Pituitary tumors in patients with MEN1 syndrome

• Published in: Clinics (São Paulo, Brazil) Vol. 67; no. Suppl 1; pp. 43 - 48
• Main Authors: Syro, Luis V., Scheithauer, Bernd W., Kovacs, Kalman, Toledo, Rodrigo A., Londoño, Francisco J., Ortiz, Leon D., Rotondo, Fabio, Horvath, Eva, Uribe, Humberto
• Format: Journal Article
• Language: English; Portuguese
• Published: Brazil Elsevier España, S.L.U 01.01.2012; Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo; Faculdade de Medicina / USP; Elsevier España
• Subjects: Adenoma - genetics; Adenoma - pathology; Genes, Neoplasm - genetics; Genetics; Germ-Line Mutation - genetics; Humans; MEDICINE, GENERAL & INTERNAL; Multiple Endocrine Neoplasia Type 1; Multiple Endocrine Neoplasia Type 1 - genetics; Multiple Endocrine Neoplasia Type 1 - pathology; Mutation; Pituitary Neoplasms; Pituitary Neoplasms - genetics; Pituitary Neoplasms - pathology; Review; Syndrome; Pituitary Neoplasms; Genetics; Review; Multiple Endocrine Neoplasia Type 1
• ISSN: 1807-5932; 1980-5322; 1980-5322
• DOI: 10.6061/clinics/2012(Sup01)09

We briefly review the characteristics of pituitary tumors associated with multiple endocrine neoplasia type 1. Multiple endocrine neoplasia type 1 is an autosomal-dominant disorder most commonly characterized by tumors of the pituitary, parathyroid, endocrine-gastrointestinal tract, and pancreas. A MEDLINE search for all available publications regarding multiple endocrine neoplasia type 1 and pituitary adenomas was undertaken. The prevalence of pituitary tumors in multiple endocrine neoplasia type 1 may vary from 10% to 60% depending on the studied series, and such tumors may occur as the first clinical manifestation of multiple endocrine neoplasia type 1 in 25% of sporadic and 10% of familial cases. Patients were younger and the time between initial and subsequent multiple endocrine neoplasia type 1 endocrine lesions was significantly longer when pituitary disease was the initial manifestation of multiple endocrine neoplasia type 1. Tumors were larger and more invasive and clinical manifestations related to the size of the pituitary adenoma were significantly more frequent in patients with multiple endocrine neoplasia type 1 than in subjects with non-multiple endocrine neoplasia type 1. Normalization of pituitary hypersecretion was much less frequent in patients with multiple endocrine neoplasia type 1 than in subjects with non-multiple endocrine neoplasia type 1. Pituitary tumors in patients with multiple endocrine neoplasia type 1 syndrome tend to be larger, invasive and more symptomatic, and they tend to occur in younger patients when they are the initial presentation of multiple endocrine neoplasia type 1.