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1/83. adenoma of the ampulla of vater: a genetic condition?

    The etiology of adenoma of the ampulla of vater is not well understood. Previous authors reported the association of this neoplasm with polycystic kidney disease of two fraternal sisters. They concluded that these two conditions were somehow related. We describe a case of ampullary adenoma associated with polycystic kidney disease. This presentation raises again the question of a possible link between these two diseases.
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2/83. brain metastases from adenoendocrine carcinoma of the common bile duct: a case report.

    A 68-year-old man with metastatic brain tumors from adenoendocrine carcinoma of the common bile duct is reported. A common bile duct tumor and a metastatic liver tumor had been resected 6 years and 3 years prior to admission, respectively. Microscopically they showed two components; moderately differentiated tubular adenocarcinoma and neuroendocrine carcinoma. He presented with headache and vomiting and MRI revealed two metastatic brain tumors. They were successfully resected and radiotherapy was carried out. Histological diagnosis of the metastatic brain tumors was neuroendocrine carcinoma, but carbohydrate antigen (CA)-19-9 and carcinoembryonic antigen (CEA)-immunoreactive cells were observed without glandular pattern. Immunohistochemically serotonin and pancreatic polypeptide were detected, but somatostatin was not. As the endocrine cells demonstrated in the normal extrahepatic bile ducts are only somatostatin-containing D cells, these cells are considered to originate as part of a metaplastic process. To our knowledge, this represents the second case of adenoendocrine carcinoma of the common bile duct.
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3/83. adenomyoma of the common bile duct.

    An unusual case of adenomyoma of the common bile duct is reported. A 31 year-old woman with intermittent epigastralgia was found to have cholestasis and stenosis of the common bile duct. A malignant tumor could not be excluded. Resection of the common bile duct with the tumor was performed with choledochojejunostomy reconstruction. The tumor consisted of glandular hyperplasia and proliferation of smooth muscle fibers and fibrous connective tissue. The patient was well at the 30-month follow-up. Although the benign tumors of the extrahepatic bile ducts are rare, the clinical importance lies in the recognition that they can cause biliary tract obstruction. Intra-operative frozen section for histologic examination is required to make the diagnosis and plan the surgical procedure. Complete extirpation of the lesion is recommended. A radical operation is not necessary.
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4/83. Papillary adenoma of the distal common bile duct.

    A 73-year-old man with a papillary adenoma located in the distal common bile duct is reported. He underwent pylorus-preserving pancreatoduodenectomy. The lesion in the common bile duct featured papillary proliferation of the epithelium and fibrous elements with diffuse infiltration by inflammatory cells. Positive staining for MIB-1 (Ki-67) and p53 was identified in the nuclei of the proliferative epithelium. These findings suggested the malignant potential of this lesion. Further progress in imaging diagnostic techniques should increase the frequency with which such lesions are discovered. Even now, if mural irregularities and defects are found in the extrahepatic biliary system, especially the distal common bile duct, the possibility of such borderline biliary adenoma should be taken into consideration when making a diagnosis.
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ranking = 3
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5/83. Unusual manifestation of an ampullary tumor presenting with severe upper gastrointestinal bleeding.

    Ampullary tumors can occasionally ulcerate and present as frank gastrointestinal bleeding. The most common clinical presentation is jaundice like in other tumors of the biliary tree. We report on a 68-year-old man who presented with severe upper gastrointestinal hemorrhage secondary to an asymptomatic mass of the ampulla of vater. An endoscopic biopsy specimen revealed a villous adenoma with moderate dysplasia. A curative resection was performed, and pathological work-up revealed the presence of an infiltrating, moderately differentiated ampullary adenocarcinoma.
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6/83. Composite neuroendocrine and adenocarcinoma of the common bile duct associated with clonorchis sinensis: a case report.

    The biliary tract has neuroendocrine cells of endoderm origin similar to the gastrointestinal tract, however neuroendocrine tumors of the biliary tract are rare. We report a composite glandular-endocrine cell carcinoma of the common bile duct in a 64-year-old Korean man which was associated with clonorchis sinensis. The patient complained of right upper quadrant abdominal pain. Several parasites of clonorchis sinensis were removed during the percutaneous transbiliary drainage. Endoscopic retrograde cholangiopancreatography revealed a polypoid mass (3 x 3 cm) with central ulceration in the common bile duct. pancreaticoduodenectomy was performed. Microscopic examination of the tumor revealed a composite small cell neuroendocrine carcinoma and adenocarcinoma. The small cell carcinoma component showed positive reaction to chromogranin a and neuron-specific enolase and it was located mainly in the deeper portion of the mass. The well-differentiated adenocarcinoma component showed a positive reaction to carcinoembryonic antigen and it was situated in the superficial portion of the mass. Exclusively, the small cell component metastasized to the lymph node. It is suggested that this tumor could arise from a multipotential stem cell and showed neuroendocrine and glandular differentiation and that clonorchis sinensis could be a predisposing factor, as in cholangiocarcinoma.
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7/83. Ampullectomy of carcinoma of the papilla of vater in an elderly patient without jaundice.

    A 79 year-old woman was admitted to Aioi City Hospital for a closer examination of hepatic dysfunction. A filling defect was observed at the distal end of the intrapancreatic common bile duct by computed tomography combined with drip infusion cholangiography. The diagnosis of adenoma with dysplasia at the papilla of Vater was obtained by a biopsy performed during duodenoscopy. As a result, we performed an ampullectomy. Histologic examination revealed a papillary adenocarcinoma which partly extended just beyond the muscle of Oddi. The patient made an uneventful recovery and was discharged on the 35th postoperative day. Here, based upon our experience, we discuss such problems as the accuracy of preoperative diagnosis and the indications for ampullectomy.
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8/83. A case of adenoma of the ampulla of vater.

    A case of adenoma of the ampulla of vater is presented in a 48-year-old man who was admitted with epigastric pain. Gastroendoscopy revealed a sessile tumor about 3 cm in diameter at the ampulla of vater, and the pathological diagnosis of a biopsy specimen was adenoma with moderate atypia. Because of the possibility of a malignant component, pylorus-preserving pancreatoduodenectomy was performed to avoid local recurrence. The final pathological diagnosis was adenoma with severe atypia.
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ranking = 3.5
keywords = adenoma
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9/83. Pancreatobiliary adenosquamous carcinoma (report of two cases).

    Adenosquamous carcinomas are rare malignant tumors of the pancreas and periampullary region. We present two cases of pancreatic and periampullary adenosquamous carcinoma with clinicopathologic, histo- and immunohistochemical findings. A 51-year-old and a 48-year-old man presented with right upper quadrant pain of three months duration and jaundice for two weeks. Both cases had an elevation of liver enzymes and CA 19-9. In the first case, computerized tomography showed a 3 cm-mass at the pancreatic head. In the second one, endoscopic retrograde choledocopancreatography (ERCP) revealed a mass at the ampulla Vateri localization. A pancreatoduodenectomy was performed for both cases. Gross pathologic examination displayed a solid, gray-white colored 4 cm-tumor at the pancreatic head and a solid, pink-white colored, 2 cm-tumor at the periampullary region bulging into the duodenal lumen. Microscopically, both tumors were composed of solid nests of squamous cells with pearl formation and mucin-containing glandular cells and diagnoses were adenosquamous carcinoma. Furthermore, histo- and immunohistochemical findings were consistent with microscopic diagnoses.
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10/83. Ampullary adenocarcinoma in neurofibromatosis type 1. Case report and literature review.

    Periampullary tumors in patients affected by Neurofibromatosis Type 1 (NF-1) are usually carcinoids or stromal tumors and, rarely, adenocarcinomas. We report a case of an adenocarcinoma of the ampulla of vater in a 54-year-old woman with NF-1 admitted to the hospital with jaundice and undergoing pancreato-duodenectomy. Histologically, the resected specimen showed an adenocarcinoma of the ampulla as being a part of a complex atypical epithelial proliferation extended from the papilla to the mucosa of the duodenum and distal choledochus, islet-cell adenomatosis of the pancreas and multiple gastric, duodenal, jejunal stromal tumors. The ampullary and periampullary adenocarcinomas in NF-1 patients have peculiar features, suggesting a widespread predisposition to cancer development in periampullary tissues and requiring widely demolitive surgery. Moreover, they occur at a younger age than those occurring in non-NF-1 patients, may be associated with additional periampullary epithelial tumors, are often operable and may present long survival.
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keywords = adenoma
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