The American Association of Endocrine Surgeons, Patient Education Site

Background: Neuroendocrine tumor/carcinoid tumor features

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Can these tumors be inherited?

Specific genetic disorders and chromosomal abnormalities are associated with a predisposition for developing neuroendocrine/carcinoid tumors. While the majority of neuroendocrine/carcinoid tumors are not associated with a genetic syndrome (sporadic), about 10% are associated with one of the following inherited diseases.

Multiple endocrine neoplasia. The most common inherited genetic syndrome associated with neuroendocrine/carcinoid tumors is multiple endocrine neoplasia type 1 (MEN 1). MEN 1 is a syndrome characterized by pancreatic, parathyroid, and pituitary endocrine neoplasms. Less frequently, patients with the MEN 1 syndrome develop carcinoid tumors, adrenal tumors, and tumors of the ovaries and thyroid. Approximately 50% of patients from MEN 1 families will develop the syndrome and the distribution between genders is equal. This pattern of inheritance is consistent with an autosomal dominant trait. The MEN 1 syndrome has been associated with mutations in the menin gene, on chromosome 11q13. Menin, a presumed tumor suppressor, is a nuclear protein interacting with Jun D, an AP1 transcription factor. There have been over 1,300 different mutations of Menin described to date. About 30-60% of patients with MEN1 will develop a neuroendocrine tumor.

Von Hippel-Lindau disease. Less common than the MEN 1 syndrome is von Hippel-Lindau disease (vHL). This is a syndrome of cerebelloretinal hemangioblastomatosis. Tumors of the pancreas, kidney, epididymis, and cysts or angiomas of the kidneys or liver are also associated with this syndrome. The pattern of inheritance appears to be autosomal dominant. About 11-17% of patients with VHL will develop a neuroendocrine/carcinoid tumor. All of these tumors were non-functional, and more than half of the patients had multiple tumors.

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