-
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 -
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 -
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 -
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 -
Signal Transduction and Targeted Therapy Jan 2021Mucoepidermoid carcinoma (MEC) is the most common type of salivary gland cancers and patients with advanced, metastatic, and recurrent MECs have limited therapeutic...
Mucoepidermoid carcinoma (MEC) is the most common type of salivary gland cancers and patients with advanced, metastatic, and recurrent MECs have limited therapeutic options and poor treatment outcomes. MEC is commonly associated with a chromosomal translocation t(11;19) (q14-21;p12-13) that encodes the CRTC1-MAML2 oncogenic fusion. The CRTC1-MAML2 fusion is required for MEC growth in part through inducing autocrine AREG-EGFR signaling. Growing evidence suggests that MEC malignancy is maintained by cancer stem-like cells. In this study, we aimed to determine critical signaling for maintaining MEC stem-like cells and the effect of combined targeting of stem cell signaling and CRTC1-MAML2-induced EGFR signaling on blocking MEC growth. First, we evaluated the significance of Notch signaling in regulating MEC stem-like cells. Aberrantly activated Notch signaling was detected in human fusion-positive MEC cells. The inhibition of Notch signaling with genetic or pharmacological inhibitors reduced oncosphere formation and ALDH-bright population in vitro and blocked the growth of MEC xenografts in vivo. Next, we investigated the effect of co-targeting Notch signaling and EGFR signaling, and observed enhanced inhibition on MEC growth in vivo. Collectively, this study identified a critical role of Notch signaling in maintaining MEC stem-like cells and tumor growth, and revealed a novel approach of co-targeting Notch and EGFR signaling as a potential effective anti-MEC treatment.
Topics: Animals; Carcinoma, Mucoepidermoid; Cell Proliferation; ErbB Receptors; Erlotinib Hydrochloride; Heterografts; Humans; Mice; Molecular Targeted Therapy; Neoplastic Stem Cells; Oncogene Proteins, Fusion; Receptors, Notch; Salivary Gland Neoplasms; Signal Transduction; Trans-Activators; Transcription Factors; Translocation, Genetic
PubMed: 33473104
DOI: 10.1038/s41392-020-00388-0 -
International Journal of Molecular... Jan 2023Patients with advanced/recurrent mucoepidermoid carcinoma (MEC) have a poor prognosis. This study aimed to establish and characterize human mucoepidermoid carcinoma cell...
Patients with advanced/recurrent mucoepidermoid carcinoma (MEC) have a poor prognosis. This study aimed to establish and characterize human mucoepidermoid carcinoma cell lines from the initial surgical specimen and biopsy specimen upon recurrence from the same patient to provide a resource for MEC research. MEC specimens from the initial surgical procedure and biopsy upon recurrence were used to establish cell lines. The established cell lines were cytogenetically characterized using multi-color fluorescence in situ hybridization and detection, and the sequence of the chimeric gene was determined. Furthermore, the susceptibility of head and neck mucoepidermoid carcinoma to standard treatment drugs such as cisplatin, 5-fluorouracil, and cetuximab was investigated. We successfully established unique MEC cell lines, AMU-MEC1, from an initial surgical specimen and AMU-MEC1-R1 and AMU-MEC1-R2 from the recurrent biopsy specimen in the same patient. These cell lines exhibited epithelial morphology and developed in vitro-like cobblestones. They shared eight chromosomal abnormalities, including der(19)ins(19;11)(p13;?), which resulted in a chimeric gene, indicating the same origin of the cell lines. The susceptibility of all cell lines to cisplatin and 5-fluorouracil was low. Interestingly, EGFR dependency for cell growth decreased in AMU-MEC-R1 and AMU-MEC-R2 but was retained in AMU-MEC1. These cytogenetic and biochemical findings suggest that the established cell lines can be used to investigate the disease progression mechanisms and develop novel therapeutics for MEC.
Topics: Humans; DNA-Binding Proteins; Trans-Activators; Carcinoma, Mucoepidermoid; In Situ Hybridization, Fluorescence; Cisplatin; Transcription Factors; Biopsy; Cell Line, Tumor; Fluorouracil; Salivary Gland Neoplasms; Oncogene Proteins, Fusion
PubMed: 36675234
DOI: 10.3390/ijms24021722 -
Journal of Cancer Research and... 2022Mucoepidermoid carcinoma (MEC) is a rare malignant thyroid neoplasm. Cases of MEC with papillary, insular, and anaplastic thyroid carcinoma have been reported. Here, we...
Mucoepidermoid carcinoma (MEC) is a rare malignant thyroid neoplasm. Cases of MEC with papillary, insular, and anaplastic thyroid carcinoma have been reported. Here, we present a case of follicular carcinoma with extensive MEC-like differentiation. A 62-year-old female presented with complaint of thyroid swelling for 10 years. Cytological features were suggestive of follicular neoplasm. Contrast-enhanced computed tomography showed metastasis to lung and vertebrae. Salivary glands and breasts were normal on examination and imaging. Subtotal thyroidectomy with bilateral neck dissection surgery was performed. The specimen was submitted for histopathological examination. Microscopy showed features of follicular carcinoma with capsular and vascular invasion along with an additional MEC-like morphology. Follicular carcinoma with extensive MEC-like differentiation is a rare observation. Since the tumor was sparing salivary glands and breasts, we considered it as mucoepidermoid differentiation over a collision tumor. However, immunohistochemistry and molecular analysis were the limitations.
Topics: Adenocarcinoma, Follicular; Carcinoma, Mucoepidermoid; Female; Humans; Middle Aged; Thyroid Neoplasms; Thyroidectomy
PubMed: 35900576
DOI: 10.4103/jcrt.JCRT_1422_20 -
American Journal of Ophthalmology Jul 2022In this study, we evaluated the clinicopathologic and molecular characteristics of lacrimal apparatus mucoepidermoid carcinoma (MEC) to define its typical diagnostic... (Observational Study)
Observational Study
PURPOSE
In this study, we evaluated the clinicopathologic and molecular characteristics of lacrimal apparatus mucoepidermoid carcinoma (MEC) to define its typical diagnostic features.
DESIGN
Retrospective observational case series.
METHODS
Institutional pathology records between 2011 and 2021 were searched for all cases of lacrimal apparatus MEC.
RESULTS
A total of 2 male and 6 female patients ranging in age from 18 to 83 years (median 56, mean 54) were included. Six lacrimal apparatus MECs were found in the lacrimal gland, and 2 cases occurred in the lacrimal sac and nasolacrimal duct. Histologically, there were 6 cases of conventional MEC, 1 clear-cell variant of MEC, and 1 oncocytic variant of MEC for a total of 8 cases. There were 3 low-grade cases and 5 high-grade cases. All 8 cases were evaluated via immunohistochemistry, and the results were positive (scores 1-4) for pankeratin, 34betaE12, p63, p40, CK7, CK8, and CK19, with a relatively higher expression of p63 observed in high-grade MEC. The presence of human papillomavirus (HPV) type 6 DNA was found in 4 patients. MAML2 fluorescence in situ hybridization was positive for MAML2 rearrangement in 3 lacrimal gland tumors (2 low-grade and 1 high-grade). Six tumors were managed with radical resection, and 2 patients underwent orbital exenteration. Postoperative radiation therapy was delivered to 6 patients, and chemotherapy was administered to 1 patient.
CONCLUSIONS
MECs of the lacrimal apparatus are rare tumors, and the rate of MAML2 translocations is lower than that in salivary MECs. Lacrimal gland and lacrimal sac MECs may not be of the same subtypes intrinsically because of the difference in MAML2 translocation, anatomy, and clinical course. The etiologic function of HPV type 6 infection should be explored in lacrimal apparatus MECs. Radical surgery is the treatment of choice. The description of these unique findings may assist in the definitive diagnosis of and improve our understanding of lacrimal apparatus MEC.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Carcinoma, Mucoepidermoid; Eye Neoplasms; Female; Humans; In Situ Hybridization, Fluorescence; Lacrimal Apparatus; Male; Middle Aged; Papillomavirus Infections; Retrospective Studies; Salivary Gland Neoplasms; Trans-Activators; Translocation, Genetic; Young Adult
PubMed: 35288069
DOI: 10.1016/j.ajo.2022.03.008 -
Thoracic Cancer Aug 2022Pulmonary mucoepidermoid carcinoma (PMEC) is uncommon. The purpose of this study was to evaluate the clinicopathological features, diagnostic criteria, treatment...
Pulmonary mucoepidermoid carcinoma (PMEC) is uncommon. The purpose of this study was to evaluate the clinicopathological features, diagnostic criteria, treatment options, and prognostic factors relating to primary PMEC. Clinical data on 45 patients with primary PMEC were collected and analyzed retrospectively at Tianjin Medical University General Hospital and the First People' Hospital of Longquanyi District Chengdu from January 2008 to December 2020. The 45 patients (25 males and 20 females) ranged in age from 22 to 72 years, with a median age of 49 and an average age of 47.7. All the patients underwent surgery, with 32 receiving only surgery and 13 receiving both surgery and postoperative chemotherapy. A total of 34 instances of low-grade tumors and 11 cases of high-grade tumors were discovered during postoperative pathological diagnosis. Forty-five patients were followed for 13 to 78 months, and four died during this period. In all four instances, a lung infection unrelated to the tumor was determined to be the cause of death. The MAML2 gene translocation was detected in 40 of 45 patients, with 34 of them testing positive. Radical surgery with lymph node dissection is an efficient treatment for PMEC. The prognosis is poor for patients with advanced disease, a negative MAML2 gene translocation, lymph node metastases, and high-grade tumors.
Topics: Adult; Aged; Carcinoma, Mucoepidermoid; Female; Humans; Lung Neoplasms; Male; Middle Aged; Neoplasm Staging; Prognosis; Retrospective Studies; Transcription Factors; Translocation, Genetic; Young Adult
PubMed: 35747926
DOI: 10.1111/1759-7714.14536 -
Zhongguo Yi Xue Ke Xue Yuan Xue Bao.... Jun 2022Objective To improve the understanding and diagnostic accuracy of pulmonary mucoepidermoid carcinoma(PMEC) by analyzing the imaging and clinical characteristics.Methods...
Objective To improve the understanding and diagnostic accuracy of pulmonary mucoepidermoid carcinoma(PMEC) by analyzing the imaging and clinical characteristics.Methods The clinical and CT data of 27 cases of PMEC confirmed by histopathology in the First Medical Center of Chinese PLA General Hospital from January 2016 to December 2020 were retrospectively analyzed,including the location,size,margin,density,enhancement characteristics,accompanying signs,and pathological grade.Results The 27 cases included 6(6/27,22.2%) of large airway type,14(14/27,51.9%) of hilar type,and 7(7/27,26.9%) of peripheral type.The CT manifestations of 20 cases of large airway and hilar PMEC were soft-tissue nodules or mass with clear boundary in the lumen of the trachea and main bronchi,including 6 cases of mild enhancement,4 cases of moderate enhancement,5 cases of marked enhancement,and 5 cases of uneven enhancement.Three of the 20 cases showed calcification.The 7 cases of peripheral PMEC showed soft-tissue nodules or masses in the lungs,including 3 cases of mild enhancement,1 case of moderate enhancement,and 3 cases of marked enhancement. Obstructive pneumonia or atelectasis and bronchiectasis with mucus plug formation occurred in 16(16/27,59.3%) cases,lymph node metastasis in 9(9/27,33.3%) cases,and multiple organ metastasis in 8(8/27,29.6%) cases.Age(=-3.132,=0.005),enlarged lymph node (=9.281,=0.003),and distant metastasis(=7.816,=0.008) were statistically significant in the low-grade group and high-grade group. Conclusion PMEC have some unique imaging features,and recognizing these signs is conducive to the differential diagnosis and the improvement of the diagnostic accuracy.
Topics: Carcinoma, Mucoepidermoid; Child, Preschool; Humans; Lung Neoplasms; Lymphatic Metastasis; Retrospective Studies; Tomography, X-Ray Computed
PubMed: 35791941
DOI: 10.3881/j.issn.1000-503X.14939