Cases reported "Pancreatic Neoplasms"

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1/786. Cushing's syndrome due to ectopic adrenocorticotropic hormone production by a non-metastatic gastrinoma after long-term conservative treatment of zollinger-ellison syndrome.

    This report concerns a case of a Cushing's syndrome 10 years after first diagnosis of a zollinger-ellison syndrome within the same patient. In a 69-year-old female patient symptoms of hypergastrinaemia have been successfully treated with a proton pump inhibitor. Cushing's syndrome was the result of ectopic adrenocorticotropic hormone production by a large cystic gastrin-producing tumour of the pancreatic tail. After resection by subtotal pancreatectomy serum adrenocorticotropic hormone, cortisol, gastrin levels and secretin infusion test returned to normal. In contrast to all other previously published cases of ectopic adrenocorticotropic hormone syndrome associated with zollinger-ellison syndrome, this tumour had not metastasized into the liver and did not show local invasive growth.
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2/786. learning from case reports: diagnostic issues in an epidemiologic study of pancreatic cancer.

    epidemiologic studies on exocrine pancreatic cancer show a large heterogeneity in diagnostic criteria applied to define "caseness." Reanalyses conducted after review of diagnostic information have yielded substantially different results than those based on more crude classifications of disease. During a multicenter prospective study on mutations in the K-ras gene in pancreatic and biliary diseases, hospital diagnoses from 602 patients were reviewed by a panel of experts. There were two main motivations to do so: a generic interest for the quality of the diagnostic data, and the anticipation that a firm diagnosis could be needed to assess whether patients whose tumors did not harbor the mutation were true negatives or false negatives. In addition, the review of diagnoses was helpful to minimize tissue misclassification, and it had a high educational value for clinicians and epidemiologists. This article illustrates why and how this was so through a brief presentation of the 10 most significant cases. With respect to selection and classification of subjects, the main issues that studies on pancreatic cancer need to address are the differential diagnosis of exocrine pancreatic cancer and pancreatitis, the differential diagnosis of exocrine pancreatic cancer and other abdominal tumors, and the use of survival as a hallmark of pancreatic cancer. In epidemiologic studies of pancreatic cancer, it is warranted that a panel of experts centrally reviews all the existing diagnostic evidence (cytohistological and other) of all patients, regardless of whether they have cytohistological confirmation and of their hospital discharge diagnosis.
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3/786. Cytologic findings in noninvasive intraductal papillary-mucinous carcinoma of the pancreas. A report of two cases.

    BACKGROUND: Intraductal papillary-mucinous carcinoma of the pancreas is a new diagnostic term proposed by the 1996 world health organization classification of the exocrine pancreas. So far, there have been only a few reports concerning its cytologic findings, especially in noninvasive cases. CASES: The clinical and cytohistologic findings in two cases of noninvasive intraductal papillary-mucinous carcinoma of the pancreas were reviewed. Cytologic specimens were obtained from pure pancreatic juice in the dilated main pancreatic duct during the operation (case 1) and during endoscopic retrograde pancreatography (ERP) (case 2). Both cases showed three-dimensionally or individually scattered tumor cells with an increased nuclear/cytoplasmic ratio and prominent nucleoli. CONCLUSION: Our cases suggest that pancreatic juice cytology during ERP or surgery is useful in diagnosing pancreatic cancers and that it may detect noninvasive intraductal papillary-mucinous carcinoma of the pancreas.
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4/786. hyperinsulinism: molecular aetiology of focal disease.

    Persistent hypoglycaemia in infancy is most commonly caused by hyperinsulinism. A case is reported of the somatic loss of the maternal 11p in an insulin secreting focal adenoma in association with a germline SUR-1 mutation on the paternal allele in a baby boy with hyperinsulinism diagnosed at 49 days old. A reduction to homozygosity of an SUR-1 mutation is proposed as a critical part of the cause of focal hyperinsulinism.
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5/786. 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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6/786. Intraductal ultrasonography in six patients with endoscopic biliary stenting.

    The development of endoscopic biliary stenting (EBS), using the Wallstent, to treat patients with obstructive jaundice secondary to unresectable tumors of the pancreas or biliary ducts has led to improved quality of life in these patients. We followed six patients with intraductal ultrasonography (IDUS) after insertion of a Wallstent. In two patients, endoscopic ultrasonography (EUS) was also performed, and in three patients IDUS was repeated every few months. IDUS allowed ingrowth of the tumor or formation of debris in the stent to be observed clearly and easily. Therefore IDUS was considered to be a powerful tool to follow patients after stenting and to decide on the next treatment when reobstruction occurred.
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7/786. 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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8/786. Sclerosing mesenteritis seen clinically as pancreatic pseudotumor: two cases and a review.

    Sclerosing mesenteritis is an uncommon nonneoplastic inflammatory process in the mesentery that is seen as a pseudotumor, usually involving the small bowel mesentery, the mesenteric fat, and less commonly, the mesentery of the large bowel. We report two cases of sclerosing mesenteritis and review the literature on this rare disease. Both patients had pain, profound weight loss, and a mass on computed tomography (CT) scan of the abdomen. The provisional diagnosis was pancreatic neoplasm on the basis of clinical presentation and imaging studies. The diagnosis of sclerosing mesenteritis was established by histologic findings in biopsy material obtained at laparotomy in both cases. Interval histologic studies in one patient who had a high CA 19-9 level, progressive biliary ductal and partial duodenal compression, revealed a transitional histologic pattern from predominant inflammation and fat necrosis to predominant fibrosis. This may explain the varied descriptive terms used in the literature to describe this entity.
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9/786. Gastroenteropancreatic neuroendocrine tumor metastases to the thyroid gland: differential diagnosis with medullary thyroid carcinoma.

    neuroendocrine tumors (NET) of the thyroid gland are rare. Apart from medullary thyroid carcinoma (MTC), metastases of gastroenteropancreatic (GEP) NET may also occur. Features of six patients (five men, one female: age range, 39-67 years) with thyroid metastases from a GEP-NET are described. Thyroid metastases were bilateral in all patients and were associated with enlarged neck lymph nodes in five. In four cases, the thyroid tumor was either the first sign of the disease (n = 2) or was an isolated site of recurrence (n = 2). The tumors were well (n = 3) or poorly differentiated (n = 3). Five tumors for which the primary site could be determined corresponded to foregut-derived tumors (3 lungs, 1 thymus and 1 pancreatic NET). One tumor demonstrated calcitonin (CT) production as shown by immunohistochemistry and elevated plasma CT levels. However, the disease history and the clinical features strongly favored a metastasizing GEP-NET. No tumoral RET proto-oncogene mutation was found in this patient. The differential diagnosis between metastatic GEP-NET and MTC is crucial because prognosis, work-up, and treatment differ greatly.
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10/786. Minute carcinoma of the pancreas measuring 1 cm or less in diameter--collective review of Japanese case reports.

    BACKGROUND/AIMS: According to Tsuchiya's collective review on small pancreatic cancer measuring 2 cm or less in diameter (5), more than half of them had obstructive jaundice and the 5-year survival rate was as low as 30%. Thus, a more aggressive diagnostic approach is needed to detect a smaller and more curable cancer of the pancreas. METHODOLOGY: Thus, we collected 36 reported cases of "minute" pancreatic cancer measuring 1 cm or less in diameter, from Japanese medical literature, to analyze the relationships between the diagnostic processes and long-term results. RESULTS: Excluding 3 patients with obstructive jaundice, the other 33 patients did not show any specific initial symptoms. However, 28 (78%) out of 36 patients showed an elevation in serum pancreatic enzyme levels and/or glucose intolerance. Among the 35 patients who had received ultrasonography (US) and/or computed tomography (CT), 20 (57%) patients showed duct dilation alone, whereas only 9 patients (26%) showed tumor mass. Among 35 patients who received an endoscopic retrograde pancreatography (ERP), all patients showed positive findings such as obstruction/stenosis, filling defect or duct dilation. All 36 patients underwent pancreatectomy and the 5-year survival rate was 57%. However, the 5-year survival rate was 34% in the 13 patients with jaundice and/or tumor mass depicted in US/CT, while it was 69% for the 22 patients without these two findings (p < 0.05). CONCLUSIONS: These data lead us to conclude that an elevation of serum pancreatic enzyme levels, glucose intolerance, and duct dilation alone depicted by US/CT should not be overlooked. ERP should be more widely applied to such patients, instead of persisting in delineating the tumor mass by US/CT or follow-up by tumor marker.
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