MECT1

Pathology

 MAML2-MECT1 gene fusion by t(11;19)(q21;p13) in

• mucoepidermoid carcinoma
• benign Warthin tumor
• cutaneous clear cell hidradenoma (15729701)

The MECT1/MAML2 translocation is identified in a large proportion of mucoepidermoid carcinomas (MEC) of the salivary gland and is an emerging favorable prognosticator.

MECT1/MAML2 translocation is highly specific for MEC and imparts a better prognosis. However, it is frequent even within high-grade MEC and can be seen in lethal cases suggesting that translocation status should not supersede conventional parameters. (20588178)

There are 2 distinct subgroups within high-grade MEC, and the translocation negative tumors may actually be more appropriately categorized as another tumor type (such as adenosquamous carcinoma). (20588178)

References

 A Reappraisal of the MECT1/MAML2 Translocation in Salivary Mucoepidermoid Carcinomas. Seethala RR, Dacic S, Cieply K, Kelly LM, Nikiforova MN. Am J Surg Pathol. 2010 Jun 28. PMID: 20588178

 Tirado Y, Williams MD, Hanna EY, Kaye FJ, Batsakis JG, El-Naggar AK. CRTC1/MAML2 fusion transcript in high grade mucoepidermoid carcinomas of salivary and thyroid glands and Warthin’s tumors: implications for histogenesis and biologic behavior. Genes Chromosomes Cancer. 2007 Jul;46(7):708-15. PMID: 17437281

 Winnes M, Molne L, Suurkula M, Andren Y, Persson F, Enlund F, Stenman G. Frequent fusion of the CRTC1 and MAML2 genes in clear cell variants of cutaneous hidradenomas. Genes Chromosomes Cancer. 2007 Jun;46(6):559-63. PMID: 17334997

 Tonon, G.; Modi, S.; Wu, L.; Kubo, A.; Coxon, A. B.; Komiya, T.; O’Neil, K.; Stover, K.; El-Naggar, A.; Griffin, J. D.; Kirsch, I. R.; Kaye, F. J. :
t(11;19)(q21;p13) translocation in mucoepidermoid carcinoma creates a novel fusion product that disrupts a Notch signaling pathway. Nature Genet. 33: 208-213, 2003. PubMed ID : 12539049

 Behboudi A, Enlund F, Winnes M, Andren Y, Nordkvist A, Leivo I, Flaberg E, Szekely L, Makitie A, Grenman R, Mark J, Stenman G. Molecular classification of mucoepidermoid carcinomas-prognostic significance of the MECT1-MAML2 fusion oncogene. Genes Chromosomes Cancer. 2006 May;45(5):470-81. PMID: 16444749