Cases reported "Adenoma, Islet Cell"

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1/6. Nonfunctioning endocrine tumor of the pancreas with extrapancreatic growth and cyst formation: report of a case.

    We report a case of nonfunctioning endocrine tumor of the pancreas with extrapancreatic growth and cyst formation. A 48-year-old woman was admitted to our hospital with an upper abdominal mass, which was detected during a routine medical checkup. The preoperative diagnosis was a solid cystic tumor of the pancreas. The tumor, which measured about 7cm in diameter, arose from the head of the pancreas and had a thin stalk, measuring about 1 cm in diameter. The histopathological diagnosis was a malignant nonfunctioning endocrine tumor of the pancreas. Cyst formation occurred in 67% of the reported cases with extrapancreatic growth in japan. It is speculated that the cyst formation in this disease is related to the large size of the tumor and to the extrapancreatic growth.
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2/6. Islet cell tumor arising from a heterotopic pancreas in the duodenal wall with ulceration.

    A rare case of symptomatic islet cell tumor arising from heterotopic pancreas in the duodenum with ulceration is described. Gastrointestinal bleeding was the only sign observed in this patient. Tagged red blood cell scan, upper endoscopy, and computed tomography scan showed active bleeding ulcer from a periampullary mass. Removal of the submucosal tumor was done to prevent future re-bleeding. Histologic and immunohistochemical characterization of the tumor showed an endocrine tumor that expressed a variety of endocrine peptides.
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3/6. glucagonoma syndrome. Report of two cases and literature review.

    The glucagonoma syndrome is characterized by dermatitis, stomatitis, elevated serum glucagon levels, abnormal glucose tolerance, weight loss, and anemia--all in association with a glucagon-secreting alpha-cell tumor of the pancreas. A review of 21 cases showed strikingly similar features. A generalized, symmetrical dermatitis initially appeared to be asteatotic or eczematous over the perineum, buttocks, and lower extremities. Gradually, a more characteristic migratory necrolytic erythema with transient bulla formation and erosions developed in intertriginous and dependent areas. Histologically, the most specific features included necrolysis of the upper epidermis, with liquefaction necrosis of the granular cell layer and subcorneal clefting or blister formation. The dermatologist is often first to examine such patients; early recognition of this syndrome with prompt surgical removal of the primary pancreatic lesion may afford cure of the neoplasm.
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4/6. Islet cell tumor presenting as massive upper gastrointestinal bleeding in pregnancy.

    A 27-yr-old woman presented in the 6th month of pregnancy with massive gastrointestinal bleeding due to erosion of a pancreatic islet cell tumor into the duodenum. Most islet cell tumors present with neuroendocrine manifestations; massive bleeding is a very rare presenting sign. This is the first reported case of a pregnant woman with massive bleeding from islet cell tumor erosion who underwent successful resection and delivery of a healthy term child 3 months later.
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5/6. Malignant glucagonoma syndrome: response to chemotherapy.

    A 58-year-old white woman with known metastatic glucagonoma had widespread necrolytic migratory erythema characteristic of the glucagonoma syndrome. She did not respond to conventional chemotherapy with streptozocin. After one course of dacarbazine therapy, she had remission of the glucagonoma clinically with regression of tumor metastases as defined by liver scanning. After 10 months and additional courses of dacarbazine therapy, she remains in clinical remission. plasma glucagon levels have decreased although they remain at two to four times the upper limit of normal. On several occasions there was resolution of this patient's rash after intravenous glucose in the absence of supplemental amino acids. We conclude that dacarbazine is an effective mode of chemotherapy for malignant glucagonoma.
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6/6. Ultrastructural studies of necrolytic migratory erythema.

    necrolytic migratory erythema is a disorder highly suggestive of glucagonoma syndrome. We carried out histologic and electron microscopic studies of the skin lesions in a 57-year-old woman with the glucagonoma syndrome. light microscopic studies revealed hyperkeratosis, parakeratosis, formation of clefts in the upper epidermis, and scattered dyskeratotic cells. Ultrastructurally, the intercellular spaces were widened in the upper epidermis and desmosomes were reduced in number. The cytoplasm of affected cells showed vacuolar degeneration; the organelles were lysed or absent. There were scattered dark cells and dyskeratotic cells. These changes appear to represent a degenerative process of the keratinocytes.
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