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1/42. Pancreatic adenocarcinoma in a patient with peutz-jeghers syndrome: report of a case and literature review.

    We present a rare case of pancreatic adenocarcinoma in a 47-year-old man with the peutz-jeghers syndrome. The patient underwent pancreatoduodenectomy with partial resection of the portal vein. We also review the current literature concerning peutz-jeghers syndrome associated with malignant tumors, especially pancreatic cancer. To our knowledge, this is the first report of a peutz-jeghers syndrome patient with pancreatic cancer having pancreatoduodenectomy and pathologically diagnosed with invasive ductal adenocarcinoma of the pancreas. The Peutz-Jeghers syndrome patients with pancreatic cancer were relatively young. As the pancreatic cancer in these patients was advanced and most were unresectable at diagnosis, the prognoses of these patients were extremely poor. Surgical resection offers the only chance for cure or long-term survival for peutz-jeghers syndrome patients, if the tumor is localized without distant metastasis. Therefore, screening even for young patients with peutz-jeghers syndrome is necessary for early detection of cancer.
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ranking = 1
keywords = cancer
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2/42. Biliopancreatic fistula associated with intraductal papillary-mucinous pancreatic cancer: institutional experience and review of the literature.

    Intraductal papillary-mucinous tumour is clinicopathologically characterized by papillary growth and mucin production within the pancreatic duct system. The category includes a wide range of dysplasia, ranging from adenoma to carcinoma, the latter designated as intraductal papillary-mucinous cancer. In general, the tumor renders a favorable prognosis after complete resection. However, intraductal papillary-mucinous tumor with overt invasion outside the gland has been reported to have a poor prognosis, as is the case with the usual type of duct cell cancer of the pancreas. We experienced two cases of intraductal papillary-mucinous cancer with obstructive jaundice due to impaction of thick mucus protruding from the pancreas via a "spontaneous" biliopancreatic fistula. Preoperative examinations of both patients showed a large intraductal papillary-mucinous tumor in the head of the pancreas with fistula formation between the intrapancreatic portion of the common bile duct and the main pancreatic duct. Histopathological investigation of the two resected specimens suggested that the fistula may not have developed from invasion by papillary or tubular adenocarcinoma, but from compression and destruction of the intercalating tissues by abundant mucinous secretion. The first patient died of peritoneal carcinomatosis with clinicopathologic features of pseudomyxoma peritonei 6 years after surgery. The second patient is alive and has been well for 2 years postoperatively. review of the world literature showed that half of the patients with intraductal papillary-mucinous cancer plus biliopancreatic fistula had no stromal invasion around the fistula, indicating that the fistula might have been caused by mechanical pressure. However, the other half of the cases did have stromal invasion around the fistula. Two-thirds of these cases, including our own patients, had foci of mucinous carcinoma in the stroma around the fistulization, implying that mucinous lakes in the stroma may have served as part of the "waterway" from the pancreatic duct to the bile duct, assisted by increased pressure by mucus production. Since intraductal papillary-mucinous cancer with biliopancreatic fistula has a comparatively favorable prognosis, surgical resection should be considered.
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ranking = 1.8
keywords = cancer
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3/42. Hypervascular liver metastasis from hypovascular ductal cell carcinoma of the pancreas.

    In a case of hypervascular metastatic liver tumor, the vascularity of primary focus, pancreatic carcinoma was hypovascular. Based on the imaging findings, we thought before the operation that the two lesions were double cancers. Histological examination showed that the stromal volume of metastatic tumorous tissue was richer than that of the primary focus. It was suggested that the difference in the stromal volume was related to the difference of the vascularity. Some foctors originating in stromal cells might be involved in angiogenesis.
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ranking = 0.2
keywords = cancer
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4/42. Intraductal acinar cell carcinoma of the pancreas.

    We describe a purely intraductal acinar cell carcinoma involving branch ducts of the pancreas in a 74-year-old man, which presented as recurrent episodes of acute pancreatitis. Endoscopic ultrasound examination revealed an intraductal mass bulging into the main pancreatic duct suggesting, pre-operatively, an intraductal mucinous papillary tumour. Gross examination showed several dilated branch ducts that contained haemorrhagic tumour material without any solid or true cystic formation within the pancreatic parenchyma. Using histology, a purely intraductal acinar cell carcinoma was observed, involving branch ducts only, associated with foci of carcinoma in situ in adjacent exocrine parenchyma. The main pancreatic duct was free of disease except for its communication with a cancerous branch duct. A concomitant neuroendocrine microadenoma was incidentally found during slide screening. immunohistochemistry performed on the intraductal proliferation confirmed zymogen secretion with positive staining for alpha-1 anti-chymotrypsin and anti-trypsin and the persistence of diastase-periodic acid-Schiff positive granules in the apical pole of the tumour cells. Neuroendocrine markers were negative in the acinar cell carcinoma and positive in the neuroendocrine microadenoma. To our knowledge, this is the first report of an intraductal acinar cell carcinoma of the pancreas involving branch ducts and sparing the main pancreatic duct.
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ranking = 0.2
keywords = cancer
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5/42. Two-staged treatment with local resection and percutaneous isolated hepatic chemoperfusion for advanced pancreatic cancer with multiple liver metastases: report of a case.

    There have been disappointingly few effective treatment modalities for multiple liver metastases from pancreatic cancer. Percutaneous isolated hepatic perfusion, which was developed by us for delivering dose-intensive chemotherapy to the liver, has a high efficacy in the majority of patients with multiple primary and secondary liver tumors. We herein report the first experience of a two-stage treatment with extended local resection and subsequent two percutaneous isolated hepatic perfusions for advanced pancreatic ductal adenocarcinoma with liver metastases. The second percutaneous isolated hepatic perfusion with high-dose cisplatin and mitomycin G demonstrated a distinct regression of metastatic liver tumors. Although a long-term patient survival was not obtained due to local recurrence, liver metastases have been well controlled ever since. Given that further studies establish the efficacy of percutaneous isolated hepatic perfusion also in this field, this modality would be used as prophylaxis as well as treatment of liver metastasis in patients with advanced pancreatic ductal adenocarcinoma.
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ranking = 1
keywords = cancer
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6/42. Intraductal papillary-mucinous tumors of the pancreas: presentation of eight cases.

    BACKGROUND AND STUDY AIMS: The mucinsecreting intraductal papillary-mucinous tumors of the pancreas are a rare entity with characteristic duodenoscopic and radiological features. These tumors have a broad spectrum of presentation. The diagnosis requires highly experienced and attentive endoscopists. Once diagnosed, resection of the tumor is recommended to prevent progression to malignancy. This surgical procedure has a low acceptance, mainly in the elderly patients. Therefore, a "wait and see" strategy may be necessary in those patients refusing operation. patients AND methods: Between 1996 and 2000, intraductal papillary-mucinous tumors of the pancreas were diagnosed in eight patients (5 females, 3 males) using endoscopic retrograde cholangiopancreatography, and confirmed by brush cytology and/or histology and analyzed retrospectively. RESULTS: The patients showed typical endoscopic and radiological findings: dilatation and mucin extrusion of the papillary orifice, easy passage of instruments into the dilated main pancreatic duct, which showed patchy filling defects after application of contrast medium. patients were informed about the diagnosis in detail. Two patients were inoperable. Surgery was recommended to the other six patients. Two of the six patients agreed to surgical treatment. The remaining four patients were included in a follow-up protocol. These patients were observed for up to 5 years. Only one patient showed minor progression of ERCP-findings. CONCLUSION: Intraductal papillary-mucinous tumors of the pancreas can be well characterized by means of endoscopy and radiology. Poor compliance to surgical treatment makes individual multidisciplinary management and follow-up necessary.
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ranking = 0.042970550261029
keywords = malignancy
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7/42. Invasive ductal adenocarcinoma of the remnant pancreatic body 9 years after resection of an intraductal papillary-mucinous carcinoma of the pancreatic head: a case report and comparison of dna sequence in K-ras gene mutation.

    Recently, there have been a few case reports of invasive ductal adenocarcinoma (IDC) developed in the remnant pancreas after partial pancreatectomy for intraductal papillary-mucinous neoplasm (IPMN). It is necessary to clarify their histogenetic relationships among two sporadic tumors and their surrounding duct epithelium and it would be more reliable if genetic analysis is added to the conventional histology. We report a 76-year-old woman who received pancreaticoduodenectomy for IPMN with a focal in situ carcinoma (IPMC), which was transitional to the surrounding duct epithelium with papillary proliferation and a wide variety of dysplasia. Nine years after the operation, she died of IDC in the remnant pancreatic body and its surrounding duct epithelium consisted of hyperplastic mucous cells with slight-mild dysplasia. Analysis of K-ras mutation at codon 12 (wild-GGT) by direct sequencing after polymerase chain reaction indicated that their transitioning patterns differed from each other: CGT in IPMC; no mutation in the mildly dysplastic duct epithelium around IPMC; GAT in IDC of the remnant pancreas; and AGT in mucous cell hyperplasia with mild dysplasia close to the IDC. This is the first report in which the dna sequence of K-ras mutation was determined for the two sporadic pancreatic cancers and surrounding duct changes. The following two suggestions are made: (1) the cell-origin might have differed between the two types of cancer (IDC and IPMC); and (2) no precursor lesion toward IDC or IPMC was identified in their surrounding duct epithelium.
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ranking = 16.902228988078
keywords = neoplasm, cancer
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8/42. Intraductal papillary and mucinous pancreatic tumour: a new extracolonic tumour in familial adenomatous polyposis.

    Familial adenomatous polyposis (FAP) is characterised by the development of numerous colorectal adenomatous polyps. Other extracolonic benign or malignant lesions have been reported previously in association with FAP but precancerous lesions in the pancreas have never been described. We report the first case of intraductal papillary and mucinous pancreatic tumour (IPMT) in a patient with FAP. A 48 year old man with a well documented past history of FAP was admitted for epigastric pain, weight loss, and new onset diabetes mellitus. Spiral computed tomography scan revealed a large tumour in the pancreatic head with upstream main pancreatic duct dilatation. Endoscopic ultrasonography confirmed these data. Mucous secretion was seen at duodenoscopy and a lesion in the main pancreatic duct was confirmed by retrograde pancreatography. The patient underwent a pancreaticoduodenectomy for suspected IPMT. Histological examination of the resected specimen confirmed an IPMT with in situ carcinoma. Twelve months after resection, the patient remained free of tumour relapse. Genetic analysis showed loss of the wild allele of the adenomatous polyposis coli gene in IPMT, causing inactivation of both alleles and demonstrating that IPMT was not incidental in this patient. IPMT should be included in the extracolonic localisation of FAP.
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ranking = 0.2
keywords = cancer
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9/42. carcinosarcoma of the pancreas: a case report and review of the literature.

    We report the case of a 74-year-old white man with a mass in the head of the pancreas, which was found incidentally on computerized tomographic scan during a workup for deep vein thrombosis. endoscopy with pancreatic duct brushings yielded a diagnosis of adenocarcinoma. A pancreaticoduodenectomy followed, with complete resection of the tumor. Pathologic examination showed 2 distinct components. One component was a conventional infiltrating pancreatic ductal adenocarcinoma, and the other component was high-grade sarcoma with features of malignant fibrous histiocytoma. To our knowledge, this carcinosarcoma is the seventh reported case of a primary pancreatic neoplasm with mixed carcinomatous and sarcomatous elements.
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ranking = 16.502228988078
keywords = neoplasm
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10/42. Mucin-secreting bile duct adenoma--clinicopathological resemblance to intraductal papillary mucinous tumor of the pancreas.

    We describe a case of mucin-secreting biliary adenoma associated with obstructive jaundice due to mucin production. Duodenal endoscopy revealed mucin secretion through an enlarged papilla of Vater. On the cholangiogram, large filling defects caused by mucin were observed in the dilated bile duct. Granular and/or papillary mucosa was noted from the hilar to the common bile duct by using percutaneous transhepatic cholangioscopy. biopsy specimens taken from the papillary mucosa revealed slightly papillary projection without any evidence of malignancy. This lesion was diagnosed as mucin-secreting superficial adenoma and pancreatoduodenectomy was performed according to the malignant behavior. Using a histologic examination of the specimen, pure adenoma was documented. Since the surgical margin was positive, radiation was added for the residual adenoma in the intrahepatic bile ducts. The patient has been well without any evidence of recurrence for 21 months since her operation. It is only recently that the term 'intraductal papillary mucinous tumor (IPMT)', which is accepted as a distinct disease entity of pancreas, has begun to be used for mucin-secreting bile duct tumor. This case also seemed to clinicopathologically resemble an IPMT of the pancreas.
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ranking = 0.042970550261029
keywords = malignancy
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