Cases reported "Intestinal Neoplasms"

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1/3. Recurrent malignant schwannoma of the small bowel.

    Primary malignant schwannoma of the small bowel is an extremely rare disease. Only 24 malignant schwannomas of the small bowel have been reported in the medical English literature. We report a primary malignant schwannoma of the distal ileum in a 53-year-old woman, who was admitted to the hospital because of abdominal pain. CT scan revealed a semisolid mass in the small intestine of the serosa. The tumour measuring 14 x 13 x 8 cm and 100 cm of the distal ileum were resected. We observed a recurrence after seventeen months and the recurrent tumour measured 6 x 2.5 x 1 cm was excised.
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2/3. A case report of primary osteosarcoma of the ovary.

    Primary osteosarcoma is one of the rare tumors affecting ovaries. This case is being reported for its rarity, along with a review of the literature. In this study, the patient, a 43-year-old woman, presented with an abdominopelvic mass. Exploratory laparotomy was performed, which revealed an extensive left ovarian mass infiltrating the uterus, small bowel, and urinary bladder. Subtotal hysterectomy and small bowel resection followed by end-to-end anastomosis and bilateral salpingo-ophorectomy were performed. Histopathology showed primary osteosarcoma of the ovary. Eighteen days after surgery, she started presenting with progressive abdominal distention and ultimately developed subacute intestinal obstruction. She was started on carboplatin and epirubicin combination but failed to respond and died of fast progressive disease. It is concluded that primary osteosarcoma of the ovary is a rare disease with poor prognosis.
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keywords = rare disease
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3/3. Primary intestinal T-cell lymphoma and sclerosing cholangitis: a cytokine-mediated association?

    A 63-year-old woman with a 1-year history of abdominal pain and intrahepatic cholestasis developed anorexia, weight loss, lassitude and diarrhoea. Studies led to a diagnosis of primary intestinal T-cell lymphoma involving especially the proximal small intestine and infiltrating the mesenteric lymph nodes, bone marrow and skin. An associated severe hypoalbuminaemia (1.3 g dL-1) was most probably the result of protein-losing enteropathy. liver biopsy demonstrated concentric fibrosis of the bile ducts ('onion skin' lesions, with an inflammatory cell infiltrate and lymphoid aggregates) and was considered almost pathognomonic of primary sclerosing cholangitis. Sudden death due to pulmonary embolism occurred and a limited autopsy confirmed the diagnosis. Other associated diseases such as coeliac disease or inflammatory bowel disease were not found. This first report of the simultaneous occurrence of two rare diseases - primary sclerosing cholangitis and intestinal T-cell lymphoma - may indicate an intriguing association, possibly mediated by the effect of cytokines released by the infiltrating T-cells into the portal circulation.
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keywords = rare disease
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