2017
DOI: 10.1007/s00428-017-2104-4
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Clinicopathologic and genetic features of primary bronchopulmonary mucoepidermoid carcinoma: the MD Anderson Cancer Center experience and comprehensive review of the literature

Abstract: Primary bronchopulmonary mucoepidermoid carcinoma (BPMEC) is a rare tumor. The fusion protein MECT1-MAML2 has been implicated as a causative genetic event in salivary and BPMECs. Several studies have shown the impact of MECT1-MAML2 on the diagnosis and prognosis of salivary gland mucoepidermoid carcinoma; however, few studies have been published regarding MECT1-MAML2 in the context of primary BPMEC. We describe the clinicopathologic, genetic, and outcome data of 16 patients with BPMEC. Clinicopathologic featur… Show more

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Cited by 34 publications
(26 citation statements)
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“…The diagnosis of mucoepidermoid carcinoma can be supported by consistent labeling for p40/p63 in intermediate and squamous cells, positivity for intracytoplasmic mucin with mucin stains, and the demonstration of mastermind like transcriptional coactivator 2 gene (MAML2) rearrangements detected in tumors by FISH at a rate of 77% to 100%. [101][102][103] The result of staining for SRY-box 10 (SOX10) is usually negative in mucoepidermoid carcinoma but can be positive in a subset. 104 The main differential diagnosis in the lung is with adenosquamous carcinoma; expression of TTF1/napsin A in the glandular component would support the former, whereas MAML2 rearrangement would support the latter.…”
Section: Salivary Gland-type Tumorsmentioning
confidence: 99%
“…The diagnosis of mucoepidermoid carcinoma can be supported by consistent labeling for p40/p63 in intermediate and squamous cells, positivity for intracytoplasmic mucin with mucin stains, and the demonstration of mastermind like transcriptional coactivator 2 gene (MAML2) rearrangements detected in tumors by FISH at a rate of 77% to 100%. [101][102][103] The result of staining for SRY-box 10 (SOX10) is usually negative in mucoepidermoid carcinoma but can be positive in a subset. 104 The main differential diagnosis in the lung is with adenosquamous carcinoma; expression of TTF1/napsin A in the glandular component would support the former, whereas MAML2 rearrangement would support the latter.…”
Section: Salivary Gland-type Tumorsmentioning
confidence: 99%
“…However, in contrast to adenosquamous carcinoma, these tumors occur at a relatively younger age, with a median of about 40 years, with about equal sex ratio, and are generally central large airway tumors ( Figure 5, A) rather than peripheral nodules. 70,71 In this regard, in many cases the location of tumor and the epidemiology is sufficiently distinct to favor mucoepidermoid carcinoma over adenosquamous carcinoma.…”
Section: Immunotherapymentioning
confidence: 99%
“…However, carcinoma in situ and overtly keratinizing areas are not seen. 71 Low-grade tumors are associated with improved outcome relative to high-grade tumors. 73 In low-grade tumors, neither the gland-forming nor the solid/squamous component is difficult to identify.…”
Section: Immunotherapymentioning
confidence: 99%
“…The loss of fragile histidine triad protein (FHIT) is strongly related to pancreatic ductal adenocarcinomas [ 33 ]. MAML2-MECT1 fusion is a driver in salivary gland and bronchial gland mucoepidermoid carcinoma [ 34 , 35 ]. MYBL1 is a driver of adenoid cystic carcinoma when related to MYB [ 36 , 37 ].…”
Section: Resultsmentioning
confidence: 99%