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mucoepidermoid carcinoma

Wednesday 14 June 2006

pulmonary mucoepidermoid carcinoma; mucoepidermoid carcinoma of the bronchi;

Digital cases

HPC:95: pulmonary mucoepidermoid carcinoma.
JRC:6447 : pulmonary mucoepidermoid carcinoma.
JRC:19401 : pulmonary mucoepidermoid carcinoma.

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Mucoepidermoid carcinoma

Definition: Primary pulmonary mucoepidermoid carcinomas (MECs) are the third most frequent pulmonary malignant neoplasm in children.

Localization

salivary mucoepidermoid carcinoma
sinusal mucoepidermoid carcinoma
bronchial mucoepidermoid carcinoma (pulmonary mucoepidermoid carcinoma )
thymic mucoepidermoid carcinoma

Clinical synopsis

atelectasis
recurrent respiratory infections (15750450)

Microscopcial synopsis

possible prominent tumor-associated lymphoid proliferation (15725811)

Variants

oncocytic mucoepidermoid carcinoma
sclerosing mucoepidermoid carcinoma
acinar mucoepidermoid carcinoma

Cytogenetics

t(11;19)(q21;p12 p13.11)

creating MECT1-MAML2 fusion gene
MECT1-MAML2 fusion transcript expression is a prognostic marker in infantile lung mucoepidermoid carcinoma. (17618868)
t(11;19) appears to be the primary chromosomal aberration for pulmonary mucoepidermoid carcinomas in children. The MECT1-MAML2 fusion transcript is associated with a better prognosis in MEC tumors. (17618868)

Grade

Mucoepidermoid carcinoma (MEC) shows differences in biological behaviour depending mainly on its histological grade. High-grade tumours usually have an aggressive biological course and they require additional oncological treatment after surgery.

Cytogenetics

t(11;19)(q21;p13) translocation (8019965)

Molecular biology

MECT1/MAML2 fusion gene by t(11;19)(q21;p13) translocation (55%) (16444749)

Mucoepidermoid carcinomas (MECs) of the salivary and bronchial glands are characterized by a recurrent t(11;19)(q21;p13) translocation.
It results in a MECT1-MAML2 fusion in which the CREB-binding domain of the CREB coactivator MECT1 (also known as CRTC1, TORC1 or WAMTP1) is fused to the transactivation domain of the Notch coactivator MAML2.

Activation of the EGFR/ERK pathway in high-grade mucoepidermoid carcinomas of the salivary glands. (20664595)

The EGFR/ERK pathway is activated in high-grade MECs with aggressive behaviour.
Patients with these tumours who require oncological treatment in addition to surgery could benefit from EGFR and mitogen-activated protein kinase pathway inhibitors.

See also

Tumors

Carcinomas

References

Localization

Mucoepidermoid carcinoma of the breast. Di Tommaso L, Foschini MP, Ragazzini T, Magrini E, Fornelli A, Ellis IO, Eusebi V. Virchows Arch. 2004 Jan;444(1):13-9. PMID: 14634807

Molecular biology

Activation of the EGFR/ERK pathway in high-grade mucoepidermoid carcinomas of the salivary glands. Lujan B, Hakim S, Moyano S, Nadal A, Caballero M, Diaz A, Valera A, Carrera M, Cardesa A, Alos L. Br J Cancer. 2010 Aug 10;103(4):510-6. PMID: 20664595

CRTC1 rearrangements in the absence of t(11;19) in primary cutaneous mucoepidermoid carcinoma. Lennerz JK, Perry A, Dehner LP, Pfeifer JD, Lind AC. Br J Dermatol. 2009 Oct;161(4):925-9. PMID: 19438452

Chromosomal imbalances, 11q21 rearrangement and MECT1-MAML2 fusion transcript in mucoepidermoid carcinomas of the salivary gland. Verdorfer I, Fehr A, Bullerdiek J, Scholz N, Brunner A, Krugmann J, Hager M, Haufe H, Mikuz G, Scholtz A. Oncol Rep. 2009 Aug;22(2):305-11. PMID: 19578770

Mucoepidermoid carcinoma of the cervix: another tumor with the t(11;19)-associated CRTC1-MAML2 gene fusion. Lennerz JK, Perry A, Mills JC, Huettner PC, Pfeifer JD. Am J Surg Pathol. 2009 Jun;33(6):835-43.PMID: 19092631

Salivary gland-type lung carcinomas: an EGFR immunohistochemical, molecular genetic, and mutational analysis study. Macarenco RS, Uphoff TS, Gilmer HF, Jenkins RB, Thibodeau SN, Lewis JE, Molina JR, Yang P, Aubry MC. Mod Pathol. 2008 Sep;21(9):1168-75. PMID: 18587327

t(11;19) translocation and CRTC1-MAML2 fusion oncogene in mucoepidermoid carcinoma. O’Neill ID. Oral Oncol. 2009 Jan;45(1):2-9. PMID: 18486532

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

A study of MECT1-MAML2 in mucoepidermoid carcinoma and Warthin’s tumor of salivary glands. Martins C, Cavaco B, Tonon G, Kaye FJ, Soares J, Fonseca I. J Mol Diagn. 2004 Aug;6(3):205-10.PMID: 15269296 [Free]

Multiple reciprocal translocations in salivary gland mucoepidermoid carcinomas. Tonon G, Gehlhaus KS, Yonescu R, Kaye FJ, Kirsch IR. Cancer Genet Cytogenet. 2004 Jul 1;152(1):15-22. PMID: 15193437

Mucoepidermoid carcinoma of the lung with t(11:19)(q21:p13): a link to new biology. Roberts TG Jr, Barry M, Skarin AT. Clin Adv Hematol Oncol. 2003 Aug;1(8):486-7. PMID: 16258437

t(11;19)(q21;p13) translocation in mucoepidermoid carcinoma creates a novel fusion product that disrupts a Notch signaling pathway. Tonon G, Modi S, Wu L, Kubo A, Coxon AB, Komiya T, O’Neil K, Stover K, El-Naggar A, Griffin JD, Kirsch IR, Kaye FJ. Nat Genet. 2003 Feb;33(2):208-13. PMID: 12539049

Cytogenetics

Mucoepidermoid carcinoma of the bronchus in a 15-year-old girl with complex cytogenetic rearrangement involving 11q and over-expression of cyclin D1. Barrett W, Heaps LS, Diaz S, Sharma P, Arbuckle S, Smith A. Med Pediatr Oncol. 2002 Jul;39(1):49-51. PMID: 12116081

A child with a t(11;19)(q14-21;p12) in a pulmonary mucoepidermoid carcinoma. Stenman G, Petursdottir V, Mellgren G, Mark J. Virchows Arch. 1998 Dec;433(6):579-81. PMID: 9870694

Genotypic characterization of a primary mucoepidermoid carcinoma of the parotid gland by cytogenetic, fluorescence in situ hybridization, and DNA ploidy analysis. el-Naggar AK, Lovell M, Killary AM, Batsakis JG. Cancer Genet Cytogenet. 1996 Jul 1;89(1):38-43. PMID: 8689608

A mucoepidermoid carcinoma of minor salivary gland with t(11;19)(q21;p13.1) as the only karyotypic abnormality. El-Naggar AK, Lovell M, Killary AM, Clayman GL, Batsakis JG. Cancer Genet Cytogenet. 1996 Mar;87(1):29-33. PMID: 8646736

Translocation (11;19)(q21;p13.1) in mucoepidermoid carcinoma of salivary gland. Horsman DE, Berean K, Durham JS. Cancer Genet Cytogenet. 1995 Apr;80(2):165-6. PMID: 7736438

Translocation(11;19)(q14-21;p12) in a parotid mucoepidermoid carcinoma of a child. Dahlenfors R, Wedell B, Rundrantz H, Mark J. Cancer Genet Cytogenet. 1995 Feb;79(2):188. PMID: 7889519

Nordkvist A, Gustafsson H, Juberg-Ode M, Stenman G. Recurrent rearrangements of 11q14-22 in mucoepidermoid carcinoma. Cancer Genet Cytogenet. 1994 Jun;74(2):77-83. PMID: 8019965

References

t(11;19)(q21;p12 p13.11) and MECT1-MAML2 fusion transcript expression as a prognostic marker in infantile lung mucoepidermoid carcinoma. Serra A, Schackert HK, Mohr B, Weise A, Liehr T, Fitze G. J Pediatr Surg. 2007 Jul;42(7):E23-9. PMID: 17618868

Carcinoid and mucoepidermoid bronchial tumours in children. Fauroux B, Aynie V, Larroquet M, Boccon-Gibod L, Ducou le Pointe H, Tamalet A, Clément A. Eur J Pediatr. 2005 Dec;164(12):748-52. PMID: 16133240

Pulmonary mucoepidermoid carcinoma with prominent tumor-associated lymphoid proliferation. Shilo K, Foss RD, Franks TJ, DePeralta-Venturina M, Travis WD. Am J Surg Pathol. 2005 Mar;29(3):407-11. PMID:15725811

A child with a t(11;19)(q14-21;p12) in a pulmonary mucoepidermoid carcinoma. Stenman G, Petursdottir V, Mellgren G, Mark J. Virchows Arch. 1998 Dec;433(6):579-81. PMID: 9870694

Mucoepidermoid carcinoma of the bronchus: a case report and review of the literature. Granata C, Battistini E, Toma P, Balducci T, Mattioli G, Fregonese B, Gambini C, Rossi GA. Pediatr Pulmonol. 1997 Mar;23(3):226-32. PMID: 9094733

Childhood tracheobronchial mucoepidermoid carcinoma: a case report and review of the literature. Torres AM, Ryckman FC. J Pediatr Surg. 1988 Apr;23(4):367-70. PMID: 3290425

Mucoepidermoid tumors of the lung. Yousem SA, Hochholzer L. Cancer. 1987 Sep 15;60(6):1346-52. PMID: 3040215

Mucoepidermoid bronchial adenoma in a 6-year-old girl: a case report and review of the literature. Archer RL, Grogg SE, Sanders SP. J Thorac Cardiovasc Surg. 1987 Sep;94(3):452-4. PMID: 3306166

Childhood bronchial mucoepidermoid tumors: a case report and review of the literature. Mullins JD, Barnes RP. Cancer. 1979 Jul;44(1):315-22.PMID: 455258

Adenoid cystic carcinoma (cylindroma) and mucoepidermoid carcinoma of the bronchus. Factors affecting survival. Conlan AA, Payne WS, Woolner LB, Sanderson DR. J Thorac Cardiovasc Surg. 1978 Sep;76(3):369-77. PMID: 210333