Cases reported "Adenoma, Islet Cell"

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1/59. Ultrasound-guided laparoscopic resection of pancreatic islet cell tumors.

    Pancreatic islet cell tumors represent a diverse group of neuroendocrine lesions. These tumors may be singular or multiple, benign or malignant, sporadic, or part of the constellation of multiple endocrine neoplasia type 1. Tumors such as insulinomas and gastrinomas produce gastrointestinal peptides that lead to diagnosis. Nonfunctioning lesions may be found incidentally or by screening patients at high risk for such tumors. Successful management of patients with pancreatic islet cell tumors relies on accurate localization and sound operative technique. With proper preoperative localization, advanced laparoscopic methods can be used to manage patients with these pancreatic neoplasms. Preoperative localization of pancreatic islet cell tumors was difficult in the past. Standard imaging and localizing modalities, such as computed tomography scanning, magnetic resonance imaging, angiography, transabdominal sonography, and portal venous sampling, yield only 24% to 75% accuracy. Consequently, many biochemically suspected lesions cannot be imaged with current techniques. Decreased tactile sensation of laparoscopy adds complexity to intraoperative identification. Endoscopic sonography and laparoscopic sonography provide accurate preoperative and intraoperative localization to enhance laparoscopic and open resection. The authors treated two patients with islet cell neoplasms using endoscopic sonography to preoperatively visualize the tumors and laparoscopic sonography to guide laparoscopic enucleation. Their approach and difficulties are discussed.
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ranking = 1
keywords = neoplasm, complex
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2/59. Malignant islet cell tumor with sarcomatous differentiation.

    Malignant mesenchymal neoplasms of the pancreas are rare and malignant islet cell tumors with sarcomatous dedifferentiation are rarer still. We present a case of malignant islet cell tumor with sarcomatous differentiation, which to our knowledge is only the second reported case showing such a combination of morphologic features. Clinically, the neoplasm was not hormonally active and immunohistochemical staining was negative for gastrin, glucagon, insulin and somatostatin. The sarcomatous component strongly reacted with an antibody directed against vimentin, and a minority of cells stained strongly with antisera directed against desmin and smooth muscle actin. The spindle cell component was nonreactive with antibodies directed against factor viii. The myogenous direction of differentiation in the present tumor is similar to that seen in the prior case report of malignant islet cell tumor with rhabdomyosarcomatous differentiation.
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ranking = 0.9956144868012
keywords = neoplasm
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3/59. Overexpression of the Sm-like proto-oncogene in primary and metastatic pancreatic endocrine tumors.

    CONTEXT: The cancer associated Sm-like proto-oncogene mRNA has been found to be overexpressed in the majority of pancreatic adenocarcinomas and is necessary for the transformed phenotype in pancreatic cancer cell lines. However, expression levels have not been examined in other types of pancreatic neoplasms, such as pancreatic endocrine tumors. SETTING: Fifteen primary pancreatic endocrine tumors, including five insulinomas and 10 non-functioning tumors, along with seven hepatic metastatic pancreatic endocrine tumors. MAIN OUTCOME MEASURES: Quantitative expression levels of cancer associated Sm-like mRNA were measured by real-time PCR. Overexpression was defined as a two-fold or greater value when compared to the expression levels found in normal pancreatic islet cells obtained from healthy donors. RESULTS: In primary tumors, four of the 10 non-functioning pancreatic endocrine tumors were found to overexpress cancer associated Sm-like mRNA (40%). Three of the five (60%) insulinomas also overexpressed cancer associated Sm-like mRNA. In total, cancer associated Sm-like mRNA was overexpressed in seven of 15 primary tumors (47%) and in the majority (71%, 5 of 7) of the hepatic metastases. CONCLUSIONS: Our results indicate that the cancer associated Sm-like mRNA gene may also play a role in the tumorigenesis of pancreatic endocrine tumors.
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ranking = 0.4978072434006
keywords = neoplasm
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4/59. ghrelin expression in islet cell tumors: augmented expression of ghrelin in a case of glucagonoma with multiple endocrine neoplasm type I.

    ghrelin is a 28-amino acid peptide that regulates GH release together with GHRH and somatostatin. The expression of ghrelin has been detected in the stomach, small intestine, hypothalamus, pituitary gland, kidney, placenta, and testis. Recently it was reported that ghrelin is present in pancreatic alpha-cells and that it stimulates insulin secretion. In this study, we examined the ghrelin expression in two cases of glucagonoma and two cases of insulinoma by Northern blot analysis and immunohistochemistry. ghrelin expression was identified in a case of glucagonoma associated with multiple endocrine neoplasm type I both by Northern blot analysis using total rna and by immunohistochemistry, although the plasma ghrelin level was not elevated. This is the first case of tumor in which ghrelin gene expression was detected by Northern blot analysis using total rna.
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ranking = 2.489036217003
keywords = neoplasm
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5/59. Solid adenoma with exclusive hepatocellular differentiation: a new variant among pancreatic benign neoplasms?

    We report a unique, previously unreported pancreatic tumor with hepatoid differentiation associated with serous microcystic adenoma in a 70-year-old man. These two lesions localized, respectively, at the body and the tail of the pancreas, were found incidentally on abdominal ultrasonography. serum alpha-fetoprotein was not increased and no hepatic lesion was displayed on computed tomography. A subtotal pancreatectomy with splenectomy was performed. The patient is alive and well 12 months after resection. Pathological examination showed a very unusual encapsulated solid tumor with hepatocytic differentiation, bile production and immunoreactivity for hepatocyte paraffin-1 antibody. The tumor cells were negative for endocrine (neuron-specific enolase, chromogranin a, synaptophysin) and acinar (amylase, trypsin) markers. Ultrastructurally, zymogen and neurosecretory granules were absent. The features of the tumor were almost indistinguishable from those of hepatocellular adenoma; therefore, we believe that this solid hepatoid tumor may represent a variant of pancreatic adenoma. Recognition of this entity is important because the only reported pancreatic hepatoid tumors to date have been malignant. The main differential diagnoses include hepatoid ductal adenocarcinoma, hepatoid acinar cell carcinoma, primitive hepatoid endocrine tumor, and metastatic hepatocellular carcinoma.
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ranking = 1.9912289736024
keywords = neoplasm
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6/59. Primary uterine tumors and multiple endocrine adenomatosis, type I.

    Multiple endocrine adenomatosis, Type I was initially diagnosed in a 35-year-old woman with primary chief cell hyperplasia of the parathyroids. Approximately 5 years later, vaginal bleeding developed and a well-differentiated endometrial adenocarcinoma was recognized. An adenomatoid tumor of the uterus was discovered in addition to a nonfunctional islet cell tumor of the pancreas. Multiple endocrine adenomatosis is reviewed in relation to possible gynecologic neoplasms.
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ranking = 0.4978072434006
keywords = neoplasm
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7/59. Cystic islet cell tumors of the pancreas. A clinico-pathological report of two nonfunctioning cases and review of the literature.

    Cystic islet cell tumors of the pancreas are extremely rare. The authors report their personal experience with two cases of nonfunctioning cystic endocrine neoplasms. The tumor was diagnosed preoperatively in one case by ultrasonography (US)-guided fine-needle aspiration cytology, while in the other it was identified only in the surgical specimen after a clinical-radiologic diagnosis of pancreatic mucinous cystic tumor. Immunohistochemical assay showed positivity for the generic neuroendocrine markers (neuron specific enolase, or NSE, synaptophysin, and chromogranin a) in both cases and also for glucagon in one case. The neoplasms were resected by distal pancreatectomy with splenectomy and intermediate pancreatectomy respectively. Both patients are alive and recurrence-free 6 mo and 2.5 yr, respectively, after surgery. The authors also review the existing literature, discussing the pathogenesis of such tumors and the imaging techniques and surgical strategies adopted in their management.
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ranking = 0.9956144868012
keywords = neoplasm
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8/59. A 20-year review of pediatric pancreatic tumors.

    Pancreatic tumors are rare surgical problems in infants and children. A 20-year audit (1971 to 1991) of this institution showed six patients ranging in age from 3 weeks to 16 years who were operated on for pancreatic neoplasms. Five of these tumors were malignant, bringing the reported experience to 71 cases. This series of malignancies included three solid cystic tumors, one insulin-secreting tumor, and one pancreatoblastoma. The clinical presentations varied: three had abdominal pain, one developed hypoglycemia, and one had an abdominal mass with jaundice. In five of the six patients pancreatic pathology was suspected preoperatively. All were treated primarily with pancreatic resection including one pancreatoduodenectomy. No radiotherapy or chemotherapy was used. The perioperative mortality was 0% with a morbidity of 50%. The long-term results are encouraging, with all patients alive after a mean follow-up of 7.8 years. These data suggest that aggressive surgical therapy is warranted in the management of pediatric pancreatic tumors.
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ranking = 0.4978072434006
keywords = neoplasm
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9/59. Acinar cell carcinoma of the pancreas.

    A case of acinar cell carcinoma of the pancreas is presented. The differential diagnosis is discussed and includes endocrine neoplasm of the pancreas (islet cell and carcinoid tumor) and a poorly differentiated adenocarcinoma. The separation of the various entities by light microscopic, histochemical, immunocytochemical, and ultrastructural methods is described. The acinar cell carcinoma characteristically is positive for pancreatic digestive enzymes by immunocytochemistry, and at the ultrastructural level zymogen-type granules can be demonstrated; these have a tendency to aggregate in the apical region of acinar structures. Clinically the acinar cell carcinoma is an aggressive malignant neoplasm that may present with a characteristic syndrome of disseminated fat necrosis. survival is generally less than 1 year.
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ranking = 0.9956144868012
keywords = neoplasm
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10/59. Extensive acrochordons and pancreatic islet-cell tumors in tuberous sclerosis associated with TSC2 mutations.

    Acrochordons are frequently encountered benign skin lesions that may occasionally represent underlying pathology. Pancreatic islet-cell tumors are rare neoplasms and few cases have been described in patients with tuberous sclerosis complex (TSC). A 39-year-old man presenting in acute renal failure was referred to us for further diagnostic evaluation of coincidentally noted dysmorphic features. physical examination revealed over 1,000 acrochordons in addition to findings meeting criteria for TSC. The diagnosis was confirmed by disclosure of mutation in the TSC2 gene. Further evaluation revealed pancreatic islet cell tumors. Acrochordons are a common skin lesion, but when presenting in an atypical manner or unusual number may be a sign of TSC and underlying occult pathology thereby warranting evaluation of TSC2. Additionally, mutations in TSC2 gene may be a risk factor for developing pancreatic islet-cell tumors.
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ranking = 0.5021927565994
keywords = neoplasm, complex
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