Management of endocrine disease: a clinical update on tumor-induced hypoglycemia.
Tumor-induced hypoglycemia (TIH) is a rare clinical entity that may occur in patients with diverse kinds of tumor lineages and that may be caused by different mechanisms. These pathogenic mechanisms include the eutopic insulin secretion by a pancreatic islet β-cell tumor, and also the ectopic tumor insulin secretion by non-islet-cell tumor, such as bronchial carcinoids and gastrointestinal stromal tumors. Insulinoma is, by far, the most common tumor associated with clinical and biochemical hypoglycemia. Insulinomas are usually single, small, sporadic, and intrapancreatic benign tumors. Only 5-10% of insulinomas are malignant. Insulinoma may be associated with the multiple endocrine neoplasia type 1 in 4-6% of patients. Medical therapy with diazoxide or somatostatin analogs has been used to control hypoglycemic symptoms in patients with insulinoma, but only surgical excision by enucleation or partial pancreatectomy is curative. Other mechanisms that may, more uncommonly, account for tumor-associated hypoglycemia without excess insulin secretion are the tumor secretion of peptides capable of causing glucose consumption by different mechanisms. These are the cases of tumors producing IGF2 precursors, IGF1, somatostatin, and glucagon-like peptide 1. Tumor autoimmune hypoglycemia occurs due to the production of insulin by tumor cells or insulin receptor autoantibodies. Lastly, massive tumor burden with glucose consumption, massive tumor liver infiltration, and pituitary or adrenal glands destruction by tumor are other mechanisms for TIH in cases of large and aggressive neoplasias.
Department of Endocrinology, Hospital Ramón y Cajal, Ctra. de Colmenar, Km 9.100, 28034 Madrid, Spain.
Pedro
P
Iglesias
Juan J
JJ
Díez
D001323:Autoantibodies / Q000276:immunology; D001984:Bronchial Neoplasms / Q000150:complications / Q000378:metabolism; D002276:Carcinoid Tumor / Q000150:complications / Q000378:metabolism; D005770:Gastrointestinal Neoplasms / Q000150:complications / Q000378:metabolism; D046152:Gastrointestinal Stromal Tumors / Q000150:complications / Q000378:metabolism; D052216:Glucagon-Like Peptide 1 / Q000378:metabolism; D006801:Humans; D007003:Hypoglycemia / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D007334:Insulin-Like Growth Factor I / Q000378:metabolism; D007335:Insulin-Like Growth Factor II / Q000378:metabolism; D007340:Insulinoma / Q000150:complications / Q000378:metabolism; D009369:Neoplasms / Q000150:complications* / Q000276:immunology / Q000378:metabolism; D010190:Pancreatic Neoplasms / Q000150:complications / Q000378:metabolism; D010257:Paraneoplastic Syndromes / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D011972:Receptor, Insulin / Q000276:immunology; D013004:Somatostatin / Q000378:metabolism
D001323:Autoantibodies; D013004:Somatostatin; D007334:Insulin-Like Growth Factor I; D007335:Insulin-Like Growth Factor II; D052216:Glucagon-Like Peptide 1; D011972:Receptor, Insulin