Cases reported "Pancreatic Neoplasms"

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1/80. Pancreatic cancer and fibrinogen storage disease.

    BACKGROUND: Ductal adenocarcinoma is the most common type of pancreatic carcinoma while squamous, carcinosarcoma, sarcoma, giant cell carcinoma, and clear cell types are all rare. Hepatocellular fibrinogen storage disease is also an uncommon disorder which may be associated with hepatocellular carcinoma. Two cases of pancreatic carcinoma were encountered in a family with fibrinogen storage disease, further raising the possibility of a predilection to malignancy in this unusual disorder. The tumour in one case was of the rare clear cell type. These two cases are the basis for this report. methods: Sections were cut from retrieved paraffin embedded tissue and stained for routine histology. immunohistochemistry using the avidin-biotin technique was applied for the expression of the markers p53 (D07), carcinoembryonic antigen (CEA), c-erbB-2, epithelial membrane antigen (EMA), and alpha-fetoprotein (AFP). RESULTS: Both cases were adenocarcinoma of pancreatic ductal origin. The tumour in one case showed features of a clear cell carcinoma. The tumour cells expressed p53, CEA, and EMA immunoreactivity and were negative for c-erbB-2 and AFP. CONCLUSIONS: Hepatocellular fibrinogen storage disease is rare and has been described in association with chronic hepatitis, cirrhosis, and rarely with hepatocellular carcinoma. This represents the first report of its association with carcinoma outside of the liver.
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keywords = giant
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2/80. Immunohistochemical and molecular analysis of giant cell carcinoma of the pancreas: a report of three cases.

    We performed molecular biological studies as well as immunohistochemical analysis of three cases of giant cell carcinoma of the pancreas. Histologically, one case was a pleomorphic giant cell carcinoma consisting of pleomorphic giant/ small cells and spindle cells, one an osteoclast-like giant cell tumor composed of osteoclastoid giant cells and pleomorphic small cells, and one a pleomorphic giant cell carcinoma with osteoclastoid giant cells. Immunohistochemically, pleomorphic giant cells and small pleomorphic cells were positive for epithelial and mesenchymal markers throughout the cases. Osteoclastoid cells were strongly positive for PG-M1 (CD68), but negative for lysozyme and epithelial markers. Pleomorphic spindle cells showed the same immunoreactivity as pleomorphic giant/small cells. Genetically, all cases contained a mutation in the K-ras (codons 12, 13) oncogene, but neither p53 (exons 5-8) nor p16INK4 (exons 1, 2) gene mutations were found in any case. Furthermore, loss of heterozygosity (LOH) of the p53, p161NK4. APC, and DPC4 gene loci was not found in any of the cases. Immunohistochemical study demonstrated this tumor to be of epithelial origin with mesenchymal differentiation. Genetically, initiation of the tumor is similar to that of usual ductal adenocarcinoma, but progression might be rather different. The peculiar histologic and biologic features of this tumor would be the result of changes in other functional genes.
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keywords = giant
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3/80. Giant insulinoma in a patient with multiple endocrine neoplasia-type I: a case report.

    We report a case of giant cystic insulinoma constituting part of multiple endocrine neoplasia (MEN) type I. A 29-year-old Japanese man presented with a history of recurrent hypoglycemic attacks. Endocrine examination showed hyperinsulinemia discordant with hypoglycemia, and a giant cystic insulinoma (11 x 10 cm) located in the pancreatic tail was detected radiologically. hyperprolactinemia due to pituitary adenoma and hyperparathyroidism due to parathyroid hyperplasia were also present. The insulinoma, prolactinoma and hyperplastic parathyroid gland were surgically removed. Fluorescent microsatellite analysis detected loss of heterozygosity (LOH) in chromosome 11q13 in dna samples from all resected tissues but not from white blood cells. This is a rare case of MEN type I because of the giant cystic insulinoma and the evidence of common LOH detected in all MEN type I tissues.
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4/80. Double ileoileal intussusception caused by a giant polypoid mass of heterotopic pancreas in a child.

    Heterotopic pancreatic tissue has been found in several abdominal and intrathoracic locations. In the ileum, it is a rare, usually asymptomatic, incidental finding. CONCLUSION: A unique case of a recurrent ileoileal intussusception in an 11-year-old girl is presented caused by a giant polypoid mass composed of ectopic pancreatic tissue that remained undetected during several diagnostic tests during two previous admissions and laparoscopic abdominal exploration.
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5/80. Osteoclastic giant cell tumor of the pancreas: case report and literature review.

    An unusual case of a 40-year-old woman seeking treatment for a 10-cm cystic neoplasm of the pancreas is described. Imaging revealed a large proteinaceous, fluid-filled cyst with a mural nodule. laparotomy was successful with en bloc resection. Pathologic examination revealed a neoplastic mucinous epithelial tumor with an abundance of multinucleated tumor giant cells. This presentation is consistent with literature reports of an osteoclastic-type giant-cell tumor of the pancreas. The natural history, pathologic evaluation, and clinical implications of this rare neoplasm are discussed with reference to published reports.
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6/80. Resectable pleomorphic giant cell carcinoma of the pancreas.

    We present a 72-yr-old woman who underwent surgical resection of a large pancreatic tumor. On preoperative imaging, an intratumoral cavity containing necrotic tissue was noted, mimicking the appearance of a cystic tumor. There was no invasion of adjacent organs nor distant metastasis, and histopathologic examination revealed the tumor to be a giant cell carcinoma. Postoperatively, the patient has been followed for 1.5 yr without evidence of recurrence. Giant cell carcinoma generally is associated with a poor prognosis, and patients usually die within months despite intensive multimodality therapy. Some patients with giant cell carcinomas, however, achieve long-term survival when invasion of adjacent organs and distant metastases are absent. Surgical resection is the appropriate treatment for tumors with these favorable characteristics.
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ranking = 6
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7/80. Pancreatic mucinous cystadenoma associated with celiac disease and polycystic kidneys. Case report and short review of the literature.

    We report a case of giant benign pancreatic mucinous cystadenoma in a 19-year-old woman with celiac disease and polycystic kidneys. She presented with a history of moderate episodic left flank pain evolving for a few weeks before becoming acute the night before her admission. We provide radiological and histological material and a short review of the literature.
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keywords = giant
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8/80. Osteoclast-like giant cell tumor of the pancreas.

    A rare case of osteoclast-like giant cell tumor of the pancreas is reported. A 45-year-old woman presented with upper abdominal pain and weight loss. Examination revealed a tumor in the tail of pancreas, and distal pancreatectomy with splenectomy was performed. Pathological findings showed the tumor was composed of two cell types: atypical mononuclear round cells and abundant osteoclast-like multinucleated giant cells with central nucleoli. Immunohistochemical study showed that the atypical cells were strongly reactive for vimentin and negative for CD68, while the giant cells were immunoreactive for CD68, but negative for vimentin. The tumor was diagnosed as osteoclast-like giant cell tumor of the pancreas. We report this case and review 31 cases previously described in the literature.
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ranking = 8
keywords = giant
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9/80. Xanthogranulomatous choledochitis: a previously undescribed mass lesion of the hepatobiliary and ampullary region.

    Solid liver and pancreatic masses are commonly neoplastic in nature; however, inflammatory lesions mimicking carcinoma are at times encountered in these sites. We report two cases of previously undescribed inflammatory mass lesions of the liver and pancreas that originated in the biliary tract. Detailed clinical and histologic evaluations were performed in two patients who underwent right partial hepatic lobectomy and Whipple's resection for presumed hepatic and pancreatic neoplasms. In case 1, with a remote history of cholecystectomy and recent extraction of a stone from the common bile duct, a liver mass in segment 6 was discovered incidentally. In case 2, a periampullary pancreatic mass was diagnosed radiographically following papillotomy and stent insertion for stricture and biliary calculous disease. The histologic findings in both cases were similar, localized around a part of the biliary tract, and consisted of inspissated bile, acute and chronic inflammation, abundant lipid-laden macrophages, fibrosis, and giant cell reaction. No neoplasm was identified. On the basis of the close resemblance of these features to those seen in xanthogranulomatous cholecystitis, the lesions seen here were termed xanthogranulomatous choledochitis. In conclusion, xanthogranulomatous choledochitis is a benign inflammatory process involving the biliary tract that can form a mass lesion within the liver or pancreas and thus mimic a neoplasm. Extensive sampling of the lesion is required to rule out an underlying neoplastic process. In our patients a propensity to form lithogenic bile and a prior history of biliary tract operative procedure were present.
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ranking = 1
keywords = giant
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10/80. Osteoclastoid giant cell carcinoma of the pancreas.

    Osteoclastoid (osteoclast-like) giant cell carcinoma of the pancreas is an infrequent entity. There has long been discussion concerning its epithelial or mesenchymal origin. Whether its prognosis is better than that of other exocrine tumours of the pancreas has not yet been established. We present a case of this rare entity. The patient has remained disease-free now for 30 months.
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ranking = 5
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