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Aims: The aim of study was to evaluate the impact of CRTC1-MAML2 and CRTC3-MAML2 fusions on the histological classification of mucoepidermoid carcinoma (MEC) of the salivary glands and on the prognosis of patients.
Methods And Results: MEC cases (n = 111) were screened for CRTC1-MAML2 and CRTC3-MAML2 fusions by reverse transcription polymerase chain reaction. We developed a system of 'molecular Armed Forces Institute of Pathology (AFIP) classification' that combined the AFIP histological classification proposed by Goode et al. and the presence of CRTC1-MAML2 or CRTC3-MAML2 fusions. MEC cases positive for CRTC1-MAML2 or CRTC3-MAML2 fusion formed a favourable tumour subset that was distinct from fusion-negative cases. When positive for the fusions, 'high-risk' patients, including those with a higher histological grade or an advanced clinical stage, showed an excellent prognosis. For overall survival, 'molecular AFIP classification' was selected as a powerful independent prognostic factor (P=0.0038), as was the clinical stage (P =0.0032). For disease-free survival, 'molecular AFIP classification' was also selected as an independent prognostic factor (P = 0.0006).
Conclusions: Molecular AFIP classification may be useful in predicting the prognosis of patients with MEC.
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http://dx.doi.org/10.1111/j.1365-2559.2011.03890.x | DOI Listing |
Mod Pathol
July 2025
Department of Pathology, Stanford University School of Medicine, Stanford, California. Electronic address:
Genomic rearrangements involving MAML2 have been reported in mucoepidermoid carcinoma (MEC) arising in various anatomical sites, as well as the benign counterpart of hidradenoma (HA). Depending on the location, MAML2-rearranged neoplasms may share morphologic overlap with additional diagnostic entities, including other salivary gland malignancies and cutaneous mimics. In some cases, detection of a CRTC1::MAML2 or less common CRTC3::MAML2 rearrangement by fluorescence in situ hybridization (ISH) or next-generation sequencing may be necessary to help confirm the diagnosis.
View Article and Find Full Text PDFMod Pathol
October 2023
Department of Pathology, Stanford University School of Medicine, Stanford, California. Electronic address:
Mucoepidermoid carcinoma (MEC) is exceedingly rare in the breast, with <45 cases reported in the literature. Although estrogen receptor/progesterone receptor/human epidermal growth factor 2 triple-negative, MEC is characterized as a special subtype of breast carcinoma with significantly better prognosis than conventional basal-type tumors. Cutaneous hidradenoma (HA) is considered a benign adnexal neoplasm showing histomorphologic overlap with MEC.
View Article and Find Full Text PDFHistopathology
January 2023
Department of Pathology, The Johns Hopkins Hospital, Baltimore, MD, USA.
Mucoepidermoid carcinoma (MEC) is historically defined by a mix of squamoid, intermediate, and mucous cells, but we have recently encountered several cases lacking immunoreactivity for squamous markers p40, p63, and CK5/6 despite MAML2 fusions. This study will characterise these unique tumours. Ten MEC were collected arising from the parotid gland (n = 4), submandibular gland (n = 2), nasopharynx (n = 1), base of tongue (n = 1), bronchus (n = 1), and trachea (n = 1).
View Article and Find Full Text PDFFront Oncol
March 2022
Department of Respiratory and Critical Care Medicine, The Second Affiliated Hospital of Chongqing Medical University, Chongqing, China.
Zhonghua Bing Li Xue Za Zhi
August 2021
Department of Pathology, Fudan University Shanghai Cancer Center, Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200032, China.
To investigate MAML2 gene rearrangement, gene fusion patterns, and the clinicopathological characteristics of primary pulmonary mucoepidermoid carcinoma (PMEC). Forty-six cases of primary PMEC from Fudan University Zhongshan Hospital and Fudan University Shanghai Cancer Center between 2017 and 2020 were collected. MAML2 gene rearrangement in all cases was detected by fluorescence in situ hybridization (FISH).
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