Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twelve patients with Zollinger-Ellison syndrome and one patient with WDHA syndrome are reviewed. Three of the Z-E patients exhibited MEA, two having hyperinsulinism and one hyperparathyroidism. Ages ranged from nine to 71 years. Diagnosis of Z-E syndrome was established from history, gastric acid secretion, radiologic studies, serum gastrin measurements and from actual tissue biopsy in 10 of the 12 patients. Total gastrectomy was performed in 8 of the 12 Z-E patients, with abolition of the ulcer diathesis in all. However, in none of our patients was there objective evidence of subsequent tumor regression. Three patients remain alive. Four died of tumor, one from post-total gastrectomy complications, one from post-subtotal gastrectomy in another hospital, two from ulcer hemorrhage, and one from electrolyte imbalance with autopsy diagnosis of Z-E tumor. A patient is recorded in detail who exhibited both hyperinsulinemia and hypergastrinemia from a malignant islet cell tumor, had the tumor "debulked" four times over a 14 year period and whose hepatic metastases were temporarily abolished by streptozotocin infusion. The question is raised regarding relationships between chronic organic hyperinsulinism and subsequent hypergastrinemia.
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PMID:Zollinger-Ellison syndrome: special considerations. 14 Jun 31

A 10-year series of patients operated for insulinoma at Sahlgren's Hospital, Gothenburg, is presented. Twelve patients (three men, nine women) aged 26--70 years are included in the material. The patients had a history of recurrent hypoglycemic symptoms of 1 month to 20 years. Hyperinsulinism was established by determinations of blood glucose and plasma insulin in the fasting state. Selective angiography could show the tumor in 3 out of the 12 patients. A through pancreatic mobilization and palpation was performed during operation. Solitary pancreatic tumor was found in 10 patients, and 1 of them had also metastases in the liver. One patient had two tumors, one in the head and one in the tail of the pancreas. In one patient it was not possible to find any tumor at operation. Distal pancreatic resection and splenectomy were performed when the tumor was localized in the body or tail or when no tumor was found. Tumours in the head were excised locally. Microscopy showed insulinoma without malignancy in 10 patients, malignant tumor in insulae with metastases in the liver in 1 patient, and multiple adenomatosis of insulae in the patient without any palpable tumor. The 11 patients with benign disease were examined 1--10 years after the operation and had no signs of hyperinsulinism or of diabetes.
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PMID:A ten-year material of insulinoma: diagnosis and surgical treatment. 23 Dec 95

Among 33 patients with endocrine pancreatic tumors due to multiple endocrine neoplasia type 1 (MEN-1), 19 (58%) patients had hypergastrinemia, 7 (21%) patients had hyperinsulinism, and 7 (21%) patients had clinically non-functioning lesions. At least one gross tumor was found in all patients undergoing pancreatic surgery, including those with negative localization studies prior to operation. The patients also had additional macroscopic tumors as well as numerous microadenomas, and the lesions frequently were positive for immunostaining with multiple hormones, mainly pancreatic polypeptide, insulin, glucagon, and somatostatin. Duodenal endocrine lesions were found in 4 of 5 investigated patients and stained with gastrin and somatostatin antibodies. Distal, mainly subtotal pancreatic resection, was performed in 18 patients, eventually combined with caput tumor enucleation or duodenotomy, while a few patients underwent only tumor enucleation or a Whipple procedure. The long-term outcome of operation was most favorable in patients with hyperinsulinism; only 1 patient had clinical recurrence. Patients with hypergastrinemia experienced only transitory lowering of serum gastrin values after pancreatic surgery and 47% of them had or developed metastases. Such tumor spread was seen in 57% of the patients with non-functioning lesions. Nine patients died from progressive tumor disease during follow-up. Consistent with previous studies, we found that surgery is indicated in MEN-1 patients with hyperinsulinism even if a lesion is not visualized by radiology. In addition, these indications should be extended to also include patients with only biochemical markers of disease, including elevations of gastrin, as these indicate the presence of gross tumors.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Pancreatic tumors in multiple endocrine neoplasia type 1: clinical presentation and surgical treatment. 135 27

The purpose of this study was to examine our experience with the diagnosis, surgical approach, and outcomes of surgery for organic hyperinsulinemia in the era of transhepatic venous sampling. During the period from 1978 to 1991, 50 patients were evaluated and treated for hyperinsulinemia at the University of Michigan Medical Center, all of whom underwent preoperative localization. Forty-one patients (82%) had solitary, benign tumors; four (8%) had either multiple tumors or islet cell dysplasia; and five (10%) had metastatic disease. Forty-seven patients underwent laparotomy, and the source of the hyperinsulinemia was found in all patients. In three patients (6%) preoperative localization did not accurately locate the tumor due to technical difficulties with the completion of the studies. Overall, computed tomography localized nine (26%) of 35 tumors. Angiography accurately localized 18 (44%) of 41 tumors. Transhepatic venous sampling localized 34 (94%) of 36 tumors, and was essential to successful surgical treatment in 15 patients. Compared with angiography, transhepatic venous sampling was a more accurate method of localization and should be performed in all patients in whom computed tomography and visceral angiography do not clearly identify the site of disease. Preoperative localization plays a critical role in the surgical treatment of patients with organic hyperinsulinemia and eliminates the need for blind pancreatic resection.
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PMID:Surgical approach to insulinomas. Assessing the need for preoperative localization. 155 98

The influence of diabetes mellitus on the course of breast cancer was investigated retrospectively in 752 patients. Possible unfavourable prognostic factors like overweight, lipid disorders, age and menopausal status were considered as confounders in a Cochran-Mantel-Haensel analysis. There was no difference in primary tumor status and lymph node involvement between patients with diabetes mellitus and nondiabetic patients. Diabetic patients had more often overweight, lipid disorders and were older than nondiabetic patients. Metastatic disease was highly significant correlated with primary tumor status (p less than 10(-6)) lymph node involvement (p less than 10(-10)) and diabetes mellitus (p less than 10(-5)). Overweight, lipid disorders, age and menopausal status were not correlated with metastatic disease. A possible explanation of the correlation between diabetes mellitus and metastatic disease could be hyperinsulinism in type IIB diabetes. A type IIB diabetes in most of the patients included in this study is very plausible because of the correlation between overweight, lipid disorders, old age and diabetes mellitus. This type of diabetes is characterised by a relative resistence to insulin in the target tissues and a prolonged and exceeding insulin secretion. Experimental data demonstrate that insulin stimulates the growth of breast cancer cell in vivo and in vitro.
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PMID:[Diabetes mellitus and breast cancer. A retrospective follow-up study]. 218 18

Unlike its lethal exocrine counterpart, islet cell carcinoma of the pancreas is an indolent neuroendocrine neoplasm. The majority of these tumors are hormonally active. When functioning, a number of clinical syndromes (for example, hyperinsulinism, Zollinger-Ellison and Cushing's syndromes) may be evident. Fifty-eight patients surgically treated between 1965 and 1984 were retrospectively analyzed. The purpose of this study was to determine the distribution of functioning versus nonfunctioning tumors and the response to type of therapy. Mean postoperative follow-up was 7.4 years. Survival and prognostic indices were calculated by the Kaplan-Meier method and compared with log-rank tests. Of the group, 54% had functioning and 46% nonfunctioning tumors. Gastrinomas were the most common functioning tumors encountered (19%). Of interest was the finding that nonfunctioning tumors increased steadily during the last 15 years of the study (25% to 65%). Curative resections were performed in 15 (26%) and noncurative procedures in 43 patients (74%), with an overall operative mortality rate of 3%. Symptomatic improvement was achieved in 90% (curative) and 51% (noncurative). Survival at 3 years was 87% and 66% for the curative and noncurative groups, respectively (p less than 0.1), with an overall 5-year survival of 42%. The absence of hepatic metastases was a major predictor of survival at 3 years (82% vs 56%, p less than 0.05). Survival was statistically better at 3 years in those patients with gastrinomas compared with patients with nonfunctioning tumors (91% vs 58%, p less than 0.05). Although surgical cure is rare, significant long-term palliation may be achieved in a large percentage of patients with an aggressive surgical approach, occasional total gastrectomy, combination chemotherapy, H2 blockade, when indicated, and, most recently, with the new long-acting analogue of somatostatin.
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PMID:Islet cell carcinomas of the pancreas: a twenty-year experience. 290 80

A 57-year-old man first noted tender subcutaneous nodules on his extremities and abdomen. Six months later symptoms of insulin hypersecretion became evident and hyperinsulinemia was documented. An exploratory laparotomy revealed an islet cell tumor measuring 12 cm in diameter extending anteriorly from head of the pancreas with metastases in the liver. Evidence of parathyroid and pituitary hormone hypersecretion suggests that the insulinoma may be part of a multiple endocrine neoplasia syndrome. Histological examination of the subcutaneous nodules revealed the characteristic histology of nodular fat necrosis. This is the first report of the association of an insulin-secreting islet cell carcinoma with subcutaneous nodular fat necrosis.
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PMID:Association of insulinoma with subcutaneous nodular fat necrosis. 616 10

Twenty-one patients with hyperinsulinism were studied to localize the source of the insulin production. Nineteen patients had pancreatic tumors and two patients studied after pancreatectomy had hepatic metastases. Of the 19 pancreatic tumors, 16 were identified by angiography. CT found six tumors in 14 patients studied while sonography correctly localized only three of 12 tumors. Neither CT nor sonography was positive in those patients with a negative arteriogram. Noninvasive imaging methods can localize large tumors and may be useful for detecting hepatic metastases. Angiography, however, remains the most accurate method for detecting smaller lesions.
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PMID:Localizing insulinomas with combined radiographic methods. 625 78

We report herein a rare case of malignant insulinoma which recurred as multiple liver metastasis 8 years after the initial resection. The patient was a 51-year-old Japanese man who originally presented in 1985 at the age of 43 years suffering from general malaise and syncope. The initial surgery in 1985 involved complete enucleation of a 15 x 13 mm insulinoma located in the uncus of the pancreas. Histopathologically, the tumor was diagnosed as a benign adenoma (insulinoma) which was immunohistochemically stained with only the anti-insulin monoclonal antibody. Macroscopically, there were no signs of either invasion or metastasis. During the subsequent 7 years, he did not show any symptoms or significant abnormality in laboratory data. However, in 1993, the patient again experienced syncope with hypoglycemia and hyperinsulinemia. Ultrasonography revealed multiple echogenic lesions in the liver and a second laparotomy confirmed multiple hepatic metastases from insulinoma, the histopathological findings of which were similar to those of the primary tumor from 8 years before. The patient is currently being treated with streptozotocin and 5-fluorouracil via a catheter in the hepatic artery.
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PMID:Malignant insulinoma causing liver metastasis 8 years after the initial surgery: report of a case. 754 77

Insulinoma in patients with multiple endocrine neoplasia (MEN) is a rare condition that because of its usual multicentricity presents difficulties not encountered in sporadic patients. In contrast to gastrinoma, which is the most common pancreatic neoplasm associated with MEN I, malignancy and duodenal tumors are much less common for patients with insulinomas, and excellent palliative medication is not available. Accordingly, there is a much greater reliance on surgical therapy for this group of patients. Between 1970 and 1991 a total of 19 patients had surgical treatment of MEN I-related insulinoma. Each patient had hyperinsulinemic hypoglycemia. One patient, with extensive metastases, had unresectable disease. Of the remaining 18, there were 16 (89%) multiple pancreatic tumors. Tumors were located in the neck, body, or tail in 17 cases, 10 of whom also had tumors in the head. Pancreatic resections performed were 1 total, 12 subtotal (7 also had enucleation of tumors from the pancreatic head), and 5 limited distal resections and/or enucleation (conservative resection). There was no operative mortality. One patient developed pancreatitis, fistula, and diabetes following subtotal resection and enucleation. Postoperative cure was achieved in 17 of 18 cases. Recurrent disease occurred in 2 of 5 conservative resections compared to 0 of 12 subtotal resections, with median follow-up times of 10.4 and 10.3 years, respectively. During the follow-up period, four patients died, possibly all due to MEN I-related conditions. Hyperinsulinism in MEN I is associated with the occurrence of multiple, usually benign, pancreatic islet cell tumors, and surgery is an effective treatment modality.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Surgical management of insulinoma associated with multiple endocrine neoplasia type I. 772 33


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