Hepatic Follicular Dendritic Cell Sarcoma with Epithelioid Morphology: Histopathologist's Perspective.

Follicular dendritic cell (FDC) sarcoma is an uncommon tumor of the liver with only 30 previous cases reported in the English literature. Histopathological examination is the gold standard for the diagnosis of FDC sarcoma although the diagnosis is often missed because of its rarity. It usually presents with spindle-cell morphology although epithelioid/biphasic morphology is also well-known. This morphological variation can also pose a diagnostic challenge. We discuss a case of unresectable hepatic FDC sarcoma in an adult male who was diagnosed in core biopsy. We highlight the relevant histomorphological differentials and diagnostic approaches to FDC sarcoma in this anecdote.

Perforation following pneumatic dilation of achalasia cardia in a university hospital in northern India: A two-decade experience.

Pneumatic dilation (PD) is a cost-effective first-line treatment for achalasia. The most feared complication of PD is esophageal perforation (EP). As data on EP after PD for achalasia are not widely reported, we present the frequency, risk factors, and treatment-outcome of EP. Records of patients undergoing PD for achalasia (January 1995 to September 2015) were retrospectively reviewed. Of 433 patients (age 38 years, 13-88, 57% male), and 521 dilations, 12 were complicated by EP (2.7% of patients and 2.3% of PD). EP occurred in 7 (3.4%), 4 (1.7%), and 1 (4.1%) with use of balloon diameters 30, 35, and 40 mm, respectively. In most (11/12, 92%), EP occurred during the first PD. No risk factor for EP was identified (p = 0.65 for the first dilation vs. > 1 dilation, and 0.75 for balloon size of 30 mm vs. > 30 mm). Seven patients with contrast leak on esophagogram and/or computed tomography scan underwent surgery. One other with contrast leak was successfully treated with a fully covered self-expandable metal stent (FC-SEMS); the remaining four with small leak/pneumomediastinum were managed conservatively. The median duration of hospital stay following perforation was 13 days (7-26) and 8 days (6-10) in surgery and conservative groups, respectively. No mortality was observed in either group. The frequency of EP with PD was 2.3%. Though most EP (92%) occurred during the first dilation, neither the balloon size nor repeated dilations were identified as risk factors. Both surgical and conservative approaches had a favorable outcome in appropriate settings.

Anaplastic large T-cell lymphoma presenting as an isolated osseous involvement: a case report and review of the literature.

This case describes a 50-year-old man with multiple lytic bone lesions involving the skull, pelvis and ribs and associated features of anaemia, hypercalcaemia, azotaemia and subsequently found to be non-Hodgkin's lymphoma of anaplastic large T cell type (ALCL) on biopsy taken from a soft tissue mass in the anterior chest wall as an aetiology. We report this case with an aim to expand our knowledge of isolated osseous involvement in case of non-Hodgkin's lymphoma. This case was unique in the sense of an isolated osseous involvement in ALCL without any organomegaly and lymphadenopathy with a normal bone marrow biopsy finding. So in rare instances, T-cell lymphoma can present with a multiple lytic lesion, anaemia, hypercalcaemia. Although B-cell non-Hodgkin's lymphoma can present with the above manifestation, it is extremely rare for T-cell non-Hodgkin's lymphoma.

Primary hypertrophic osteoarthropathy (incomplete form) in young adults: a case report and review of literature.

Hypertrophic osteoarthropathy is characterised by digital clubbing and periosteal reaction of long bones. Most cases are associated with malignancy or other conditions such as congenital heart disease, liver cirrhosis, pulmonary fibrosis, biliary atresia and inflammatory bowel diseases. We report a middle-aged man found to have 15-year history of clubbing of the fingers and toes on his routine check-up for dyspepsia. Skiagram of hand joints showed periosteal apposition without any periosteal reaction of long bones. The search for a secondary cause of clubbing remained negative. The primary or idiopathic form is rare and has a good prognosis and has to be differentiated from secondary form. He was eradicated successfully with Pylori kit for his antral predominant Helicobacter-induced gastritis.

Periampullary mass--a rare presentation of poorly differentiated neuroendocrine cancer of duodenum in a young adult: a case report and review of literature.

Poorly differentiated neuroendocrine tumour in the periampullary region of the duodenum is a rare entity. This entity usually present in old men. Here we report a periampullary poorly differentiated neuroendocrine cancer (PDEC) of duodenum presenting in a young man with subacute history of jaundice, abdominal pain, pancreatitis and constitutional symptoms. MRI localised the tumour and endoscopy-guided biopsy of the lesion proved the diagnosis. Although palliative surgery and chemotherapy were planned, the patient opted to leave against medical advice.

Sweet's syndrome in a patient with infective endocarditis: a rare clinical entity.

Sweet's syndrome, also known as acute febrile neutrophilic dermatosis, has been associated with malignancy, autoimmune disease and collagen vascular disease. The association of infective endocarditis and Sweet's syndrome is rare. The authors report a case of Sweet's syndrome in a patient with infective endocarditis. Infective endocarditis should be excluded in patients of rheumatic heart disease presenting with Sweet's syndrome. Alternatively, Sweet's syndrome should be considered as a differential diagnosis when a patient with infective endocarditis develops skin lesions.

Rare association of Visceral leishmaniasis with Hodgkin's disease: A case report.

We present here a case of young male with complaints of fever and swelling in the neck for eight months. History of progressive weakness associated with weight loss was present. Physical examination revealed pallor, multiple enlarged cervical lymph nodes and hepatosplenomegaly. Investigations showed pancytopenia, hyperglobinemia and Leishman-Donovan bodies on bone marrow aspiration. Serological test confirmed diagnosis of visceral leishmaniasis. However, cervical lymph node aspiration and biopsy were suggestive of Mixed cellularity Hodgkin's disease. This made it a very rare case of Leishmaniasis as an opportunistic infection in a patient of pre-chemotherapy Hodgkin's disease. There was marked improvement in haematological profile and regression of hepatosplenomegaly with Amphotericin B treatment followed by favourable response to chemotherapy. The case emphasizes the suspicion for leishmaniasis as a masquerader and as an opportunistic infection in haematological malignancies.

Mediastinal mass-a rare presentation of desmoplastic small round cell tumour.

Primary mediastinal desmoplastic small round cell cancer is an uncommon tumour usually located in the abdomen and pelvis. Here the authors report an extremely rare case of a young male with a primary desmoplastic small round cell tumour in the anterior and middle mediastinum. The patient had non-specific complaints but an abnormal shadow was seen in a routine chest x-ray. He was diagnosed as having mediastinal mass with few lung parenchymal deposits on CT. Mediastinoscopy and guided biopsy revealed desmoplastic small round cell tumour. Desmoplastic small round cell tumour is a rare and aggressive tumour which rarely involves the mediastinum as a primary site. The nature of the lesion and its prognosis were explained to the patient. He was offered chemotherapy and radiotherapy for the tumour management. He refused treatment and left against medical advice.

A rare case of non-insulinoma pancreatic hypoglycaemia syndrome (niphs) in an adult due to localised islet cell hyperplasia-successfully managed by enucleation.

Persistent hyperinsulinemic hypoglycaemia is caused most commonly by an insulinoma in adults or by nesidioblastosis in neonates. In adults, localised islet cell hyperplasia is a rare disorder characterised by localised proliferation of islet cells. The authors present the case of a previously healthy non-obese middle aged female with new-onset severe hypoglycaemia. Laboratory findings confirmed a case of hyperinsulinemic hypoglycaemia. Endoscopic ultrasonography, intra-arterial calcium stimulation test and intraoperative ultrasonography showed a lesion in the uncinate process that was enucleated. Rest of the pancreas was normal. Histological examination and immunostaining of the resected tissue revealed pancreatic tissue with maintained acinar pattern consistent with diagnosis of localised islet cell hyperplasia. The patient did not have further episodes of hypoglycaemia following the procedure. Localised islet cell hyperplasia with such a very high insulin level is exceedingly rare in adult populations and not reported in literature. This diagnosis cannot be easily made through routine diagnostic laboratory or radiological procedures and likely requires a histological diagnosis. Management of this rare entity is by enucleation.