1/82. 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 = endocrine (Clic here for more details about this article) |
2/82. 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 = 0.55555555555556keywords = endocrine (Clic here for more details about this article) |
3/82. 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 = 0.77777777777778keywords = endocrine (Clic here for more details about this article) |
4/82. 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 = 0.11111111111111keywords = endocrine (Clic here for more details about this article) |
5/82. 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 = 0.55555555555556keywords = endocrine (Clic here for more details about this article) |
6/82. 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 = 0.11111111111111keywords = endocrine (Clic here for more details about this article) |
7/82. 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 = 0.11111111111111keywords = endocrine (Clic here for more details about this article) |
8/82. 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 = 0.88888888888889keywords = endocrine (Clic here for more details about this article) |
9/82. insulinoma with fibrillar inclusions and acinar cell elements.Islet cell tumors associated with exocrine elements are rare. An insulinoma was removed from the head of the pancreas of a 33-year-old woman. Ultrastructural and immunohistochemical studies demonstrated that, in addition to the endocrine cells, the tumor had a small population of cells with an acinar cell morphology. Rare cells exhibiting both endocrine and exocrine features (amphicrine cells) were also identified. Another unusual finding in this case was the presence of a large number of intracytoplasmic filamentous inclusions that, even though they have been observed in other neoplasms, have not previously been reported in endocrine tumors of the pancreas. The demonstration of cells with mixed endocrine features supports the concept that both the endocrine and exocrine portions of the components of the pancreas have a common embryologic origin.- - - - - - - - - - ranking = 0.55555555555556keywords = endocrine (Clic here for more details about this article) |
10/82. radio-frequency ablation for symptom control in a patient with metastatic pancreatic insulinoma.Malignant insulinomas are very rare endocrine tumours with a variable clinical course. We describe a 51-year-old man who had two large insulinomas resected from the body of the pancreas and 19 years later, having again become symptomatic, was found to have hepatic metastases. Medical treatment with diazoxide and octreotide failed to control his symptoms, but repeated hepatic embolization effected both symptomatic and biochemical improvements for a further 5 years. When symptoms recurred but further embolization failed to control his symptoms the hepatic metastases were treated by outpatient percutaneous radio-frequency ablation. He remains symptom-free 18 months later and levels of insulin and pro-insulin have nearly normalized. The survival, with liver metastases, for 27 years in a man with a malignant insulinoma has not been described previously. Malignant insulinoma may follow a rather indolent course and symptoms respond well to locally destructive therapies. Hepatic embolization is less traumatic than hepatic lobe resection and radio-frequency ablation offers an alternative if vascular access to the tumour is no longer possible.- - - - - - - - - - ranking = 0.11111111111111keywords = endocrine (Clic here for more details about this article) |
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