptoms.


A 35-year-old female with a history of intractable hypertension (for the duration of a year), probably renovascular, was referred by her nephrologist to our department for SRA. She was a smoker. The patient denied any family history of hypertension. Her physical examination revealed a blood pressure of 150/100 mmHg (multiple readings taken from both arms on different occasions were similar). Her cardiovascular, respiratory, and central nervous system examinations were unremarkable. No evidence of retinopathy on fundus examination. There was no carotid, abdominal or femoral arterial bruits. ECG, chest radiograph and transthoracic echocardiography were normal. Her blood urea nitrogen (BUN) and serum creatine were within normal limits. The patient received 3 antihypertensive medications including a beta-blocker, diuretic and a calcium channel blocker. SRA was performed and revealed a single renal artery on the right and two renal arteries supplying the left kidney, upper and lower poles (anatomical variation). SRA showed the typical FMD lesion which is characterized by its classic "string of beads" appearance, consisting of alternating areas of narrowing and dilatation, located in the middle portion of the lower left renal artery (accessory artery) associated with moderate stenosis (reduction in luminal diameter greater than 50%) (Figure ). The upper left renal artery was preserved. In addition, SRA revealed a small saccular aneurysm of the distal right renal artery (Figure ). Percutaneous balloon dilatation of the stenotic lesion (middle portion of the accessory renal artery) was successfully performed (Figure ). Following angioplasty, her blood pressure normalized over a period of several months using a single antihypertensive medication (atenolol 50 mg once daily), rather than 3 medications.


A 66-year-old woman with TB hip fused in childhood complained of severe contralateral knee pain and intense fatigability during walking accompanied by leg length discrepancy (LLD), severe PO, and secondary scoliosis for the past 10 years. She underwent right hip arthrodesis for TB arthritis with antibiotics, and the affected hip joint was completely inactivated. Owing to a 20° flexed-adducted and ankylosed hip on the right side, high ipsilateral PO occurred with secondary lumbosacral scoliosis on the left convex side (Figure ).
The spina malleolar distance on the affected side was 2 cm shorter than that on the contralateral side. The natural standing posture represented excessive knee flexion of the longer leg to adjust for the LLD (Figure -). If the left knee was completely extended, she was forced to stand on the toes of the affected right foot, indicating plantar flexion of the foot of the shorter limb (Figure ). Whole lower limb antero-posterior radiographs standing on one side showed osteoarthritis of the left knee and a change in PO (Figure ). Moreover, preoperative Cobb angles of the right thoracolumbar and left lumbosacral scoliosis were 40° and 60°, respectively (Figure -). Spinal flexibility on bending or traction radiographs was limited to 30° and 53°, respectively, indicating an almost spontaneously fused spine (data not shown). The preoperative sharp angle was 34° on the affected side and 33° on the contralateral side, respectively.
We decided to manage the ankylosed hip prior to the rigid spine deformities, without directly treating the osteoarthritis of the knee. We speculated that perfect correction of her spinal deformity was impossible. It may have been difficult to make the pelvis horizontal position; we, therefore, aimed to place an acetabular cup at 25° anteversion and 30° inclination, which was considerably less than the ideal 45° normal cup placement appropriate for a PO of 28° (Figure ). THA was performed through the Hardinge approach followed by adductor tendonectomy. The acetabular cup size was 50 mm and the ball size 32 mm, so call large diameter ball. After implantation, the intraoperative range of motion was 35° of flexion, 10° of extension, and 0° of abduction. Moreover, soft tissue releases around the hip joint including adductor tendonectomy improved the range of abduction from 0° to 10°.
Posterior correction and fusion from T10 to the pelvis were performed with autologous iliac bone and allografts at one week after THA, with the anterior release (Figure -). The surgeries were successful without any intraoperative complications. However, it was not possible to match the plumb line at C7 and the center of the sacral vertical line (Figure ). Although slight PO remained postoperatively, the final standing posture and fatigability during walking improved dramatically.
Careful patient education was mandatory to prevent postoperative hip dislocation. Thoracolumbosacral orthosis immobilization was maintained for half a year after surgery. After gait rehabilitation for a few months, she had no difficulty in walking and also experienced indirect improvement of the severe knee pain at the final follow-up. Two years after surgery, spinal arthrodesis was completely accomplished by vigorous bone formation, without hip problems such as postoperative hip dislocation and loosening. Final radiographs showed improved alignment on sagittal and coronal planes (Figure -).
Measurement methods of radiographic values associated with spinopelvic parameters were shown in Table . Perioperative radiographic values (preop/after THA/after ASD surgery/final follow-up) were shown in Table . In particular, preop. and final follow-up values were as follows: thoracolumbar Cobb angle (°): 40/25, lumbosacral Cobb angle (°): 60/14, C7 plumb line shift (mm): 24/0, PI: 61°, PT (°): 15/19, LL (°): 23/60, pelvic incidence minus lumbar lordosis (PI-LL): 38/1, the sagittal vertical axis (mm): 80/0, and PO (°): 28/15, respectively. The inclination angle of the acetabular cup changed from 52° to 35° after ASD surgery; however, the anteversion angle did not change (Figure -).
The Japanese Orthopaedic Association score of the left hip improved slightly from 61 to 69 points. The SRS-22 (function, pain, self-image, mental health, satisfaction) scores also changed 51 points from (2.8/1.4/1.8/4.2/none) preoperatively to 100 points (4.0/5.0/4.2/5.0/4.5) two years postoperatively. In particular, pain and self-image improved significantly, and the patient was satisfied with our surgical treatment.


We present the case of a 64-year-old man who presented to the emergency department because of severe left flank pain radiating to the groin for two days duration. The pain was burning in nature and had been increasing in severity. He rated the pain as 9 on the 10-point severity scale. The pain was constant and was not related to posture, meal intake, or urination. The patient visited the emergency department earlier that day and he was discharged with conservative measures and was given an appointment with the urology team. However, the pain progressed in severity and it became intolerable. Further, the prescribed analgesics failed to provide any clinical improvement. There was no history of change in the urine color or fever. The patient did not have any history of urinary stone disease. He had a long-standing history of diabetes mellitus, hypertension, and dyslipidemia. He also had a history of coronary artery disease for which he underwent percutaneous coronary intervention. The patient had poor control of these conditions and his compliance to medications was poor. He had not undergone any previous surgeries. He was not known to have any drug or food allergies. He was a retired teacher. The patient had a 30 pack-years history of smoking. He had never consumed alcohol. His family history was non-contributory.
Upon examination, the patient appeared in pain. His vital signs revealed tachycardia (128 bpm), tachypnea (18 bpm), normal temperature (37.1℃), and maintained blood pressure (110/75 mmHg). The abdomen was soft and lax with generalized tenderness. The cardiorespiratory examination was unremarkable. The urine dipstick indicated the presence of red blood cells with negative leukocyte esterase and nitrites. Initial laboratory markers showed hemoglobin of 14.1 g/dL, leukocytes count of 8,000/μL, and platelets count of 380,000/μL. Other biochemical parameters, including liver enzymes, urea, and electrolytes, were within the normal limits (Table ).
The patient was seen by the Urology Team for suspected renal colic. Non-contrast computed tomography scan for the kidneys, ureters, and bladder was performed. The scan demonstrated a large high attenuation fluid seen in the left side of the retroperitoneum suggestive of a hematoma (Figure ). Subsequently, the scan was repeated in the arterial phase after the administration of intravenous contrast. It showed an aneurysmal dilatation of the left common iliac artery with surrounding hematoma (Figure ). The contralateral common iliac artery and the abdominal aorta were normal. The patient was taken for an emergency laparotomy. The hematoma was evacuated and a Dacron graft of the affected artery was successfully inserted. The patient had a successful recovery. The patient was discharged on the fourth postoperative day. After three months of follow-up, the patient had no active complaints.


We report a 44-year-old Caucasian woman who initially presented in 2003 with a multitude of progressive lichenoid 2–4 mm papules starting two years ago, particularly marked on the forehead right above both eyebrows, on the dorsal aspects of the forearms, shoulders, legs as well as on the gluteal region, accompanied by severe pruritus (Fig. ). Clinically, the papules were judged as discrete, circumscribed, erythematous to skin-coloured, firm skin eruptions associated with an induration and stiffening of the affected lesions. Furthermore, the patient complained about a progressive thickening of the glabella. Even though we cannot ensure continuous clinical deterioration, at the time of the initiation of therapy, there was no hint for a beginning stabilisation or even improvement of symptoms. Otherwise, she felt healthy and well. The general examination was without pathological findings. Neither a topical nor a systemic therapy was yet applied.
Skin biopsies were taken from the left forearm/wrist, both legs and the left shoulder. Routine histological examination including haematoxylin-eosin, PAS labelling and Alcian blue staining revealed marked mucinous deposition within the upper and mid dermis beside an increased appearance of fibroblasts, collagen bundles and a discrete inflammatory infiltration.
Additionally, immunohistochemical investigations were performed in order to enumerate CD4+ (T helper cells), CD68+ (macrophages), anti-mastcell-tryptase+ (mastcells), decorin+ (collagen fibril stability protein), MIB-1+ (Ki-67+ proliferating cells), CD20+ (B lymphocytes), and FGF-R+ (fibroblast growth factor receptor bearing cells) cells taking consecutive sections (Table ). A punch skin biopsy measuring 3 mm in diameter was taken from affected skin of the right forearm. 5 μm paraffin-embedded sections were cut, mounted on slides and routinely preserved. Prior to the single immunolabelling, different pretreatments were performed for antigen retrieval (Table ). The alkaline phosphatase anti-alkaline phosphatase (APAAP) technique using the labelled streptavidin-biotin (LSAB) method was used to enumerate immunopositive cells at an individual dilution (Table ) taking consecutive sections. The alkaline phosphatase fast red detection kit utilised a biotinylated secondary antibody that binds to the primary antibody. This step was followed by the addition of an streptavidin enzyme conjugate binding to the biotin present on the secondary antibody. Afterwards the specific antibody-secondary-antibody-streptavidin-enzyme-complex was detected using a precipitating enzyme reaction product. Each step was incubated for a precise time and temperature. The alkaline phosphatase was used as enzyme; the chromogene fast red could be visualised. Cells were evaluated semiquantitatively (absent (-), rare (o), moderate (+), frequent (++)) directly below the dermoepidermal junction. Immunopositive cells were evaluated 'blind' separately in two view fields in a row (0.25 mm × 0.25 mm each) directly below the dermoepidermal junction resulting in a length of 0.25 mm and 0.50 mm in depth. In order to avoid a sampling error, a number of sections were randomly reevaluated by a second observer. In case of a significant difference, the sections had to be recounted by both observers. In brief, immunolabelling revealed occasional perivascular CD4+ and CD20+ lymphocytes located in the papillary dermis and a high number of anti-mastcell-tryptase+ cells within the subepithelial perivascular infiltrate revealing a continuing decrease with increasing depth (Fig. ). Simultaneously, an increased dermal expression of MIB-1+ cells (Fig. ), morphologically predominantly fibroblasts, within the upper and mid dermis and sporadic FGF-R+ cells in an unspecific distribution could be detected. CD68 immunohistochemistry and intradermal decorin levels did not alter remarkably as compared to healthy controls (data not shown). Exact results of all immunohistochemical stainings are detailed in Table .
Complete laboratory measurements unveiled the following pathological results: leucocytes 10870 μL-1, lymphocytes 13.2%, IgG 2000 mg/dl. There was no increase in B cell count. Immunoelectrophoresis disclosed albumin 51.4%, alpha-2 globuline 10.5%, gamma globuline 22.9%. Cranial x-ray, x-ray of the thorax, ultrasound of the abdominal organs, electrocardiography and urinary investigations were unremarkable. Blood smear cytological evaluation revealed beginning qualitative but still no quantitative changes as defined by leukocytic aberrations pointing towards a leftward shift. Serum immunofixation demonstrated an IgG-kappa paraproteinaemia. No elevation of the IgG-lambda paraprotein was assessed. Bone marrow biopsies displaying reactive lymphoid infiltration including minimal extension of plasma cells with monoclonal immunoglobuline production provided evidence for monoclonal gammopathy of undetermined significance (MGUS) without distinct morphological characteristics of a plasmocytic plasmocytoma or plasmoblasts.
Initially, 20 MHz ultrasound scanning producing cross section images of the skin was established in order to measure both structure and thickness of the skin at the dorsal aspects of the left wrist and the right forearm. The total thickness of the skin was measured from the entrance echo to the border between the dermis and the subcutaneous tissue. A cutaneous diameter of 2291 μm at the left wrist (lesional skin) and of 1106 μm at the right forearm (non-lesional skin) could be assessed.
In our unit, an experimental treatment modality using oral MTX 12.5 mg once per week followed by a subsequent folic acid application on the following day for a 6-months-period was subsequently initiated. MTX was well tolerated by our patient. After the first three months, the continuous progress of skin lesions during the last two years could be stopped and our patient experienced subjectively an improvement and objectively a stable clinical outcome without new lesions. Subjective impression of amelioration could not be confirmed by means of ultrasound measurement. Within the following two months, no further improvement could be evaluated, whereas no further progression such as formation of new lesions or increase of stiffness could be observed. Due to the unsatisfying clinical results and declining acceptance by our patient, MTX treatment was stopped and a subsequent therapeutic attempt with medium-dose UVA1 cold-light phototherapy was initiated. Irradiation consisting of 50 J/cm2 single-dose UVA1 (CL 300000 liquid, Photomed, Hamburg, Germany) was performed four times a week for three weeks followed by two times a week for further two weeks resulting in a cumulative dose of 800 J/cm2 after five weeks. Meanwhile, the skin status again remained stable, whereas no improvement could be observed. Therefore, our patient broke up phototherapy. To date, skin condition has slightly worsened without any current treatment modality.


In October 2001, a left pelvic adnexal mass was palpated during a routine physical examination of a 41-year-old gravida-1 para-1 Caucasian female. The patient's past medical history was significant for tuberous sclerosis (diagnosed previously based on seizures, radiographic evidence suggestive of lymphangioleiomyomatosis, cutaneous hypopigmented macules and bilateral cystic renal disease that culminated in end-stage renal disease in 1989). A transvaginal ultrasound showed a heterogeneous complex adnexal mass whose size was estimated at 7.0 × 4.8 cm. Also noted was a well-defined lesion (<1.0 cm) in the uterine cervix (figure ); the clinical impression of the latter lesion was a leiomyoma. CA-125 (13 U/ml) and CA19-9 (21 U/ml) levels were within normal limits. The decision was made to resect the adnexal mass and in November 2001, the patient underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy. The surgical procedure was complicated by severe intra abdominal adhesions (secondary to long-term peritoneal dialysis), the lyses of which resulted in two inadvertent nicks to small bowel segments that necessitated the excision of those segments. The patient did not receive any adjuvant or neoadjuvant therapy, and she remains alive with no evidence of recurrent or metastatic disease after 35 months of close surveillance.
Standard representative sections, including the entirety of the uterine cervix were processed routinely for microscopic examination. Sections were fixed in 10% neutral buffered formalin, embedded in paraffin and stained with hematoxylin and eosin. Selected sections were stained for Periodic Acid Schiff (PAS) with and without diastase pre-digestion. The immunohistochemical profile of the tumor was evaluated on 4μ thick, formalin-fixed, deparaffinized sections using a DAKO Autostainer (Carpinteria, CA, USA) based on the avidin-biotin-peroxidase complex. Specifications for the various immunohistochemical stains that were utilized are listed in table . The extent and intensity of the immunoreactivity for each antibody was scored semi-quantitatively on a 1+(+) to 4+(++++) scale representing increasing staining extent and intensity. Labeling index for Ki-67 was calculated by assessing at least 4000 cells and determining the percentage showing unequivocal nuclear staining. In our literature review, the significance of the differences between the group means of two continuous variables (patient age and sizes of lesions) was determined using the student's t-test (Excel®, Microsoft Inc, Redmond, WA). DNA ploidy analysis was performed on isolated tumor nuclei according to standard procedures. Comparative genomic hybridization [] was performed on tumor tissue samples from the cervical mass as previously described [].
Gross and microscopic assessment of the left adnexal mass showed it to be a 7 cm hemorrhagic cyst devoid of any specific lining and involving the ovarian parenchyma. For the uterine cervical mass, a distinct lesion was not grossly appreciated. The ectocervical and endocervical surfaces and the endometrial cavity were described as unremarkable. Microscopically, the cervical mass was unencapsulated but possessed a deceptively circumscribed appearance at scanning magnification, attributable to the architectural homogeneity of its "core" (Figure ). However, the peripheral regions of the tumor showed a significant degree of infiltration. The tumor's maximal dimension was estimated at 2.2 cm, extending from just below the ectocervical basement membrane (Figure ) and extending proximally to the lower uterine segment and attaining 2 cm in depth (the peripheral limits of the tumor were at least 1 cm from the parametrial margins). The aforementioned central "core" (1 cm) was probably responsible for its radiographic appearance and consisted of fascicles of spindle cells with a smooth muscle appearance (Figure ). The spindle cells displayed bland nuclei with regularly distributed chromatin and only rarely conspicuous nucleoli. Towards the periphery, the spindle cells displayed increasingly PAS+, diastase sensitive cytoplasm (Figure ), although occasional cells displayed dense eosinophilia. At its most peripheral regions, the tumor was composed predominantly of solid sheets of large epithelioid cells with bland nuclear features, abundant clear cytoplasm, and well-defined cytoplasmic membranes. Although predominantly solid in the epithelioid regions, a pseudo-alveolar pattern was also evident (Figure ). At the most proximal regions near the lower uterine segment, the tumor appeared to be "invading" in single cells in a hyalinized stroma. The nuclei of the epithelioid cells showed a mild to moderate degree of nuclear atypia, manifested mostly as nucleomegaly and irregularity of nuclear membranes in the absence of hyperchromasia. Rare cells displayed bizarrely enlarged nuclei and multinucleation with a "smudged" chromatin pattern consistent with degenerative atypia (Figure ). Also identified in these regions were CD68+ foamy histiocytes mostly in single cells but occasionally in aggregates especially around the endocervical glands. No tumor necrosis was identified and mitotic figures were extremely sparse (<1/50 HPF). Small arching sinusoidal vessels were prominent throughout the tumor, but no large malformed vascular profiles were present. Pigment-laden cells and adipocytes were not present in the cervical mass. Small bowel segments measuring 21 cm in total length were also processed. Grossly, irregular areas of transmural thickening were noted. Microscopically, aggregates of epithelioid cells with more eosinophillic cytoplasm and vacuolated cytoplasm were noted in the lamina propria in two out of twelve sections (Figure ). In the both ovaries, similar aggregates of cells were present in a distinctive perivascular location in the hilar regions. These aggregates were either subendothelial (predominantly), adventitially attached to affected vessels, or present as free aggregates in the perihilar fat (Figure ). Each measured less than 1 mm in maximum dimension. No intraluminal tumor cells were seen. CGH and DNA ploidy analysis of the cervical mass showed a balanced chromosomal profile (figure ) and diploid nuclei, respectively. Immunohistochemically, both the epithelioid cells and spindle cells stained diffusely with HMB-45 (Figure ) and Melan-A in a cytoplasmic pattern at all sites (cervix, ovary, bowel). Scattered spindle cells showed unequivocal immunoreactivity for S100 while the epithelioid cells were negative. Both components showed at least focal immunoreactivity for muscle markers: smooth muscle actin and desmin with the spindle cells predominating both in the quantity of cell stained and the intensity of staining where positive. The complete immunohistochemical profile of the tumor is shown in table .


A one-year-old boy was noted to have a striking increase in head circumference as compared to a previous measurement. Neurological examination and developmental status were normal at that point. Within 2 weeks, the patient deteriorated rapidly, with poor mobilization, feeding and verbalization. He was brought to the emergency room where an emergent computed tomographic scan showed a large left hemispheric cerebral mass (Figure ) with an underlying cystic component and a more superficial area of bright enhancement; the rest of the brain showed massive edema. He was emergently admitted and within 24 hours, a gross resection of the tumor was carried out. At surgery, following a parietal craniotomy, 50–60 cc of straw colored fluid was aspirated from the cystic component through a taut dura. After the excision of the dura, the bright area of enhancement previously noted was an area of tumor attachment to the dura in the parietal region. Otherwise, the tumor showed a well-demarcated interface with the subjacent normal brain parenchyma and a complete gross resection was achieved. A follow-up magnetic resonance image at 12 months post-surgery showed no evidence of tumor recurrence. Functionally, the patient was felt to have a mild right hemiparesis and some probable language delay, but otherwise showed no neurological deficits.


A 45-year old male presented to our unit with acute onset macroscopic haematuria. His past medical history was significant in that he had been diagnosed with adenocarcinoma of the distal oesophagus 2 years prior and had undergone curative resection after neo-adjuvant chemotherapy. At that time the tumour was found to be poorly differentiated with evidence of local nodal spread. He had been reviewed regularly by the oncologists and remained asymptomatic until the onset of frank haematuria.
He subsequently underwent cystoscopy, which revealed a solid bladder tumour on the right lateral wall, which was treated with trans-urethral resection. Pathological examination confirmed a poorly differentiated mucus-secreting adenocarcinoma, identical histologically to the original oesophageal tumour (Figures and ).
A diagnosis of metastatic oesophageal adenocarcinoma to the bladder was made. A CT scan did not demonstrate any pelvic tumour outside the bladder and therefore metastasis by the trans-coelomic route was essentially excluded, indicating haematogenous spread of the primary oesophageal carcinoma. The patient was referred for further oncological therapy but unfortunately died 4 months later from disseminated carcinoma.


22-year-old white female, with abdominal pain. Video-laparoscopy showed a liver mass and multiple peritoneal implants diagnosed as DSRCT. Six months after the diagnosis, she started chemotherapy for four months, and reduction of tumor mass was observed. One month after the end of chemotherapy, the tumor was removed. Macroscopically, tumor mass measured 5.0 × 4.0 × 3.8 cm and was involving uterus, pericolic tissue, and vagina. Histological analysis shows also involvement of both ovaries and large bowel wall. Ten out of 13 lymph nodes showed metastasis of DSRCT. The peritoneal fluid colleted during surgery was negative for neoplastic cells. Eight months after the first surgery, she presented with a recurrence in the abdominal cavity and a new resection of the tumor mass showed involvement of cecal appendix. Peritoneal fluid sample collected at that time was positive for malignant cells. In the follow up examination, seven months after the second surgery, it was found an inguinal tumor mass of 15 mm. FNA was performed and showed DSRCT metastasis. After the diagnosis, this patient was transferred to another institution.


Seven year-old male with back pain and fever. CT scan showed pleural effusion and a mediastinal mass measuring 16.0 × 9.0 cm. Tumour mass showed involvement of soft tissues. Surgical biopsy and pleural drainage were performed. The patient was treated with radiotherapy and chemotherapy, but died 8 months after the diagnosis.


Male, 15-year-old had acute abdominal pain and was submitted to an exploratory laparotomy that disclosed a large pelvic mass, involving epiplon and sigmoid, cecum, liver and peri-aortic lymph nodes. This patient had multiple nodules on peritoneal surface. The biopsy of tumor was performed. One month after the diagnosis, chemotherapy was initiated. The patient was submitted to chemotherapy during 8 months, with reduction of more than 50% of tumor mass. A second laparotomy was done to excise retroperitoneal and retrovesical mass. At this time peritoneal fluid sample was collected. After surgery, chemotherapy was continued. The patient is alive, with residual disease.


A 72-year-old gentleman presented with pain and swelling around the right testis. On admission, he was jaundiced. Scrotal examination revealed a firm and tender mass, separate from the testis, in the region of the epididymis and distal spermatic cord. Ultrasound examination showed prominence and thickening of the right epididymis, normal right testicular appearances, and a right hydrocele. Computed tomography demonstrated marked dilatation of the intrahepatic biliary tree extending distally through the extrahepatic ducts to the confluence of the left and right hepatic ducts. The common hepatic and bile ducts were not clearly seen although a subtle infiltrate encasing the vessels at the porta hepatis was noted. No other significant pathology affecting the thorax, abdomen, or pelvis was seen. Serum bilirubin and alkaline phosphatase were elevated in keeping with the obstructive picture. Endoscopic retrograde cholangio-pancreatography revealed a long and irregular stricture of the common bile duct extending into the common hepatic duct. A 150 mm metal stent was inserted to relieve the obstruction through which bile was subsequently seen to drain effectively. A clinical and radiological diagnosis of cholangiocarcinoma was made.
Subsequently, an uncomplicated right inguinal orchidectomy was undertaken.


A 56-year-old female with acute pancreatitis was admitted under our care. She was known to suffer from diverticular disease and had a myocardial infarction in the past. She was allergic to metronidazole and buscopan. She had no family history of drug allergies.
During the initial course of management she was given 50 mg of ranitidine as a slow intravenous bolus for epigastric discomfort. Few minutes after the injection, the patient complained of itching at the injection site that spread to involve the entire upper limb. She also complained of swelling of her tongue and difficulty in breathing. Within minutes her level of consciousness deteriorated and she became comatose. The initial examination revealed the following features: GCS 6/15, a grossly oedematous face, neck and extremities, a grossly swollen tongue, congested conjunctivae, cyanosis, diffuse rhonchi over both lung fields.
Despite immediate administration of intramuscular adrenaline, intravenous hydrocortisone and high flow oxygen the patient progressed into a cardio respiratory arrest. Cardiopulmonary resuscitation was commenced. Patient was intubated with difficulty and was resuscitated successfully. She was transferred to the Intensive Care Unit for ventilatory and inotropic support. Two days later she was weaned off the ventilator and by then she was haemodynamically stable without inotropic support. She made a full recovery in 3 days. A skin sensitivity test prior to the patient's discharge revealed the patient's sensitivity to Ranitidine.


A 45 year old gentleman is referred to a colorectal clinic with diarrhoea and faecal incontinence for 2 months. He has also suffered with progressively worsening urinary symptoms: hesitancy, frequency and nocturia, poor stream and sensation of incomplete emptying and possible intermittent retention, with urge incontinence over 2 years. Previous review by Urologists revealed no abnormality on cystoscopy, X-ray KUB or renal ultrasound and a diagnosis of prostatitis was made. There was no history of pain, lower limb neurological symptoms and he had normal erectile function. There was no history of diabetes, multiple sclerosis or other neurological disease. On clinical examination, resting and squeeze anal tone were reduced. Perianal sensation was intact. Colonoscopy was requested which revealed a normal colon. Faecal calprotectin and stool cultures were normal. At this stage anorectal physiology was not requested as primary anorectal pathology was not thought to be the cause of these symptoms and there was a clear clinical picture of incontinence therefore manometry would not have altered management. A referral was made to Neurology and re-referral to Urologists.
Neurological review 1 month later revealed newly developed mild lower limb numbness when sitting down which disappeared on walking. However there was no deterioration in function. Full central and peripheral neurological examination only revealed a brisker reflex in the right knee.
An MRI of the spine showed a lesion just distal to the conus, causing the roots of the cauda equina to be displaced peripherally at the level of L1/2. The lesion enhanced fairly uniformly and appearances were thought to be in keeping with an ependymoma (Figure ).
A month later laminectomy and excision of this tumour was performed with electrophysiological intra-operative monitoring. Histology revealed a classical ependymoma (WHO Grade II) with no myxopapillary regions (Figure ). Perivascular pseudorosettes were evident, and mitoses were very infrequent with no necrosis. The immunohistochemistry showed strong focal positivity for GFAP and S100, but only very focal positivity for EMA. The proliferation index was low at approximately 2%.
Six months after initial presentation he had some residual urinary symptoms but his faecal incontinence had resolved.


The patient was a 53 year old man with a history of osseous hydatosis since the age of 30. He had had three surgical operations due to the disease. He had low back pain and radicular pain in both legs which had been aggravated over the past month. His past history for any other illnesses was negative. There wasn't a history of coughing or other respiratory symptoms. His general condition was good. On examination we detected low back tenderness in the lumbar area. He had a painful tumor-like mass in the right hemipelvis which was slightly warm without overlying erythema. Straight leg raising test (SLR) was positive in both legs. He had motor and sensory deficit, muscular atrophy and gate disturbance. No other abnormal findings were detected.
Simple x-ray of the right femur showed advanced lytic bone destruction, centered in the proximal two thirds of the right femur. In addition there were the appearances of nailing due to previous surgery (fig. ). The plain pelvic x-ray also revealed marked lytic and sclerotic lesions involving the right hemipelvis (Figure ). A whole body bone scintigram revealed multiple foci of increased radiotracer uptake in the lower lumbar spine, left sacroiliac region and right knee. In addition, there was soft tissue bulging in the right hemipelvis as well as a displaced and disconfigured right femur (Figure ). Chest x ray was normal.


A 65-year-old man was hospitalized with a 1-month history of exertional shortness of breath and productive cough. He had a history of coronary artery disease and was under medical treatment with amiodarone (200 mg/day) for approximately 4 years, due to recurrent atrial fibrillation. On his first hospital admission the patient was in sinus rhythm with 86 beats per minute and blood pressure within normal range. His physical examination revealed fine, late inspiratory crackles on both lung bases and no other signs of congestive heart failure. Small bilateral pleural effusions were present on the chest X-ray, whereas CT scanning of the thorax revealed a pattern of pulmonary fibrosis that was attributed to chronic amiodarone treatment, after excluding other causes with fiberoptic bronchoscopy (bronchoalveolar lavage and transbronchial biopsy). A myocardial infarction was ruled out, amiodarone treatment was discontinued and the patient was discharged on sotalol 120 mg/day.
Twenty four hours later, he was readmitted to the emergency department with fever (up to 38.6°C), severe dyspnea and production of pink, frothy sputum. On second hospital admission the patient was cyanotic, restless and irritable [arterial blood gases (ABGs) without supplemental oxygen: pH: 7.49; pO2: 45 mmHg; PCO2: 35 mmHg; SpO2: 85%], with pulse of 145 beats/min, blood pressure 140/80 mmHg and respiratory rate of 35/min. Physical examination revealed regular tachycardic rhythm with S3/S4 gallop, whereas rales presented in all lung fields. His electrocardiogram (ECG) showed a sinus tachycardia, without evidence of acute myocardial ischemia. Blood count and routine serum biochemistry tests were normal. The patient was intubated and transferred to the ICU, where he was started on bronchodilators, furosemide diuresis and broad spectrum antibiotics (ciprofloxacin plus amoxycilline/clavulanic acid), as the initial impression was of an acute pulmonary edema due to decompensated heart failure or concomitant severe respiratory infection. However, plasma thyroid function tests were indicative of severe thyrotoxicosis that was attributed to chronic amiodarone treatment (Figure ), [free T4:26 ng/dL, (normal range: 0.7–1.9); Thyroid Stimulating Hormone (TSH) <0.01 μIU/ml, (normal range: 0.38–3.80); free T3:9.5 pg/mL, (normal range: 1.4–3.8)] and he was begun on propylthiouracil (600 mg, po, tid), prednisone (30 mg, daily, IV), propranolol (40 mg, qid), furosemide (40 mg/h, IV) and low molecular weight heparin. At the same time, the patient remained under sedation with midazolame and remifentanyl and occasionally, under neuromuscular block with cis-atracurium. Antithyroglobulin, antimicrosomal and TSH-receptor antibody results were negative. The ultrasonography of the thyroid gland was more or less normal (slightly increased gland size) whereas color flow Doppler sonography (CFDS) demonstrated a heterogeneous pattern with decreased flow.
A slight clinical amelioration was observed a few days later (Figure ), while serum free T4 and free T3 decreased promptly (FT4: 5.5 ng/dL, FT3: 5.13 pg/mL). All blood, urine and sputum cultures remained negative, whereas procalcitonin (PCT) and C-reactive protein (CRP) levels were within normal range. Nevertheless, as the patient had difficulties in weaning from mechanical ventilation, he underwent a percutaneous tracheotomy during the 10th day of stay in ICU. Forty eight hours later, he developed tachycardia and hypotension. His plasma thyroid function tests were indicative of recurrent thyrotoxicosis (Figure , TSH <0.01 μIU/ml, FT4:14.5 ng/dL, FT3: 6.4 pg/mL). ST segment depression was observed in all ECG leads, without an increase on serum myocardial enzymes. Cultures of different origin remained negative. Deep vein ultrasonography, D-Dimers assay and transthoracic echocardiography were negative for pulmonary thromboembolism. His ventricular response was unable to control despite escalating doses of β-blockers, whereas inotropic support failed to restore the failing circulation. The patient ultimately developed multiple organ failure and was pronounced dead seven days later.