Insulinoma
Insulinoma: Introduction Print Section Listen Insulinomas are such rare tumors that even most specialists treat only a handful of these patients over their entire careers. Moreover, even most major referral…
Multiple Endocrine Neoplasia Type 1
Definition Print Section Listen Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant genetic predisposition to develop multiglandular parathyroid disease, benign and malignant neuroendocrine tumors (NET of the pancreas…
Carcinoid Tumors
Carcinoid Tumors: Introduction Print Section Listen Carcinoid tumors were first described in a 1907 paper titled “Karzinoide Tumoren des Dünndarms,” or “Cancer-like Tumors of the Gut,” by the German pathologist…
von Hippel-Lindau Disease
Definition Print Section Listen von Hippel-Lindau (VHL) disease is an autosomal dominant syndrome that predisposes individuals to a variety of tumors. VHL is associated with tumors in a variety of…
Multiple Endocrine Neoplasia Type 2
Epidemiology and Risk Factors Print Section Listen Sipple described pheochromocytoma associated with carcinoma of the thyroid gland in 1961.1 Seven years later, multiple endocrine neoplasia type 2 (MEN2) was recognized…
Other Functional and Nonfunctional Neuroendocrine Tumors
Epidemiology Print Section Listen The pancreas is a retroperitoneal organ that is composed of multiple lineages of neuroendocrine cells interspersed within the larger structure of its exocrine ductal system. The…
Gastrinoma
Epidemiology Print Section Listen In 1955, Zollinger and Ellison1 described two patients with jejunal ulceration, gastric acid hypersecretion, and a non-β pancreatic islet cell tumor. The diagnostic triad of gastric…
Hypercortisolism
Definition Print Section Listen Hypercortisolism is a disorder characterized by excessive circulating cortisol from either endogenous or exogenous sources, which can cause detrimental changes to nearly every organ system. Collectively,…
Adrenocortical Carcinoma
Definition and Epidemiology Print Section Listen Adrenocortical carcinoma (ACC) is a rare malignant endocrine neoplasm with an estimated incidence of 0.5 to 2 cases per 1 million people annually in…
Pheochromocytoma and Paraganglioma
Definition Print Section Listen Pheochromocytomas and paragangliomas, otherwise referred to as extra-adrenal pheochromocytomas, are rare neuroendocrine tumors that originate from neural crest cells of the autonomic nervous system.1 They secrete…
