Glucagonoma

A glucagonoma a pancreatic ?-cell tumor, the glucagon is secreted, thereby causing hyperglycemia and a characteristic rash. The diagnosis is confirmed by elevated glucagon levels and imaging tests. The tumor is localized by means of CT and endoscopic ultrasonography. Treatment consists of resection.

The glucagonoma is an endocrine pancreatic tumor (Endocrine pancreatic tumors), which develops from the alpha cells of the pancreas. Glucagonomas are very rare, but similar to other islet cell tumors is that the primary tumor and the metastases grow slowly, a survival of 15 years is common. 80% of the glucagonomas are malignant. The average age at the first symptoms is 50 years, 80% of patients are female. A few patients have multiple endocrine neoplasia type I (multiple endocrine neoplasia, type I (MEN)).

A glucagonoma a pancreatic ?-cell tumor, the glucagon is secreted, thereby causing hyperglycemia and a characteristic rash. The diagnosis is confirmed by elevated glucagon levels and imaging tests. The tumor is localized by means of CT and endoscopic ultrasonography. Treatment consists of resection. The glucagonoma is an endocrine pancreatic tumor (Endocrine pancreatic tumors), which develops from the alpha cells of the pancreas. Glucagonomas are very rare, but similar to other islet cell tumors is that the primary tumor and the metastases grow slowly, a survival of 15 years is common. 80% of the glucagonomas are malignant. The average age at the first symptoms is 50 years, 80% of patients are female. A few patients have multiple endocrine neoplasia type I (multiple endocrine neoplasia, type I (MEN)). Because symptoms and complaints Glucagonomas produce glucagon, the symptoms are the same as for diabetes mellitus. Frequently there are weight loss, anemia normochromic, Hypoaminoazidämie and hypolipidemic; the most prominent clinical sign is a chronic, the extremities involvierende dermatitis, often accompanied by a smooth shiny bright red tongue and cheilitis. The exfoliative, red-brown, erythematous lesions with superficial necrolysis are called nekrolytisches migratory erythema. Diagnosis Serumglucagonspiegel CT and endoscopic ultrasonography for localization Most patients with a glucagonoma have glucagon levels> 1000 pg / ml (normal <200). However, moderate elevations in renal failure, acute pancreatitis, severe stress and fasting occur. A correlation with the symptoms is required. Patients should receive an abdominal CT followed by endoscopic ultrasonography; MRI or PET are used when the CT diagnosis does not continue. Therapy Surgical resection for localized disease chemotherapy for metastatic disease octreotide to suppress glucagon The resection of the tumor relieves all the symptoms. Nichtresektable, metastatic or recurrent tumors are treated with a combination of streptozotocin and doxorubicin, this may reduce the levels of circulating immunoreactive glucagon, reduce symptoms and increase the response rates (50%), but an improvement in overall survival is unlikely. Newer chemotherapies that are studied in glucagonoma include temozolomide-based regime, everolimus or sunitinib. Octreotidinjektionen suppress some of the Glucagonproduktion and relieve the erythema, but also glucose tolerance may decrease because octreotide also reduces insulin secretion. Octreotide may alleviate anorexia and caused by the catabolic effect of excess glucagon weight loss quickly. Responsively patients can be converted to a long-acting Octreotidpräparat (20-30 mg i.m. once a month). Patients often have to take additional Octreotidmedikation pancreatic enzymes because octreotide inhibits the secretion of pancreatic enzymes. Locally-applied, oral or parenteral zinc can cause erythema disappear, but it can also by simple hydrogenation or i.v. Administration of amino acids or fatty acids decrease, so that the erythema is not caused solely by a zinc deficiency.

Health Life Media Team

Leave a Reply