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Multiple endocrine neoplasia type 4 (MEN4) is a very rare form of MEN (see this term), an inherited cancer syndrome, characterized by parathyroid and anterior pituitary tumors, possibly associated with adrenal, renal, and reproductive organ tumors. The prevalence of multiple endocrine neoplasia type 4 is unknown, but the syndrome is very rare.
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Multiple endocrine neoplasia (MEN) constitutes a group of autosomal dominant disorders characterized by a broad spectrum of endocrine and nonendocrine diseases. Depending on the clinical presentations and genetic mutation, MEN is divided into different types.
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MEN type 4 (MEN4) is the most recently identified type of MEN. Although it shares a similar phenotype spectrum to MEN1, MEN4 is rare. The difference between MEN1 and MEN4 lies in the germline gene mutation: in MEN1, there is a mutation of the MEN1 gene; in MEN4, there is a mutation in the cyclin-dependent kinase inhibitor 1B gene ( CDKN1B ).
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Four major forms of MEN are recognized and referred to as types 1-4 (MEN1-MEN4), and each form is characterized by the development of tumors within specific endocrine glands ( Table 1) ( Thakker, 1998 ).
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Multiple endocrine neoplasia (MEN) is characterized by the occurrence of tumors involving two or more endocrine glands within a single patient. Four major forms of MEN, which are autosomal dominant disorders, are recognized and referred to as: MEN type 1 (MEN1), due to menin mutations; MEN2 (previou.
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Multiple endocrine neoplasia type IV (MEN4), previously known as MENX, is a rare type of MEN with clinical overlap with MEN1 but is due to a CKDN1B mutation. It is characterized by 1,2: parathyroid adenoma primary hyperparathyroidism in 80% anterior pituitary adenoma neuroendocrine tumors (pulmonary, gastrointestinal, pancreatic)
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Three types of multiple endocrine neoplasia (MEN) syndromes are most common: MEN type 1, MEN type 2A and MEN type 2B. MEN syndromes are caused by genetic changes; therefore, they tend to run in the families. Symptoms of MEN are different based on the type of the disease and the glands involved. Genetic testing is recommended if you have family.