1/88. Cytogenetic and CGH studies of four neuroendocrine tumors and tumor-derived cell lines of a patient with multiple endocrine neoplasia type 1.A malignant insulinoma (LOHG-I), a carcinoid of the lung (LOHG-L), a parathyroid adenoma (LOHG-NSA), and a fibroma (LOHG-F) were obtained from a patient with multiple endocrine neoplasia type 1 (MEN1). Long-term cultures were established. Essential neurobiological properties of the cell lines were proven immunocytochemically and by electron microscopy. Molecular analysis of the germline dna showed a 4 bp deletion in exon 3 of the MEN1 gene. Cytogenetic and CGH analyses of the tumors/tumor cell lines revealed diploidy and balanced and unbalanced structural aberrations different for each tumor. chromosomes 6q21, 11q and 17q were most frequently involved in clonal structural aberrations.- - - - - - - - - - ranking = 1keywords = multiple endocrine, endocrine neoplasia, multiple endocrine neoplasia, endocrine, neoplasia (Clic here for more details about this article) |
2/88. Giant insulinoma in a patient with multiple endocrine neoplasia-type I: a case report.We report a case of giant cystic insulinoma constituting part of multiple endocrine neoplasia (men) type I. A 29-year-old Japanese man presented with a history of recurrent hypoglycemic attacks. Endocrine examination showed hyperinsulinemia discordant with hypoglycemia, and a giant cystic insulinoma (11 x 10 cm) located in the pancreatic tail was detected radiologically. hyperprolactinemia due to pituitary adenoma and hyperparathyroidism due to parathyroid hyperplasia were also present. The insulinoma, prolactinoma and hyperplastic parathyroid gland were surgically removed. Fluorescent microsatellite analysis detected loss of heterozygosity (LOH) in chromosome 11q13 in dna samples from all resected tissues but not from white blood cells. This is a rare case of men type I because of the giant cystic insulinoma and the evidence of common LOH detected in all men type I tissues.- - - - - - - - - - ranking = 0.93394663482024keywords = multiple endocrine, endocrine neoplasia, multiple endocrine neoplasia, endocrine, neoplasia (Clic here for more details about this article) |
3/88. Laparoscopic management of neuroendocrine tumours of the pancreas.Neuroendocrine tumours of the pancreas are rare but can be fatal due to excessive secretion of regulatory peptides. The localization of these small tumours can be difficult while open surgical treatment is associated with a relatively high morbidity. Two cases are presented where endocrine tumours of the pancreas were successfully removed by laparoscopy. In the surgical treatment of endocrine pancreatic tumours, the use of laparoscopic techniques can provide a valuable means for localizing the tumour while improving the patient's comfort and decreasing operative morbidity.- - - - - - - - - - ranking = 0.11559338906458keywords = endocrine (Clic here for more details about this article) |
4/88. Malignant insulinoma which expressed a unique creatine kinase isoenzyme: clinical value of arterial embolization as a palliative therapy.A 76-year-old man with hypoglycemic coma was diagnosed as malignant insulinoma with multiple hepatic metastases. Embolization was done for two-thirds of the hepatic mass and it rapidly lowered the serum immunoreactive insulin. He was discharged without medication and has been free from hypoglycemia. After the embolization, the serum creatine kinase (CK) level increased transiently although there was no evidence of myocardial infarction. On electrophoresis, the CK activity showed an abnormal peak, suggesting mitochondrial CK. CK release after embolization has been reported in only a few cases with endocrine tumors, which might indicate some relationship between active energy metabolism and mitochondrial CK.- - - - - - - - - - ranking = 0.01651334129494keywords = endocrine (Clic here for more details about this article) |
5/88. neuroendocrine tumors of the pancreas.neuroendocrine tumors of the pancreas (NETP) are rare. We report our surgical experience of 11 patients with NETP. These included 5 patients with benign insulinomas. Raised serum insulin and c-peptide levels with hypoglycemia were always diagnostic. ultrasonography, CT, visceral angiography, arterial stimulation and venous sampling, and intraoperative ultrasound localized the tumor in 0/5, 1/5, 3/4, 2/2, 3/3 cases, respectively. The 6 other malignant NETP (one gastrinoma, 2 carcinoids, 3 non-functioning) were managed by pancreatic resection (Whipple's operation = 3, distal pancreatectomy with total gastrectomy = 1, total pancreatectomy = 1, distal pancreatectomy with left nephrectomy and proximal gastrectomy = 1). Two patients died postoperatively. We had 5 major and 2 minor postoperative complications, with 2 deaths. During follow up from 1 to 7 years, one patient with malignant carcinoid tumor died at two and half years, of local recurrence. The other 8 patients are disease-free with good quality of life.- - - - - - - - - - ranking = 0.082566706474698keywords = endocrine (Clic here for more details about this article) |
6/88. insulinoma in childhood.A 9-year-old boy with convulsions is herein described. He was diagnosed and treated for epilepsy and insufficient adrenal function for four years with no response. hypoglycemia from hyperinsulinism was found and the source of the hyperinsulinism was a tumor of the tail of the pancreas--located by computerized tomographic scan and magnetic resonance imaging. Distal pancreatectomy was performed with good results. histology of the tumor showed islet cell tumors with capsular invasion. For this type of patient, long-term follow-up should include: prevention of metastasis or recurrence, and testing for multiple endocrine neoplasia type 1.- - - - - - - - - - ranking = 0.18678932696405keywords = multiple endocrine, endocrine neoplasia, multiple endocrine neoplasia, endocrine, neoplasia (Clic here for more details about this article) |
7/88. insulinoma with subsequent association of zollinger-ellison syndrome.We report a patient with insulinoma associated with zollinger-ellison syndrome. A 67-year-old woman was first admitted to our hospital for an abdominal mass. Abdominal computed tomography (CT) revealed a large pancreatic tumor, which was then diagnosed as an unresectable pancreatic adenocarcinoma. At the age of 71, she presented symptoms of hypoglycemia. fasting blood glucose was 21 mg/dl and plasma immunoreactive insulin level was 846 microU/ ml. plasma gastrin, glucagon, vasoactive intestinal polypeptide and somatostatin levels were all normal. At the age of 73, hypoglycemic attacks occurred more frequently and she was admitted to our hospital. Abdominal CT scan showed multiple liver metastases. Chemotherapy with 5-fluorouracil and doxorubicin was performed. Three months later, she had an emergency laparotomy because of a perforated duodenal ulcer. plasma gastrin level was 1,960 pg/ml at that time. Gastric hypersecretion was well controlled with a proton pump inhibitor (lansoprazole) but she died of widespread cancer dissemination 8 years after her first admission. On autopsy, histologic examination revealed a mixed acinar-endocrine carcinoma of the pancreas. Immunohistochemical stains were positive for insulin, gastrin, and alpha1-antitrypsin.- - - - - - - - - - ranking = 0.01651334129494keywords = endocrine (Clic here for more details about this article) |
8/88. Unusual presentation of a pancreatic insulinoma in helical CT and dynamic contrast-enhanced MR imaging: case report.Insulinomas are pancreatic neoplasms that can be radiologically characterized typically because of their tendency to present intense and early contrast enhancement with a wash-out phenomenon. In this sense, we report an unusual case of a hypovascular solid pancreatic insulinoma confirmed with surgery and pathologic analysis, in a patient with normal serum insulin levels. In the two-phase helical CT, the mass behaved as a hypodense lesion with respect to the surrounding pancreatic parenchyma during the arterial phase and as a hypointense lesion during the dynamic contrast-enhanced MR imaging. Pathologic examination demonstrated a hypercellular tumor with poor vascularization of intervening stroma which showed prominent amyloid deposits.- - - - - - - - - - ranking = 1.7022296318522E-6keywords = neoplasm (Clic here for more details about this article) |
9/88. 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.- - - - - - - - - - ranking = 0.13210673035952keywords = endocrine (Clic here for more details about this article) |
10/88. Malignant insulinoma with hepatoid differentiation: a unique case with alpha-fetoprotein production.Hepatoid carcinomas are uncommon extra-hepatic neoplasms exhibiting features of hepatocellular carcinoma and they are most frequently described in the stomach. We report a 64-year-old woman with a malignant insulinoma showing focal hepatoid differentiation and biochemical evidence of alpha-fetoprotein (AFP) production. The current case is the first malignant insulinoma with hepatoid differentiation. Resection of the primary tumor followed by regional embolization was peformed. The patient died 22 months after initial presentation. Thus, the presence of hepatoid differentiation in pancreatic tumor should be noted as the tumor may be associated with elevated AFP. The features of pancreatic hepatoid carcinomas are discussed.- - - - - - - - - - ranking = 1.7022296318522E-6keywords = neoplasm (Clic here for more details about this article) |
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