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Journal of Stomatology, Oral and... Dec 2020Mucoepidermoid Carcinoma (MEC) is the most common malignant salivary gland neoplasm, representing 10 to 15% of all salivary neoplasms. A review of the literature was... (Review)
Review
OBJECTIVE
Mucoepidermoid Carcinoma (MEC) is the most common malignant salivary gland neoplasm, representing 10 to 15% of all salivary neoplasms. A review of the literature was conducted in order to determine trends in presentation, diagnostic features, treatment, and outcomes.
METHODS
A PubMed, Embase, and Scopus search was carried out. The search process was performed by 2 independent reviewing authors and inclusion criteria included systematic reviews, meta-analyses, case-controls studies, cohort studies, comparative studies, clinical trials, cross-sectional studies, descriptive studies, experimental studies, case reports, case series studies, and human studies evaluating MEC.
RESULTS
Females were more affected (54.5%) and the average age was 48.8 years. The most common location of MEC was the parotid glands (56.8%) followed by hard palate (18%). The most frequent clinical presentation was mass (65.2%) followed by ulcer (29.4%) with pressure as their main symptom (64.4%). The most frequent histologic presentation was Low grade (46.7%) followed by Intermediate grade (27.3%) and the most used treatment was surgery (76.2%). The average follow up was 138.5months, and recurrence was reported at 8.5%.
CONCLUSION
MEC showed a strong predilection for the parotid glands is frequently painful, most frequently presents as a mass and most commonly has a low-grade histologic presentation.
Topics: Carcinoma, Mucoepidermoid; Cross-Sectional Studies; Female; Humans; Middle Aged; Neoplasm Recurrence, Local; Parotid Gland; Salivary Gland Neoplasms
PubMed: 32565266
DOI: 10.1016/j.jormas.2020.06.003 -
Molecular Pathology of Salivary Gland Neoplasms: Diagnostic, Prognostic, and Predictive Perspective.Advances in Anatomic Pathology Mar 2021Salivary gland neoplasms are an uncommon and widely heterogeneous group of tumors. In recent years, there has been considerable progress in efforts to reveal the... (Review)
Review
Salivary gland neoplasms are an uncommon and widely heterogeneous group of tumors. In recent years, there has been considerable progress in efforts to reveal the molecular landscape of these tumors, although it is still limited and appears to be only the tip of the iceberg. Genomic aberrations, especially specific chromosomal rearrangements including CRTC1-MAML2 and CRTC3-MAML2 in mucoepidermoid carcinoma, MYB-NFIB and MYBL1-NFIB fusions in adenoid cystic carcinoma, PLAG1 and HMGA2 alterations in pleomorphic adenoma and carcinoma ex pleomorphic adenoma, ETV6-NTRK3 and ETV6-RET in secretory carcinoma, EWSR1-ATF1 and EWSR1-CREM in clear cell carcinoma, provide new insights into the molecular pathogenesis of various salivary gland neoplasms and help to better classify them. These genetic aberrations primarily serve as diagnostic tools in salivary gland tumor diagnosis; however, some also have promise as prognostic or predictive biomarkers. This review summarizes the latest developments in molecular pathology of salivary gland tumors with a focus on distinctive molecular characteristics.
Topics: Adenoma, Pleomorphic; Biomarkers, Tumor; Carcinoma, Adenoid Cystic; Carcinoma, Mucoepidermoid; Gene Expression Regulation, Neoplastic; Humans; Pathology, Molecular; Prognosis; Salivary Gland Neoplasms
PubMed: 33405400
DOI: 10.1097/PAP.0000000000000291 -
Mayo Clinic Proceedings Sep 2023
Topics: Humans; Carcinoma, Mucoepidermoid
PubMed: 37661153
DOI: 10.1016/j.mayocp.2023.07.018 -
Turk Gogus Kalp Damar Cerrahisi Dergisi Jan 2024Pulmonary tumors in childhood are rare, but the majority are malignant. The histopathologic spectrum is quite diverse, including inflammatory myofibroblastic tumor,... (Review)
Review
Pulmonary tumors in childhood are rare, but the majority are malignant. The histopathologic spectrum is quite diverse, including inflammatory myofibroblastic tumor, hamartoma, primary pulmonary paraganglioma, carcinoid tumor, mucoepidermoid carcinoma, pleuropulmonary blastoma, adenocarcinoma, squamous cell carcinoma, and sarcomas. Nonspecific clinical and radiological findings result in late and incorrect diagnoses. Although surgical resection is the initial and proper treatment method, additional adjuvant therapy is dependent on both tumor stage and histopathologic type.
PubMed: 38584790
DOI: 10.5606/tgkdc.dergisi.2024.25863 -
Mucoepidermoid carcinoma of the head and neck: CRTC1/3 MAML 2 translocation and its prognosticators.European Archives of... May 2022Mucoepidermoid carcinoma (MEC) of the head and neck is a prevalent malignant salivary gland tumour with a reported good outcome. The aim of this study was to report the...
PURPOSE
Mucoepidermoid carcinoma (MEC) of the head and neck is a prevalent malignant salivary gland tumour with a reported good outcome. The aim of this study was to report the outcome in our centre.
METHODS
A retrospective chart analysis with survival analyses was performed combined with fluorescence in situ hybridization (FISH) analysis to assess CRTC1/3 MAML 2 fusion gene presence.
RESULTS
Sixty-four cases of MEC were identified. Median age at presentation was 51.4 years with a predominance for parotid gland involvement. Five, 10- and 20- year disease-free survival was 98%, 90% and 68%, respectively. Overall survival was 94%, 90% and 64%, respectively. Local recurrence was seen up to 14 years after primary diagnosis; distant metastases were diagnosed up to 17 years later. The overall recurrence rate was less than 20 per cent. CRTC1/3 MAML 2 fusion gene presence showed no survival benefit.
CONCLUSION
MEC of the head and neck has a favorable outcome with the exception of high-grade MEC. PNI and nodal involvement are not rare. CRTC1/3 MAML 2 fusion gene presence showed no survival benefit. The tendency for late onset of loco-regional and distant recurrence should not be underestimated.
Topics: Carcinoma, Mucoepidermoid; DNA-Binding Proteins; Humans; In Situ Hybridization, Fluorescence; Nuclear Proteins; Retrospective Studies; Salivary Gland Neoplasms; Trans-Activators; Transcription Factors; Translocation, Genetic
PubMed: 34405264
DOI: 10.1007/s00405-021-07039-2 -
Virchows Archiv : An International... Jan 2021Thymic carcinoma encompasses a diverse group of rare tumors that occur almost exclusively in the prevascular (anterior) mediastinum. Thymic carcinomas have a worse... (Review)
Review
Thymic carcinoma encompasses a diverse group of rare tumors that occur almost exclusively in the prevascular (anterior) mediastinum. Thymic carcinomas have a worse outcome than thymomas with a median time to death of under 3 years. These tumors lack the typical lobulation of thymomas, exhibit commonly more cytologic atypia, are associated with a desmoplastic stromal reaction, and lack thymocytes, features that distinguish them from thymomas. The most common thymic carcinoma is squamous cell carcinoma; other subtypes include mucoepidermoid carcinoma, NUT carcinoma, and adenocarcinoma, among others. Largely due to multi-institutional and global efforts and meta-analysis of case reports and series, some of the thymic carcinoma subtypes have been studied in more detail and molecular studies have also been performed. Morphology and immunophenotype for the vast majority of thymic carcinoma subtypes are similar to their counterparts in other organs. Therefore, the distinction between thymic carcinoma and metastatic disease, which is relatively common in the prevascular mediastinum, can be challenging and in general requires clinical and radiologic correlation. Although surgical resection is the treatment of choice, only 46 to 68% of patients with thymic carcinoma can undergo resection as many other tumors present at high stage with infiltration into vital neighboring organs. These patients are usually treated with chemotherapy and/or radiation. The search for better biomarkers for prognosis and treatment of thymic carcinomas is important for improved management of these patients and possible targeted therapy.
Topics: Adenocarcinoma; Carcinoma, Squamous Cell; Humans; Prognosis; Thymoma; Thymus Neoplasms
PubMed: 33389148
DOI: 10.1007/s00428-020-03000-6 -
World Journal of Oncology Feb 2022Mucoepidermoid carcinoma (MEC) represents 10-15% of salivary neoplasms. Due to their low incidence, it is challenging to conduct clinical trials and develop treatment... (Review)
Review
Mucoepidermoid carcinoma (MEC) represents 10-15% of salivary neoplasms. Due to their low incidence, it is challenging to conduct clinical trials and develop treatment guidelines. Although surgery is the most common approach for a resectable tumor, various treatment options such as chemotherapy, radiotherapy, and immunotherapy have been investigated. There is a need to implement a standardized treatment protocol to effectively manage MEC as it is a common histological subtype. Furthermore, it has become essential to assess chromosomal and genetic abnormalities recently identified with MEC, including alterations of , , , , etc. These mutations are involved in the transformation of low-grade tumors to high-grade tumors, presenting a vital tool for evaluating the aggressive behavior of this carcinoma. Detailed immunohistochemical and translocation studies can help develop targeted therapies and monitor treatment response. Therefore, biomarker-driven research will immensely improve the outcome, especially in advanced cases. Based on thorough histology and chromosomal translocations, a more personalized treatment plan can improve the overall disease outcome. The purpose of this article is to elaborate on the current treatment advancements, particularly chemotherapy and targeted therapy, as an effective treatment modality for the management of MEC and highlight the comparison with traditional treatment approaches.
PubMed: 35317327
DOI: 10.14740/wjon1412 -
Cancer Medicine May 2023Mucoepidermoid carcinoma (MEC) of the breast is an extremely rare salivary gland-type tumor characterized by epidermoid, basaloid, intermediate, and/or mucinous cells...
Mucoepidermoid carcinoma (MEC) of the breast is an extremely rare salivary gland-type tumor characterized by epidermoid, basaloid, intermediate, and/or mucinous cells arranged in solid and cystic patterns. Despite their triple-negative phenotype, breast MECs are generally considered low-risk malignancies but their biology is largely unexplored; therefore, guidelines for clinical management are lacking. Here, we sought to characterize the molecular landscape of breast MECs. Thirteen cases were histologically reviewed, characterized for tumor-infiltrating lymphocytes (TILs), and were subjected to immunohistochemistry for programmed death-ligand 1 (PD-L1, clone 22C3), EGFR, and amphiregulin (AREG). Rearrangements in MAML2 and EWSR1 were investigated by fluorescent in situ hybridization. Targeted next-generation sequencing of 161 genes was performed on eight cases. Most MECs had low histological grade (n = 10, 77%), with the presence of TILs (n = 9/12; 75%) and PD-L1 combined positive score ranging from 10 to 20 (n = 4/6; 67%). All cases showed EGFR and AREG overexpression and were fusion negative. Enrichment of genetic alterations was observed in PI3K/AKT/mTOR and cell cycle regulation pathways, while only one case harbored TP53 mutations. This is the first study providing extensive molecular data on breast MECs and the largest collection of cases available to date in the literature. Breast MECs lack TP53 mutations found in high-grade forms of triple-negative breast cancers and MAML2 or EWSR1 rearrangements pathognomonic of salivary MECs. Triple-negativity and PD-L1 positivity suggest a window of opportunity for immunotherapy in these patients. The EGFR/AREG axis activation, coupled with the mutational patterns in PI3K/AKT/mTOR and cell cycle pathways warrants caution in considering MECs as low-risk neoplasms.
Topics: Humans; DNA-Binding Proteins; Trans-Activators; B7-H1 Antigen; In Situ Hybridization, Fluorescence; Carcinoma, Mucoepidermoid; Phosphatidylinositol 3-Kinases; Proto-Oncogene Proteins c-akt; Nuclear Proteins; Transcription Factors; Salivary Gland Neoplasms; ErbB Receptors; TOR Serine-Threonine Kinases; Biomarkers, Tumor
PubMed: 36916425
DOI: 10.1002/cam4.5754 -
Modern Pathology : An Official Journal... Oct 2023Mucoepidermoid carcinoma (MEC) is exceedingly rare in the breast, with <45 cases reported in the literature. Although estrogen receptor/progesterone receptor/human...
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. Rare cases of HA have also been reported in the breast, but these are relatively uncharacterized. In this study, we examined the clinicopathologic, immunohistochemical (IHC), and genetic features of 8 breast HAs, in comparison to 3 mammary MECs. All cases were positive for MAML2 break-apart fluorescence in situ hybridization. Eight cases demonstrated a CRTC1::MAML2 fusion, and one MEC harbored a CRTC3::MAML2 fusion; the latter is a novel finding in the breast. Mutational burden was very low, with only one HA exhibiting a MAP3K1 pathogenic alteration. By IHC, both MEC and HA demonstrated cell type-dependent expression of high- and low-molecular-weight keratins and p63, as well as negative to low-positive estrogen receptor and androgen receptor. Smooth muscle myosin and calponin highlighted an in situ component in the 3 cases of MEC; expression of these myoepithelial markers was negative in HAs. Additional distinguishing characteristics included the growth pattern and tumor architecture, the presence of glandular/luminal cells in HA, and overall higher IHC expression of SOX10, S100 protein, MUC4, and mammaglobin in MEC. Morphologic findings were also compared to a series of 27 cutaneous nonmammary HAs. Mucinous and glandular/luminal cells were identified in significantly more mammary HAs than nonmammary lesions. The findings provide insight into the pathogenesis of MAML2-rearranged neoplasms of the breast, underscore the overlapping genetic features of MEC and HA, and highlight similarities to their extramammary counterparts.
PubMed: 37422157
DOI: 10.1016/j.modpat.2023.100270 -
Modern Pathology : An Official Journal... Oct 2022Mucoepidermoid carcinoma (MEC) and adenosquamous carcinoma (ASC) have overlapping histopathological appearances and sites of occurrence, which may cause diagnostic...
Mucoepidermoid carcinoma (MEC) and adenosquamous carcinoma (ASC) have overlapping histopathological appearances and sites of occurrence, which may cause diagnostic difficulty impacting subsequent treatment. We conducted a systematic review of the scientific literature to determine whether molecular alterations were sufficiently different in MEC and ASC to aid in classifying the two entities. We searched Medline, Embase and Web of Science for studies reporting molecular determinations of ASC and/or MEC and screened retrieved records for eligibility. Two independent researchers reviewed included studies, assessed methodological quality and extracted data. Of 8623 identified records, 128 articles were included for analysis: 5 which compared the two tumors in the same investigation using the same methods and 123 which examined the tumors separately. All articles, except one were case series of moderate to poor methodological quality. The 5 publications examining both tumors showed that 52/88 (59%) MEC and 0% of 110 ASC had rearrangement of the MAML2 gene as detected by FISH and/or RT-PCR, but did not investigate other genes. In the entire series MEC had MAML2 gene rearrangement in 1337/2009 (66.6%) of tumors studied. The articles examining tumors separately found that MEC had mutations in EGFR (11/329 cases, 3.3%), KRAS (11/266, 4.1%) and ERBB2 (9/126, 7.1%) compared with ASC that had mutations in EGFR (660/1705, 38.7%), KRAS (143/625, 22.9%) and ERBB2 (6/196, 3.1%). The highest level of recurrent mutations was in pancreatic ASC where (108/126, 85.7%) reported mutations in KRAS. The EGFR mutations in ASC were similar in number and kind to those in lung adenocarcinoma. By standards of systematic review methodology and despite the large number of retrieved studies, we did not find adequate evidence for a distinctive molecular profile of either MEC or ASC that could definitively aid in its classification, especially in histologically difficult cases that are negative for MAML2 rearrangement. The case series included in this review indicate the relevance of MAML2 rearrangement to support the diagnosis of MEC, findings that should be confirmed by additional research with adequate study design.
Topics: Carcinoma, Adenosquamous; Carcinoma, Mucoepidermoid; DNA-Binding Proteins; ErbB Receptors; Humans; In Situ Hybridization, Fluorescence; Nuclear Proteins; Proto-Oncogene Proteins p21(ras); Salivary Gland Neoplasms; Trans-Activators; Transcription Factors
PubMed: 35871081
DOI: 10.1038/s41379-022-01100-z