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Multiple endocrine neoplasms.
Best Pract Res Clin Rheumatol. 2008 Mar; 22(1):149-63.BP

Abstract

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.

Authors+Show Affiliations

Surgery Unit, Department of Clinical Physiopathology, University of Florence, 6-50139 Florence, Italy.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Review

Language

eng

PubMed ID

18328987

Citation

Falchetti, Alberto, et al. "Multiple Endocrine Neoplasms." Best Practice & Research. Clinical Rheumatology, vol. 22, no. 1, 2008, pp. 149-63.
Falchetti A, Marini F, Luzi E, et al. Multiple endocrine neoplasms. Best Pract Res Clin Rheumatol. 2008;22(1):149-63.
Falchetti, A., Marini, F., Luzi, E., Tonelli, F., Brandi, M. L., & Brandt, M. L. (2008). Multiple endocrine neoplasms. Best Practice & Research. Clinical Rheumatology, 22(1), 149-63. https://doi.org/10.1016/j.berh.2007.11.010
Falchetti A, et al. Multiple Endocrine Neoplasms. Best Pract Res Clin Rheumatol. 2008;22(1):149-63. PubMed PMID: 18328987.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Multiple endocrine neoplasms. AU - Falchetti,Alberto, AU - Marini,Francesca, AU - Luzi,Ettore, AU - Tonelli,Francesco, AU - Brandi,Maria Luisa, AU - Brandt,Maria Luisa, PY - 2008/3/11/pubmed PY - 2008/8/8/medline PY - 2008/3/11/entrez SP - 149 EP - 63 JF - Best practice & research. Clinical rheumatology JO - Best Pract Res Clin Rheumatol VL - 22 IS - 1 N2 - 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. SN - 1521-6942 UR - https://www.unboundmedicine.com/medline/citation/18328987/Multiple_endocrine_neoplasms_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1521-6942(07)00129-5 DB - PRIME DP - Unbound Medicine ER -