PMID- 18328987 OWN - NLM STAT- MEDLINE DCOM- 20080807 LR - 20211203 IS - 1521-6942 (Print) IS - 1521-6942 (Linking) VI - 22 IP - 1 DP - 2008 Mar TI - Multiple endocrine neoplasms. PG - 149-63 LID - 10.1016/j.berh.2007.11.010 [doi] AB - Multiple endocrine neoplasia type 1 (MEN1) and type 2 (MEN2) are rare autosomal-dominant disorders characterized by primary tumours in at least two different endocrine tissues. Both syndromes present as sporadic (a single case with two of the characteristic endocrine tumours) or familial form (an MEN case plus at least one first-degree relative showing one of the characteristic endocrine tumours). MEN1 is characterized by the occurrence of parathyroid, gastro-entero-pancreatic and anterior pituitary tumours, but it can include various combinations of more than 20 endocrine and non-endocrine tumours. Generally, tumours in MEN1 are benign, although gastrinomas and foregut carcinoids may exhibit a malignant course. MEN2 is characterized by medullary thyroid carcinoma (MTC), uni- or bi-lateral pheochromocytoma, and other tumours of different endocrine tissues. If not diagnosed precociously, MTC can be fatal. MEN1 develops after tissue inactivation of both MEN1 gene copies. Activating mutations of c-RET proto-oncogene causes MEN2. FAU - Falchetti, Alberto AU - Falchetti A AD - Surgery Unit, Department of Clinical Physiopathology, University of Florence, 6-50139 Florence, Italy. FAU - Marini, Francesca AU - Marini F FAU - Luzi, Ettore AU - Luzi E FAU - Tonelli, Francesco AU - Tonelli F FAU - Brandi, Maria Luisa AU - Brandi ML LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review PL - Netherlands TA - Best Pract Res Clin Rheumatol JT - Best practice & research. Clinical rheumatology JID - 101121149 RN - 0 (MAS1 protein, human) RN - 0 (MEN1 protein, human) RN - 0 (Proto-Oncogene Mas) RN - 0 (Proto-Oncogene Proteins) RN - EC 2.7.10.1 (Proto-Oncogene Proteins c-ret) SB - IM EIN - Best Pract Res Clin Rheumatol. 2008 Dec;22(6):III. Brandt, Maria Luisa [corrected to Brandi, Maria Luisa] MH - Adenoma/genetics MH - Adrenocortical Carcinoma/genetics MH - Gastrointestinal Neoplasms/genetics MH - Humans MH - Multiple Endocrine Neoplasia/*genetics MH - Pancreatic Neoplasms/genetics MH - Pheochromocytoma/genetics MH - Pituitary Neoplasms/genetics MH - Proto-Oncogene Mas MH - Proto-Oncogene Proteins/genetics MH - Proto-Oncogene Proteins c-ret/genetics RF - 68 EDAT- 2008/03/11 09:00 MHDA- 2008/08/08 09:00 CRDT- 2008/03/11 09:00 PHST- 2008/03/11 09:00 [pubmed] PHST- 2008/08/08 09:00 [medline] PHST- 2008/03/11 09:00 [entrez] AID - S1521-6942(07)00129-5 [pii] AID - 10.1016/j.berh.2007.11.010 [doi] PST - ppublish SO - Best Pract Res Clin Rheumatol. 2008 Mar;22(1):149-63. doi: 10.1016/j.berh.2007.11.010.