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Oral Oncology Dec 2021Mucoepidermoid carcinoma (MEC) is one of the most common salivary gland malignancies. Our aim was to evaluate the prognostic impact of primary tumor site in patients...
UNLABELLED
Mucoepidermoid carcinoma (MEC) is one of the most common salivary gland malignancies. Our aim was to evaluate the prognostic impact of primary tumor site in patients with MEC.
MATERIAL AND METHODS
This cohort identified 308 patients with MEC who underwent primary surgery between 1985 and 2015. Survival outcomes were determined using the Kaplan-Meier method. Hazard ratios for primary site were determined using the Cox proportional-hazards model.
RESULTS
One hundred eighty (58%) patients were diagnosed with minor and 128 (42%) with major salivary gland cancer. Primary site in the minor salivary gland group included 137 (44%) oral cavity, 38 (12%) pharynx, 3 (0.9%) nasal cavity, and 2 (0.6%) trachea and larynx. The major salivary gland group included 118 (38%) parotid, 8 (3%) submandibular, and 2 (0.6%) sublingual. With a median follow-up of 73 months, 5-year overall survival and disease-specific survival were 84% and 91%, respectively. Patients with tumors located in the hard palate and retromolar trigone had the best survival, while patients with tumors located in the paranasal sinuses and submandibular gland had the poorest survival. After controlling for tumor grade and stage, MEC primary site was not predictive of survival or recurrence. On multivariate analysis, worse DSS was associated with stage III-IV tumors (HR: 7,11; 95% CI: 1.19-26.43; p = 0.0034) and high-grade tumors (HR: 19.12; 95% CI: 2.26-162.77; p = 0.0068).
CONCLUSIONS
While high grade and advanced overall stage were found to be independent predictors of worse survival, primary tumor site was not predictive of poor outcome.
Topics: Carcinoma, Mucoepidermoid; Humans; Neoplasm Staging; Prognosis; Retrospective Studies; Salivary Gland Neoplasms; Survival Rate
PubMed: 34768210
DOI: 10.1016/j.oraloncology.2021.105602 -
Diagnostic Pathology Jan 2014In rare condition, combined thymic epithelial tumors showing either type A or type B thymomas areas combined with thymic carcinoma components may occur in thymus.... (Review)
Review
BACKGROUND
In rare condition, combined thymic epithelial tumors showing either type A or type B thymomas areas combined with thymic carcinoma components may occur in thymus. Mucoepidermoid carcinoma (MEC) of the thymus is rare in thymic carcinoma, and so far there is no report to describe a combined epithelial tumor of thymus with MEC component. We report an unusual case of combined thymic MEC/type B2 thymoma in a middle-aged male occurring in a mass of anterior mediastinum.
CASE REPORT
A 51-year-old Chinese male patient presented with a 6-month history of right ptosis and progressive muscle weakness. Computed tomography (CT) examination revealed a solitary, well-circumscribed mass was in the anterior mediastinum with mild heterogeneous enhancement. Histologically, the mass contained two separated components and displayed typically histological features of low-grade MEC and type B2 thymoma, respectively. There was no gradual transition of these two components observed in mass, and no enlarged lymph node was found in the surrounding tissues. A diagnosis of combined thymic MEC/type B2 thymoma was made. The patient received thymectomy to resect the mass totally. After surgery, chemotherapy with regiments of cisplatin and mitomycin, and radiotherapy of the main tumor bed were performed on the patient. There was no evidence of tumor recurrence during the period of 12 months follow-up.
CONCLUSION
To our best knowledge, this is the first report of combined thymic epithelial tumor with MEC component. Although this tumor is rare, the diagnosis of a thymic MEC should be taken into consideration when a combined epithelial tumor is occasionally encountered in thymus.
VIRTUAL SLIDES
The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/9721397571157894.
Topics: Carcinoma, Mucoepidermoid; Humans; Male; Middle Aged; Neoplasms, Multiple Primary; Thymoma; Thyroid Neoplasms
PubMed: 24444077
DOI: 10.1186/1746-1596-9-8 -
Archives of Pathology & Laboratory... Sep 2007Although mucoepidermoid carcinoma of the salivary gland is relatively common, mucoepidermoid carcinoma arising from the mucous glands of the bronchus is rare. Bronchial... (Review)
Review
Although mucoepidermoid carcinoma of the salivary gland is relatively common, mucoepidermoid carcinoma arising from the mucous glands of the bronchus is rare. Bronchial mucoepidermoid carcinoma usually presents as an intraluminal mass producing luminal occlusion. Symptoms are airway obstruction and recurrent pneumonia. Macroscopically, mucoepidermoid carcinoma appears as an exophytic intrabronchial mass with intact or ulcerated bronchial mucosa. Microscopically, the tumors are located in the submucosa of the large bronchi. The tumors are usually well differentiated and contain a combination of mucus-secreting, squamous, and intermediate cells. The increased frequency of this tumor in the pediatric population suggests a genetic abnormality. Recent genetic studies have demonstrated reciprocal chromosomal translocations including t(1;11)(p22;q13), t(11;19)(q14-21;p12), and t(11; 19)(q21;p13). Chromosome 11 in the first translocation appears to have been altered resulting in up-regulation of the cyclin D1 gene and overexpression of cyclin D1. The t(11;19)(q21;p13) encodes a novel fusion product capable of disrupting the Notch signaling pathway.
Topics: Bronchi; Bronchial Neoplasms; Carcinoma, Mucoepidermoid; Cyclin D1; Diagnosis, Differential; Gene Expression Regulation, Neoplastic; Humans; Respiratory Mucosa; Translocation, Genetic
PubMed: 17824797
DOI: 10.5858/2007-131-1400-MCOTBA -
Medicine Dec 2017Mucoepidermoid carcinoma (MEC) of the breast is a rare entity comprising specific morphological and immunohistochemical features, and has been previously only reported... (Review)
Review
RATIONALE
Mucoepidermoid carcinoma (MEC) of the breast is a rare entity comprising specific morphological and immunohistochemical features, and has been previously only reported in 33 cases.
PATIENT CONCERNS
Four cases of MEC of the breast are reported in this study. All patients were women with ages ranging from 39 to 66 years. The lesions consisted of neoplastic solid nests and cystic spaces sometimes filled with mucoid material.
DIAGNOSES
At high power, the tumors were composed of various proportions of basaloid, intermediate, epidermoid, and mucinous cells in different cases. All cases were classified as low-grade MEC of the breast. Tumor cells exhibited low levels of hormonal receptor expression in two cases (cases 1 and 3), and immunonegativity in one case (case 2). On the contrary, estrogen receptors (ER) were positively expressed in 60% of tumor cells in case 4. Tumor cells did not express human epidermal growth factor receptor 2 (HER-2)/neu protein in all the cases.
INTERVENTIONS
Modified radical mastectomy (Auchincloss) was performed in the first two cases, while the remaining two patients underwent mastectomy plus sentinel lymph node biopsy.
OUTCOMES
All patients were alive and well without evidence of recurrent disease after a period ranging from 4 months to 156 months.
LESSONS
MEC of the breast is a rare primary carcinoma that is difficult to diagnose. Multiple tissue blocks are necessary before obtaining all cell types. Special stains for mucin and electron microscopy would be helpful in suspected cases. Hormonal factors might have an impact on the biological behavior of tumors, but further studies are needed to draw conclusions.
Topics: Adult; Aged; Breast Neoplasms; Carcinoma, Mucoepidermoid; Female; Humans; Mastectomy, Modified Radical; Middle Aged; Sentinel Lymph Node Biopsy
PubMed: 29390541
DOI: 10.1097/MD.0000000000009385 -
Head and Neck Pathology Dec 2016The aim of this study was to compare the immunoexpression of epithelial mucins (MUCs) in salivary duct cysts, papillary cystadenomas, and mucoepidermoid carcinomas and...
The aim of this study was to compare the immunoexpression of epithelial mucins (MUCs) in salivary duct cysts, papillary cystadenomas, and mucoepidermoid carcinomas and to evaluate if any of these markers could be useful for differentiating between mucoepidermoid carcinoma and papillary cystadenoma. We also sought to validate the p63 expression pattern found to differentiate between mucoepidermoid carcinoma and papillary cystadenoma. Immunoexpression of MUC1, MUC2, MUC4, MUC7, and p63 was studied and quantified in 22 mucoepidermoid carcinomas, 12 papillary cystadenomas, and 3 salivary duct cysts. The immunohistochemical evaluation was collectively performed by 3 oral pathologists. Scores and trends in proportions were assessed using the nonparametric Wilcoxon-Mann-Whitney rank sum test. Mucoepidermoid carcinomas, papillary cystadenomas, and salivary duct cysts demonstrated variable MUC expression patterns. All tumors were positive for p63 immunoexpression with p63 labeling in salivary duct cysts and papillary cystadenomas (15/15) limited to the basal layers of the cystic spaces, whereas in mucoepidermoid carcinomas (22/22) the p63 labeling extended throughout the suprabasal layers (p < 0.001). This study adds more confirmatory data to validate that the reactivity pattern of p63 protein can be used in distinguishing between papillary cystadenoma and low-grade mucoepidermoid carcinoma. Although positive reactivity in a tumor with MUC1 and MUC4 was inconclusive, negative reactivity suggests the diagnosis of a benign PC or SDC.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Biomarkers, Tumor; Carcinoma, Mucoepidermoid; Cystadenoma, Papillary; Diagnosis, Differential; Female; Humans; Immunohistochemistry; Male; Membrane Proteins; Middle Aged; Mucins; Salivary Gland Neoplasms
PubMed: 27278378
DOI: 10.1007/s12105-016-0735-4 -
Modern Pathology : An Official Journal... Feb 2013Mucoepidermoid carcinoma is the most common salivary gland malignancy, and includes a spectrum of lesions ranging from non-aggressive low-grade tumors to aggressive...
Mucoepidermoid carcinoma is the most common salivary gland malignancy, and includes a spectrum of lesions ranging from non-aggressive low-grade tumors to aggressive high-grade tumors. To further characterize this heterogeneous group of tumors we have performed a comprehensive analysis of copy number alterations and CRTC1-MAML2 fusion status in a series of 28 mucoepidermoid carcinomas. The CRTC1-MAML2 fusion was detected by RT-PCR or fluorescence in situ hybridization in 18 of 28 mucoepidermoid carcinomas (64%). All 15 low-grade tumors were fusion-positive whereas only 3 of 13 high-grade tumors were fusion-positive. High-resolution array-based comparative genomic hybridization revealed that fusion-positive tumors had significantly fewer copy number alterations/tumor compared with fusion-negative tumors (1.5 vs 9.5; P=0.002). Twelve of 18 fusion-positive tumors had normal genomic profiles whereas only 1 out of 10 fusion-negative tumors lacked copy number alterations. The profiles of fusion-positive and fusion-negative tumors were very similar to those of low- and high-grade tumors. Thus, low-grade mucoepidermoid carcinomas had significantly fewer copy number alterations/tumor compared with high-grade mucoepidermoid carcinomas (0.7 vs 8.6; P<0.0001). The most frequent copy number alterations detected were losses of 18q12.2-qter (including the tumor suppressor genes DCC, SMAD4, and GALR1), 9p21.3 (including the tumor suppressor genes CDKN2A/B), 6q22.1-q23.1, and 8pter-p12.1, and gains of 8q24.3 (including the oncogene MAFA), 11q12.3-q13.2, 3q26.1-q28, 19p13.2-p13.11, and 8q11.1-q12.2 (including the oncogenes LYN, MOS, and PLAG1). On the basis of these results we propose that mucoepidermoid carcinoma may be subdivided in (i) low-grade, fusion-positive mucoepidermoid carcinomas with no or few genomic imbalances and favorable prognosis, (ii) high-grade, fusion-positive mucoepidermoid carcinomas with multiple genomic imbalances and unfavorable prognosis, and (iii) a heterogeneous group of high-grade, fusion-negative adenocarcinomas with multiple genomic imbalances and unfavorable outcome. Taken together, our studies indicate that molecular genetic analysis can be a useful adjunct to histologic scoring of mucoepidermoid carcinoma and may lead to development of new clinical guidelines for management of these patients.
Topics: Adolescent; Adult; Aged; Carcinoma, Mucoepidermoid; Child; DNA-Binding Proteins; Diagnosis, Differential; Female; Gene Expression Regulation, Neoplastic; Humans; Male; Middle Aged; Nuclear Proteins; Oncogene Proteins, Fusion; Prognosis; Trans-Activators; Transcription Factors
PubMed: 23018873
DOI: 10.1038/modpathol.2012.154 -
Oral Oncology Apr 2021Mucoepidermoid carcinoma (MEC) is the most common type of salivary gland malignancy. Advanced or high-grade MECs are refractory to chemotherapy, often leading to tumor...
OBJECTIVES
Mucoepidermoid carcinoma (MEC) is the most common type of salivary gland malignancy. Advanced or high-grade MECs are refractory to chemotherapy, often leading to tumor recurrence/metastasis and abysmal ~35% 5-year survival. Causal links have been established between Epithelial Growth Factor Receptor (EGFR) activation and poor outcome. Herein we investigated the therapeutic efficacy of EGFR inhibition against MEC using in vitro pre-clinical models.
MATERIALS AND METHODS
Five human MEC cell lines were used in cell viability, cytotoxicity, apoptosis, cell cycle, 2D-clonogenicity, and 3D-spheroid formation assays following treatment with Erlotinib (EGFR inhibitor), SAHA (Histone Deacetylase inhibitor; HDAC) and CUDC-101 (dual EGFR-HDAC inhibitor). Effects on MEC cancer stem cells were evaluated using flow cytometry. Gene expression and pathway regulation were evaluated via qPCR and Western blot, respectively.
RESULTS
MEC cells enter a quiescent, non-proliferative yet rapidly reversible drug tolerant state upon EGFR inhibition. Despite robust suppression of MEC cell proliferation, no discernable apoptosis is detected. Combination of EGFR and HDAC inhibitors exhibits synergistic effects, exerting ~5-fold more potent cell cytotoxicity compared to HDAC or EGFR monotherapy. CUDC-101, a single molecule with dual EGFR-HDAC inhibitor moieties, exerts irreversible and potent cytotoxic activity against MEC cells and blunts MEC cancer stem-cell tumorigenicity.
CONCLUSION
MEC cells are intrinsically tolerant to EGFR inhibition. Combining EGFR and HDAC inhibitors exerts synergistic and potent cytotoxic effects, suggesting that EGFR inhibitors still hold significant promise against MEC. Future studies are needed to assess the applicability and efficacy of dual EGFR-HDAC inhibitors for the clinical management of MEC.
Topics: Carcinoma, Mucoepidermoid; Cell Line, Tumor; ErbB Receptors; Histone Deacetylase Inhibitors; Humans; Salivary Gland Neoplasms
PubMed: 33581505
DOI: 10.1016/j.oraloncology.2020.105166 -
Medicine Jul 2022Mucoepidermoid carcinoma (MEC) of the breast is a rare entity, with an estimated incidence of only 0.2% to 0.3% of all primary breast tumors. The radiological features... (Review)
Review
RATIONALE
Mucoepidermoid carcinoma (MEC) of the breast is a rare entity, with an estimated incidence of only 0.2% to 0.3% of all primary breast tumors. The radiological features of breast MEC have scarcely been investigated mainly because of its rarity. In this article, we present a case of breast MEC diagnosed at our hospital and review the literature, focusing on radiological findings and radiologic-pathologic correlations that could improve clinical management of this entity. To the best of our knowledge, our study is the first review of the literature that focuses on the radiological features of breast MEC.
PATIENT CONCERNS
A 47-year-old premenopausal woman presented with a painless palpable mass in the right breast.
DIAGNOSIS
Mammography and ultrasonography revealed a mass with suspicious malignant features, which was categorized as Breast Imaging Reporting and Data System category 4c. A 14-gauge core-needle biopsy revealed an intermediate-grade MEC of the breast. The patient underwent breast magnetic resonance imaging and chest computed tomography for preoperative evaluation. Postoperative histopathological examination confirmed a diagnosis of intermediate-grade MEC. The clinical staging was T2N0M0.
INTERVENTIONS
The patient underwent breast-conserving surgery, adjuvant chemotherapy, radiotherapy, and hormonal therapy.
OUTCOMES
No evidence of recurrence has been reported over 37 months.
LESSONS
The imaging characteristics of breast MEC were variable, and there were no specific radiological features for diagnosis. The presence of cystic components on radiological imaging is likely to be an indicator of a low-grade tumor and better prognosis, although the number of reported cases is limited.
Topics: Breast; Breast Neoplasms; Carcinoma, Mucoepidermoid; Female; Humans; Magnetic Resonance Imaging; Mammography; Middle Aged
PubMed: 35777033
DOI: 10.1097/MD.0000000000029745 -
Modern Pathology : An Official Journal... Jul 2020In 2018, the consensus meeting for the WHO Classification of Tumours of the Eye decided that conjunctival mucoepidermoid carcinoma should be reclassified as...
Conjunctival 'mucoepidermoid carcinoma' revisited: a revision of terminology, based on morphologic, immunohistochemical and molecular findings of 14 cases, and the 2018 WHO Classification of Tumours of the Eye.
In 2018, the consensus meeting for the WHO Classification of Tumours of the Eye decided that conjunctival mucoepidermoid carcinoma should be reclassified as adenosquamous carcinoma, as this represented a better morphological fit. To examine the applicability of this terminology, we studied the clinical, histopathological, immunohistochemical and molecular pathology of 14 cases that were originally diagnosed as conjunctival mucoepidermoid carcinoma. There were 7 (50%) females and 7 (50%) males. The median age was 64 years. The left eye was affected in 8 and the right eye in 6 patients. In-situ carcinoma was present in 11/14 (79%) cases and comprised in-situ squamous cell carcinoma (SCC) and conjunctival intraepithelial neoplasia with mucinous differentiation (CIN-Muc). Invasive carcinoma was present in 11/14 (79%) cases. Group 1 (1/11 cases, 9%) comprised invasive SCC only. Group 2 (6/11 cases, 55%) comprised SCC with mucinous differentiation, manifesting as scattered intracellular mucin, occasionally together with intercellular mucin, with no evidence of true glandular differentiation. Group 3 (3/11 cases. 27%) comprised true adenosquamous carcinoma. Group 4 (1/11 cases, 9%) comprised pure adenocarcinoma. Thirteen of 14 cases (93%) underwent FISH for MAML2 translocation and none were rearranged. Two cases harboured high-risk HPV (type 16 and 18). The combined findings confirm that all lesions in our study were not mucoepidermoid carcinoma, but represented predominantly SCC with mucinous differentiation and adenosquamous carcinoma. We, therefore, recommend future revision of the WHO classification to include SCC with mucinous differentiation alongside adenosquamous carcinoma.
Topics: Aged; Aged, 80 and over; Biomarkers, Tumor; Carcinoma, Adenosquamous; Carcinoma, Mucoepidermoid; Carcinoma, Squamous Cell; Conjunctival Neoplasms; Female; Humans; Male; Middle Aged; World Health Organization
PubMed: 31932683
DOI: 10.1038/s41379-020-0456-9 -
Journal of Medical Case Reports Jan 2019Warthin tumor is a common, benign, painless salivary gland neoplasm. Rarely, Warthin tumors show large areas of squamous metaplasia; such Warthin tumors are called... (Review)
Review
BACKGROUND
Warthin tumor is a common, benign, painless salivary gland neoplasm. Rarely, Warthin tumors show large areas of squamous metaplasia; such Warthin tumors are called metaplastic or infarcted Warthin tumors because they are occasionally accompanied with tumor necrosis. The histological distinction between mucoepidermoid carcinomas and the metaplastic portions of Warthin tumors can be challenging; without a genetic study, mucoepidermoid carcinomas can be misdiagnosed as metaplastic Warthin tumors. We report a case of infarcted Warthin tumor partly showing mucoepidermoid carcinoma-like epithelial metaplasia. Only two cases of infarcted Warthin tumor similar to our case have been reported.
CASE PRESENTATION
A 69-year-old Japanese man presented with a right parotid tumor. He had noticed the swelling on his right buccal region 1 year previously; the lesion had rapidly enlarged, with associated pain, 1 month previously. A radiological examination revealed a mass in the tail of the right parotid gland. Superficial parotidectomy was performed. On histological examination, the mass showed typical focal features of Warthin tumor; other areas showed coagulation necrosis of the tumor. These areas were surrounded by non-oncocytic epithelium comprising squamous and mucinous epithelial cells. Although cellular atypia of the non-oncocytic epithelium was not observed, a mixture of squamous and mucinous cells and lack of abundant lymphoid tissue mimicked low-grade mucoepidermoid carcinoma. Based on the results of fluorescence in situ hybridization, MAML2 gene rearrangement was not present in the typical portions of Warthin tumor and the mucoepidermoid carcinoma-like lesion. Therefore, a metaplastic or infarcted Warthin tumor was diagnosed. Our patient was disease-free 8 months after surgery.
CONCLUSIONS
Clinicians need to know that pain is a clinical symptom of infarcted/metaplastic Warthin tumor. Pathologists should be aware that a metaplastic Warthin tumor can mimic a low-grade mucoepidermoid carcinoma. Our case showed a mucoepidermoid carcinoma-like lesion that was confined near the area of tumor necrosis, and neither cytological atypia nor apparent invasive growth was present. These findings appeared to be histological clues of a metaplastic Warthin tumor rather than a mucoepidermoid carcinoma. Careful clinicopathological evaluation as well as genetic studies are needed to clarify the distinction between mucoepidermoid carcinoma and metaplastic portions of Warthin tumors.
Topics: Adenolymphoma; Aged; Carcinoma, Mucoepidermoid; Humans; In Situ Hybridization, Fluorescence; Male; Metaplasia; Salivary Gland Neoplasms; Salivary Glands
PubMed: 30636634
DOI: 10.1186/s13256-018-1941-3