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 is a rare pancreatic neuroendocrine tumor that is usually described as benign, sporadic, and very small (<2 cm). However, there have been rare case reports of insulinoma presenting as a giant tumor. We describe 3 cases of giant insulinomas, all of which developed liver metastases. The patients were aged 38, 63, and 67 years. Clinically, all patients presented with Whipple's triad associated with a large mass located in the pancreatic tail. The tumors ranged in size from 10 to 15 cm. On microscopic examination, the tumors were well differentiated with amyloid deposition ranging between 20% and 30%. Immunohistochemically, all 3 tumors showed strong diffuse expression of chromogranin and synaptophysin, whereas they were only focally positive for insulin. One patient developed liver recurrence 3 years after resection of the primary tumor yet remained asymptomatic without treatment. Another patient with liver recurrence underwent right hepatectomy and has been free of disease for 2 years. The third patient died of metastatic disease 13 years after initial surgery. Giant insulinomas are characterized by focal expression of insulin and high rates of liver metastases. Long-term follow-up is mandatory in these patients, as recurrence is expected after primary surgery.
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PMID:Giant insulinoma: a report of 3 cases and review of the literature. 2415 58

Insulinoma is a rare pancreatic neuroendocrine tumor. Overproduction of insulin and associated hypoglycemia are hallmark features of this disease. Diagnosis can be made through demonstration of hypoglycemia and elevated plasma levels of insulin or C-Peptide. Metastatic disease can be detected through computerized tomography (CT) scans, magnetic resonance imaging (MRI), and positron emission tomography (PET)/CT. Somatostatin receptor scintigraphy can be used not only to document metastatic disease but also as a predictive marker of the benefit from therapy with radiolabeled somatostatin analog. Unresectable metastatic insulinomas may present as a major therapeutic challenge for the treating physician. When feasible, resection is the mainstay of treatment. Prevention of hypoglycemia is a crucial goal of therapy for unresectable/metastatic tumors. Diazoxide, hydrochlorothiazide, glucagon, and intravenous glucose infusions have been used for glycemic control yielding temporary and inconsistent results. Sandostatin and its long-acting depot forms have occasionally been used in the treatment of Octreoscan-positive insulinomas. Herein, we report a case of metastatic insulinoma with very difficult glycemic control successfully treated with the radiolabeled somatostatin analog lutetium ((177)LU).
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PMID:Metastatic insulinoma managed with radiolabeled somatostatin analog. 2445 30

Insulinoma is the most common hormonally active neuroendocrine tumor of the pancreas. Low blood sugar, caused by excessive secretion of insulin from the tumor cells, leads to a variety of symptoms (e.g. signs of neuroglycopaenia), that may be mistaken for diseases of the central nervous system, psychiatric disturbances or cardiovascular conditions. That is why a lot of attention should be brought to the diagnostic tests towards insulinoma. The development of imaging techniques in the past years has lead to the possibility of locating an insulinoma of a few milimetres in size, which is helpful to avoid the need of a blind resection and preserves the pancreatic parenchyma. 90% of insulinomas are benign and surgery is the treatment of choice. However, as in any neuroendocrine tumor, a malignant form may occur--resection of the metastases is the only curative method, but radiofrequency ablation, selective internal radiation therapy, hepatic artery embolization or chemoembolization, can also have positive therapeutic applications. Systemic therapies regarding malignant insulinoma are: chemotherapy, somatostatin analogs, radiolabelled somatostatin analogs and the newly developed biological targeted therapies such as everolimus--oral inhibitor of the mammalian target of rapamycin, and sunitinib--the tyrosine kinase inhibitor.
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PMID:[The newest perspectives on diagnostic methods and treatment of insulinoma]. 2576 82

Insulinoma is an insulin-producing pancreatic neuroendocrine tumor that can be malignant in about 10% of cases. Locoregional invasion, lymph node metastases, or remote metastases are the main criteria of malignant insulinoma. Its incidence in patients with pre-existing diabetes mellitus (DM) is exceptionally rare. In this report, we describe a 66-year-old man with long-standing type 2 DM who presented with recurrent episodes of diaphoresis due to severe hypoglycemia despite the withdrawal of insulin therapy, hypercalcitoninemia, and biochemical and radiological findings suggestive of metastatic malignant insulinoma. Unfortunately, after few days of diazoxide treatment, edema, hypotension, oliguria, and water retention were observed, patient's clinical status deteriorated rapidly, and he died in our department from acute renal failure.
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PMID:Malignant Insulinoma with Multiple Liver Metastases and Hypercalcitoninemia in a Patient with Type 2 Diabetes Mellitus Presenting as Recurrent Episodes of Diaphoresis due to Severe Hypoglycemia. 3208 48


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