Multiple Endocrine Neoplasia Type 2


MEN1 (Wermer Syndrome) is an autosomal dominant cancer syndrome associated with Pituitary, Parathyroid, and Pancreatic neoplasms (3Ps). Most commonly, patients have parathyroid adenoma, pituitary microadenoma, and pancreatic or gastro-duodenal NETs (neuroendocrine tumors). Download the handout:
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Multiple Endocrine Neoplasia is a rare disease in which more than one endocrine gland gets affected, and they start forming tumors. There are Multiple Endocrine Neoplasia, that type 1 and type 2. In this video, learn about the causes, symptoms, and treatment options for Multiple Endocrine Neoplasia.
By Dr. Ramya C Valiveru
Consultant Breast Oncologist & Endocrine Surgeon
KIMS Hospitals, Gachibowli.
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The term multiple endocrine neoplasia encompasses several distinct syndromes featuring tumors of endocrine glands, each with its own characteristic pattern. In some cases, the tumors are malignant, in others, benign. Benign or malignant tumors of nonendocrine tissues occur as components of some of these tumor syndromes.
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MEN syndromes are inherited as autosomal dominant disorders.
Multiple Endocrine Neoplasia type 1 (MEN1) is a rare hereditary endocrine cancer syndrome characterized primarily by tumors of the parathyroid glands (95% of cases), endocrine gastroenteropancreatic (GEP) tract (30-80% of cases), and anterior pituitary (15-90% of cases). Other endocrine and non-endocrine neoplasms including adrenocortical and thyroid tumors, visceral and cutaneous lipomas, meningiomas, facial angiofibromas and collagenomas, and thymic, gastric, and bronchial carcinoids also occur. The phenotype of MEN1 is broad, and over 20 different combinations of endocrine and non-endocrine manifestations have been described. MEN1 should be suspected in patients with an endocrinopathy of two of the three characteristic affected organs, or with an endocrinopathy of one of these organs plus a first-degree relative affected by MEN1 syndrome.
MEN1 patients usually have a family history of MEN1. Inheritance is autosomal dominant; any affected parent has a 50% chance to transmit the disease to his or her progeny. MEN1 gene mutations can be identified in 70-95% of MEN1 patients.
Many endocrine tumors in MEN1 are benign and cause symptoms by overproduction of hormones or local mass effects, while other MEN1 tumors are associated with an elevated risk for malignancy. About one third of patients affected with MEN1 will die early from an MEN1-related cancer or associated malignancy. Entero-pancreatic gastrinomas and thymic and bronchial carcinoids are the leading cause of morbidity and mortality. Consequently, the average age of death in untreated individuals with MEN1 is significantly lower (55.4 years for men and 46.8 years for women) than that of the general population.


MRCP_Part_1_MEN_(_Multiple_Endocrine_Neoplasia_)
MEN (Multiple Endocrine Neoplasia)
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Genetic Counselor reviews the main tumors associated with Multiple Endocrine Neoplasia Type 1 and explains the genetics of this rare syndrome.
Check out a great resource for people with MEN1 or other types of Multiple Endocrine Neoplasia at www.AMENSupport.org (American Multiple Endocrine Neoplasia Support). They also have a Facebook support page.
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