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)

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

DNA nuclear content and PCNA index (proportion of PCNA reactive cells) have been studied by flow cytometry in eight pancreatic lesions producing hyperinsulinemic hypoglycemia to assess DNA ploidy and tumoral growth fraction. Adult nesidioblastosis had a diploid DNA index, two adenomas were near diploid, and four of the adenomas and the carcinoma were aneuploid. The median value of PCNA index (6.056% +/- 6.76) was significantly correlated (P < 0.05) with the mean tumor diameter (2.43 cm +/- 1.96). Tumors with a PCNA index < 6.056% showed a diameter less than the mean tumoral diameter and a benign clinical course. Tumors with PCNA index > 6.056% generally displayed a diameter bigger than the mean tumoral diameter, being associated with lymph node metastases in one case. The authors conclude that nuclear DNA and PCNA index cytometric studies are useful parameters to assess the biological behavior of pancreatic lesions producing hyperinsulinemic hypoglycemia.
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PMID:DNA ploidy and PCNA index in pancreatic lesions producing hyperinsulinemic hypoglycemia. 774 70

Primary meningeal hemangiopericytomas (formerly referred to as angioblastic meningiomas) are by most authors no longer considered to be actual meningiomas but rather thought to be intracranial hemangiopericytomas. Their biological behavior is usually malignant, with recurrences and metastases, often at intervals of years. Both intracranial and extracranial hemangiopericytomas may, however rarely, be accompanied by paraneoplastic hypoglycemias. Our own characteristic observations are based on a female patient aged 67 at the time of her death, in whom a meningeal tumor was resected first at the age of 41. Later recurrencies were removed at the age of 52 and 58 respectively. Three years prior to her death liver metastases had developed followed by increasingly frequent attacks of early morning hypoglycemia with blood sugar levels ranging between 1.4-2.3 mmol/l. Specific examinations revealed low endocrine production of insulin and a distinctly decreased insulin-like growth factor (IGF) I of 25 ng/mb (normal 120-130) and a normal value of total IGF II of 724 ng/ml (normal 400-900), though with a big macromolecular share. The observed paraneoplastic hypoglycemia is probably brought about by coincidence of blocked hepatic glucose production, suppressed lipolysis and increased peripheral glucose uptake. Autopsy revealed a third intracranial recurrence of meningeal hemangiopericytoma and a large metastatic liver. No other sites of metastases were found. Histologic, immunohistologic and electron microscopic findings showed the characteristics features of a hemangiopericytoma. Light microscopic pictures of the primary tumor, recurrences and metastases were identical. Additional autopsy findings were a papillary carcinoma of the right kidney, an angiomyolipoma of the left kidney and a thecoma of the left ovary.
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PMID:[Recurrent and metastasizing hemangiopericytoma of the meninges with paraneoplastic hypoglycemia]. 812 96

The authors report their experience with octreotide in 20 patients (median age 57 years, 10 M, 10 F) from 1984 to 1991; 16 had metastatic APUDoma: 1 PPoma with VIPoma, 1 glucagonoma, 5 gastrinoma including 1 associated to PP-oma, 9 mid-gut carcinoid; 3 patients had multiple-endocrine neoplasia type I (MEN-I) with Zollinger-Ellison syndrome (ZES) and 1 patient a non-metastatic VIPoma. Octreotide (200-750 micrograms/day) was administered bid or tid with regular laboratory controls and morphological assessment. There was a striking improvement of symptoms, particularly in the carcinoid group (reduction of flushing in all patients and of diarrhoea in 3/5), in the patient with gastrinoma + acromegaly (regression of congestive heart failure) and in the patient with non-metastatic VIPoma. The hormonal markers were markedly reduced, particularly gastrin, PP (except in the patient with PPoma + VIPoma), VIP, GH and Somatomedin-C and urinary 5HIAA in 4/9 patients with carcinoid. There was only one partial regression of metastases (gastrinoma) and 4 apparent stabilizations of tumour growth, in the 16 metastatic cases. Among them, 4 patients died: 1 glucagonoma, 1 PPoma + VIPoma, 2 mid-gut carcinoids after a treatment of 5, 16, 30, 36 months, respectively. The patient with acromegaly + ZES died after 6 years of treatment at age 81. A patient with prolactinoma, resected insulinoma, hyperparathyroidism and ZES was not improved by a short course of octreotide (hypoglycemia); he died later of recurrent insulinoma. In conclusion, octreotide is a useful drug to control most of the symptoms related to gut endocrine tumours; it may inhibit tumour growth.
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PMID:Use of octreotide in the treatment of digestive neuroendocrine tumours. Seven year experience in 20 cases including 9 cases of metastatic midgut carcinoid and 5 cases of metastatic gastrinoma. 826 71

A 54-year-old man presented with a poorly differentiated adenocarcinoma of the rectum with multiple metastases to the liver. During hospitalization the patient developed periods of hypoglycaemia due to production of "big" IGF-II by the tumour. Possible pathophysiological mechanisms of non-islet-cell tumour-induced hypoglycaemia are discussed.
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PMID:Non-islet-cell tumour induced hypoglycaemia: a case report and review of literature. 871 34

Hypoglycemia associated with a large, gastric leiomyosarcoma was diagnosed in an 11-year-old, yellow Labrador retriever. The dog had persistent hypoglycemia and low serum insulin levels. Although radiological and ultrasonographical evaluations suggested an abdominal mass involving the liver, a large, nonresectable, gastric mass was found during exploratory laparotomy. Metastasis was not identified on gross inspection of the liver or abdomen, and no metastatic lesions were seen on thoracic radiographs. The dog was euthanized. Histopathological examination of the mass revealed a poorly differentiated, gastric leiomyosarcoma that extended into the mesentery without regional lymph-node involvement.
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PMID:Gastric leiomyosarcoma associated with hypoglycemia in a dog. 878 17

A 52-year-old-woman with non-insulin-dependent diabetes mellitus developed carcinoma of the pancreas and had a Whipple's resection performed. She required pancreatic exocrine supplements and insulin post-operatively. Five years later metastatic disease became apparent, and was accompanied by episodic spontaneous hypoglycaemia necessitating the cessation of insulin therapy. Hormonal analysis was performed, off insulin, at a time of hypoglycaemia (glucose 0.9 mmol l-1) and showed negligible insulin concentrations (< 2 mU l-1) but raised IGF-II together with low IGF-I concentrations (1.85 and 0.1 U ml-1, respectively). The association between diabetes and pancreatic carcinoma, and the pathogenesis of non-islet cell tumour induced hypoglycaemia (NICTH) are discussed.
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PMID:Spontaneous hypoglycaemia in a noninsulin-dependent diabetes mellitus patient with disseminated pancreatic carcinoma. 911 88

A survey of UK patients receiving the drug diazoxide, revealed 40 patients with insulinoma on this treatment. Mean age (+/- SD) was 67 +/- 18 years, and 74% were female. Duration of treatment was 7 +/- 6 years (range 1-22). Most (55%) patients were treated with diazoxide because of tumour non-localisation (including failed previous surgery). Metastatic disease (20%) and poor surgical risk (10%) were other indications. Side-effects (notably fluid retention and hirsutism) were common (47%) but not troublesome. Treatment was highly effective--59% were symptom free and 38% had only occasional symptoms. Only one patient had frequent hypoglycaemia despite treatment. We conclude that diazoxide is effective in the management of insulinoma. Side-effects are common but not problematic. Treatment should be considered for all patients not cured by surgery, or unsuitable for surgical treatment.
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PMID:Diazoxide treatment for insulinoma: a national UK survey. 949 74

We report a 22-yr-old male patient with chronic hepatitis B and a large, well differentiated hepatoma who developed episodes of symptomatic fasting hypoglycemia, which were caused by paraneoplastic secretion of unprocessed "big" insulin-like growth factor-II. Initially, the patient presented with normal liver function, which deteriorated during the clinical course. Therapeutic attempts to reduce tumor mass failed and the patient subsequently died because of metastases of the hepatoma. The pathophysiology of non-islet cell tumor hypoglycemia, differential diagnosis, and therapeutic options are discussed.
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PMID:Hepatoma with severe non-islet cell tumor hypoglycemia. 964 37

We report a case of successful liver transplantation in a young man with metastatic hemangiopericytoma confined to the liver. He had a primary retroperitoneal hemangiopericytoma removed in 1982 and was diagnosed as having liver metastases 8 years later. Subsequently, he developed hypoglycemia, which did not respond to chemotherapy. Hepatectomy and transplantation resolved his hypoglycemic attacks. He remained well and symptom free for 4 years. Liver transplantation can provide effective palliation and should be considered in carefully selected patients with localized metastatic cancer.
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PMID:Liver transplantation for metastatic hemangiopericytoma associated with hypoglycemia. 1003 Mar 2


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