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BMJ Case Reports Aug 2014Salivary gland tumours comprise almost 5% of head and neck malignancies. Minor salivary gland tumours account for 10-15% of all salivary gland neoplasms and are usually... (Review)
Review
Salivary gland tumours comprise almost 5% of head and neck malignancies. Minor salivary gland tumours account for 10-15% of all salivary gland neoplasms and are usually malignant. The second most common minor salivary gland tumour (12-40% globally) is mucoepidermoid carcinoma. Mucoepidermoid carcinoma is more frequent in females, occurs in the fifth decade of life and is usually found in the parotid gland. However, the palate is a frequent site when it occurs in the minor glands. We report a case of a high-grade variant of mucoepidermoid carcinoma in the right retromolar trigone of a 21-year man which was treated with wide excision of the tumour with a 1.5 cm margin. Reconstruction was done with a buccal fat pad posteriorly with a pedicled lateral tongue flap. Temporal stripping and right coronoidectomy was carried out in case of post-surgical wound contraction. The patient is currently under periodic review.
Topics: Biopsy; Carcinoma, Mucoepidermoid; Diagnosis, Differential; Humans; Male; Radiography, Panoramic; Salivary Gland Neoplasms; Salivary Glands, Minor; Tomography, X-Ray Computed; Young Adult
PubMed: 25085946
DOI: 10.1136/bcr-2013-202776 -
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 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 -
Laryngoscope Investigative... Feb 2018To report on the current state of the literature on the genetics of mucoepidermoid and adenoid cystic carcinomas of the salivary glands with a focus on genomic screens... (Review)
Review
OBJECTIVE
To report on the current state of the literature on the genetics of mucoepidermoid and adenoid cystic carcinomas of the salivary glands with a focus on genomic screens and recently discovered genetic translocations.
METHODS
A PubMed based literature review was performed to query for genetics related basic science and preclinical studies about mucoepidermoid and adenoid cystic carcinomas of the salivary glands.
RESULTS AND CONCLUSIONS
Genetic translocations between CRTC1 and MAML2 in mucoepidermoid carcinoma and between MYB and NFIB in adenoid cystic carcinoma have been recently discovered and have therapeutic implications. Key signaling pathways such as the EGFR pathway in mucoepidermoid carcinoma and the Notch pathway, chromatin regulation, and c-kit mediated epithelial-mesenchymal transitions in adenoid cystic carcinoma have recently been elucidated, pointing to possible therapeutic targets in both cancers.
PubMed: 29492469
DOI: 10.1002/lio2.139 -
Cancer Jun 2016Salivary gland cancers are an incredibly heterogeneous group of tumors that include 24 histologically distinct tumor types. The use of new genetic methods has paved the... (Review)
Review
Salivary gland cancers are an incredibly heterogeneous group of tumors that include 24 histologically distinct tumor types. The use of new genetic methods has paved the way for promising advancements in our understanding of the molecular biology underlying each type of tumor. The objective of this review was to highlight common oncogenes, tumor suppressor genes, and cytogenetic and epigenetic changes associated with the most common tumor types: mucoepidermoid carcinoma, adenoid cystic carcinoma, salivary duct carcinoma, mammary analogue secretory carcinoma, hyalinizing clear cell carcinoma, carcinoma ex pleomorphic adenoma, and acinic cell carcinoma. Recent insights into the pathogenesis of each cancer subtype have helped better define and classify these tumors. Further research in salivary gland cancers should focus on determining the key genes involved in the tumorigenesis of each distinct malignancy and identifying individualized chemotherapies directed at these targets. Cancer 2016;122:1822-31. © 2016 American Cancer Society.
Topics: Animals; Humans; Salivary Gland Neoplasms
PubMed: 26928905
DOI: 10.1002/cncr.29890 -
Frontiers in Oncology 2022Esophageal mucoepidermoid carcinoma (EMEC) is a rare disease. The biological behavior and treatment of this malignancy are not well established. (Review)
Review
BACKGROUND
Esophageal mucoepidermoid carcinoma (EMEC) is a rare disease. The biological behavior and treatment of this malignancy are not well established.
METHODS
Data from 58 patients with EMEC who underwent esophagectomy were retrospectively analyzed and compared with 5028 patients with esophageal squamous cell carcinoma (ESCC). Kaplan-Meier and multivariate Cox regression analyses were conducted to investigate the association between clinicopathological factors and survival.
RESULTS
The study cohort included 36 males and 22 females with a median age of 59 years (range, 40-78 years). Of the 47 patients who underwent preoperative esophagoscopic biopsy, only 1 patient was diagnosed with EMEC. EMEC was more often found in female patients (39.7% versus 25.8%, P=0.036) and patients with EMEC had a significantly lower rate of lymph node metastasis (25.0% versus 49.4%, P<0.001) than patients with ESCC. After 1:1 propensity score matching, the 5-year overall survival rate of 55.2% for patients with EMEC was similar to that of 61.9% for patients with ESCC (P=0.399).
CONCLUSIONS
EMEC is a rare disease that more often affects females and these patients has less lymph node metastasis than patients with ESCC. Preoperative esophagoscopic biopsy has difficulty obtaining an accurate pathological diagnosis for EMEC patients. The prognosis for EMEC is similar to that for ESCC.
PubMed: 35494060
DOI: 10.3389/fonc.2022.836352 -
Vaccines Sep 2022Salivary gland neoplasms are a heterogeneous neoplasm group, including mucoepidermoid carcinoma (MECa), adenoid cystic carcinoma (AdCC), and many others.
UNLABELLED
Salivary gland neoplasms are a heterogeneous neoplasm group, including mucoepidermoid carcinoma (MECa), adenoid cystic carcinoma (AdCC), and many others.
OBJECTIVE
We aimed to identify new critical genes of MECa and AdCC using bioinformatics analysis.
METHODS
Gene expression profile of GSE153283 was analyzed by the GEO2R online tool to use the DAVID software for their subsequent enrichment. Protein-protein interactions (PPI) were visualized using String. Cytoscape with MCODE plugin followed by Kaplan-Meier online for overall survival analysis were performed.
RESULTS
97 upregulated genes were identified for MECa and 86 for AdCC. PPI analysis revealed 22 genes for MECa and 63 for AdCC that were validated by Kaplan-Meier that showed FN1 and SPP1 for MECa, and EGF and ERBB2 for AdCC as more significant candidate genes for each neoplasm.
CONCLUSION
With bioinformatics methods, we identify upregulated genes in MECa and AdCC. The resulting candidate genes as possible therapeutic targets were FN1, SPP1, EGF, and ERBB2, and all those genes had been tested as a target in other neoplasm kinds but not salivary gland neoplasm. The bioinformatic evidence is a solid strategy to select them for more extensive research with clinical impact.
PubMed: 36146635
DOI: 10.3390/vaccines10091557 -
Cancer Cytopathology Oct 2022Mucoepidermoid carcinoma (MEC) is the most common salivary gland (SG) malignancy. In this study, the author undertook analysis of a large collection of MEC cytologic...
BACKGROUND
Mucoepidermoid carcinoma (MEC) is the most common salivary gland (SG) malignancy. In this study, the author undertook analysis of a large collection of MEC cytologic cases.
MATERIALS AND METHODS
Cytopathology files were searched for MEC cases with histopathologic confirmation. Fine-needle aspiration (FNA) smears used standard technique.
RESULTS
Seventy-six cases (63 patients [M:F = 1:1; age range, 23-87 years; mean age, 58 years]) met inclusion criteria. Aspirates were primary (54 [71%]), metastatic (18 [24%]), and locally recurrent (4 [5%]). FNA sites included parotid gland (49 [64%]), regional lymph nodes (11 [14%]), submandibular gland (5 [7%]), inner canthus of eye (2 [3%]), and lung (2 [3%]); and single specimens from palate, jaw, shoulder, paranasal sinus, floor of mouth, ear canal, and effusion. Cytologic diagnoses included MEC (30 cases [39%]), suspicious for MEC (16 [21%]), non-MEC carcinoma (9 [12%]), suspicious for malignancy (SM) (2 [3%], malignant (M) (1 [1%]), SG and/or suspicious SG neoplasm (7 [8%]), atypical (3 [5%]), nonneoplastic (5 [6%]), nondiagnostic (2 [3%]), and benign SG neoplasm (1 [1%]). A total of 26% of low-grade (LG) cases were diagnosed as malignant in contrast to 87% malignant in high-grade (HG) cases. Cytomorphology depended on tumor grade. LG MEC contained intra- and/or extra-cellular mucin and more uniform cell and/or nuclear morphology, whereas cytologic atypia, anisonucleosis, and keratotic cells were more typical of HG tumors.
CONCLUSION
A malignant (M) or suspicious for malignancy (SM) cytologic interpretation was made in 76% of mucoepidermoid carcinoma (MEC) cases. In contrast to high-grade MEC (97% identified as M/SM), only 59% of low-grade (LG) MEC cases were interpreted as such, illustrating the continued diagnostic challenge posed by LG MEC using fine-needle aspiration biopsy.
Topics: Adult; Aged; Aged, 80 and over; Biopsy, Fine-Needle; Carcinoma, Mucoepidermoid; Humans; Middle Aged; Mucins; Salivary Gland Neoplasms; Submandibular Gland; Young Adult
PubMed: 35640091
DOI: 10.1002/cncy.22600 -
Acta Otorrinolaringologica Espanola 2016
Topics: Carcinoma, Mucoepidermoid; Child; Humans; Male; Parotid Neoplasms
PubMed: 26976338
DOI: 10.1016/j.otorri.2015.11.005