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1/70. Pancreatic duct cell carcinoma with positive 111In octreotide uptake.

    Duct cell adenocarcinomas may produce neuroendocrine markers such as pancreatic polypeptide, gastrin and gastrin releasing hormones. A 53 year old patient, with a history of insulin dependent diabetes, was found to have a pancreatic mass which was later pathologically demonstrated to be a duct cell adenocarcinoma. The tumor produced elevated circulating neuroendocrine markers specifically gastrin and pancreatic polypeptides. An 111In octreotide imaging showed definite uptake of octreotide by the tumor. The patient was subsequently treated with somatostatin analog which resulted in the reduction of some of the circulating endocrine markers. The patient had essentially six months of asymptomatic clinical remission but then she relapsed. octreotide scanning could be useful for selected patients with pathologic diagnosis of duct cell adenocarcinoma, because some tumors may have neuroendocrine features and can be imaged, and might even respond to somatostatin analog therapy.
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2/70. 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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3/70. 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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4/70. Thyroid metastasis from intraductal papillary-mucinous carcinoma of the pancreas. A case report.

    BACKGROUND: Intraductal papillary-mucinous carcinoma (IPMC) of the pancreas is a newly identified clinicopathologic entity of the exocrine pancreas. It has been considered a slowly growing and less-aggressive carcinoma with a favorable prognosis. There have been only a few documents reporting its distant metastasis and cytologic features, with no report of thyroid metastasis until the present. CASE: A case of IPMC occurred in a 45-year-old male, who was admitted with rapid growth and tenderness of the thyroid. Abdominal computed tomography showed the typical cystic dilatation of IPMC with adjacent organ metastasis. Fine needle aspiration of the thyroid yielded papillary fronds of carcinoma cells with nuclear pleomorphism, abundant cytoplasm and prominent nucleoli in a mucinous background. Immunohistochemical findings from the skin and thyroid characterized the papillary-mucinous carcinoma as having originated in the pancreas. CONCLUSION: This case suggests that papillary carcinoma fronds aspirated from the thyroid should be further differentiated from the primary site and that a pleomorphic nucleus in a mucinous background is a useful feature to exclude a thyroid origin. Before this, distant metastasis of IPMC to the skin and thyroid has not been reported. The prognosis of IPMC with wide, distant metastasis at an advanced stage is poor.
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5/70. Mixed duct-acinar-islet cell tumor of the pancreas: report of a case.

    A 72-year-old Japanese woman presented at our hospital complaining of altered consciousness on arising every morning. The laboratory findings showed hypoglycemia and hyperinsulinemia. Abdominal ultrasonography revealed a tumor in the body of the pancreas. With a diagnosis of insulinoma, a surgical excision of the tumor was performed. A light microscopic examination and an immunohistochemical study revealed the tumor to consist of duct, acinar, and islet cell components. Mixed tumors of the pancreas are rare, and their clinical features and pathogenesis remain unclear. A further accumulation of clinical cases as well as a large number of histopathological studies on these rare mixed tumors is needed.
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6/70. 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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7/70. 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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keywords = duct
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8/70. 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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keywords = duct
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9/70. Pancreatic ductal adenocarcinoma associated with Potter type III cystic disease.

    Although polycystic liver disease (PLD) is known to be associated with autosomal dominant polycystic kidney disease, a finding of PLD with pancreatic ductal adenocarcinoma is extremely rare. We have experienced one such case of a ductal adenocarcinoma of the pancreas in a patient with Potter type III cystic disease of the liver and kidney. A 63-year-old man was admitted to our hospital because of obstructive jaundice. Six months previously, on admission to a local hospital for treatment of diabetes mellitus, he had been found to have polycystic disease of the liver and kidney. Ultrasound examination revealed dilatation of the intrahepatic bile duct and the common bile duct. blood tests showed an elevated total bilirubin level. Abdominal computed tomography scans and magnetic resonance imaging demonstrated polycystic lesions in the liver and the bilateral kidneys. Percutaneous transhepatic cholangio-drainage was performed, and fluorography of the biliary tree revealed obstruction of the lower common bile duct, causing jaundice. This appears to be a case of independent association of pancreatic ductal adenocarcinoma with polycystic disease of the liver and kidney. The patient's sister, who also had polycystic disease of the liver and kidney, had died of squamous cell carcinoma of the tongue. Although familial associations of carcinomas with polycystic liver disease may be extremely rare, they provide a perspective for the etiology of polycystic liver disease.
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10/70. insulinoma of the pancreas with insular-ductular differentiation in its liver metastasis--indication of a common stem-cell origin of the exocrine and endocrine components.

    We describe an insulinoma of the pancreas in a 56-year-old patient, which showed insular-ductular differentiation in its liver metastasis. Although the primary tumor was uniformly endocrine in nature with insulin production, the metastasis contained two distinct cell types in organoid arrangement. One cell type was insulin-positive and was arranged in islet-like structures; the other was insulin-negative but distinctly pan-cytokeratin and cytokeratin 7 positive and arranged in ducts. In the primary tumor and the metastasis, the tumor cells were surrounded by a desmoplastic stroma. As to the histogenesis of the tumor and its metastasis, we discuss the following possibilities: (1) the tumor cells might derive from a common stem cell that matures into two phenotypically different cell lines, resembling the situation in embryogenesis and (2) one tumor cell type originates from the other by transdifferentiation (metaplasia). We conclude that the parallel occurrence of endocrine and ductal differentiation supports the concept that, under certain conditions, islet cells and ductular cells may also originate from islets and that mixed endocrine/exocrine pancreatic tumors do not necessarily arise from totipotent duct cells but might also have a primary endocrine cell origin.
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