Clinically, MECA can exhibit aggressive characteristics with a tendency for relapse and metastasize, even when it is intracapsular, or may show minimally invasive characteristics that are subclassified as de novo or ex-pleomorphic adenoma (PA) [2,4,5]. They noted an overall disease-specific survival (DSS) at 60 and 120months of 91.3% and 90.2%, respectively. The 10-year OS rate in our cohort was 53%, and the histological grade, T/N/M stage, and radiotherapy were independent prognostic risk factors for OS. Tumor cells frequently contain intracytoplasmic vacuoles, imparting a pseudolipoblast appearance. One of these studies systematically reviewed all data of 691 patients and found that those with a positive margin may benefit from adjuvant radiotherapy [9]. WebThe histopathological types included salivary duct carcinoma (8 cases), adenoid cystic carcinoma (6 cases), myoepithelial carcinoma (3 cases), Squamous cell carcinoma (2 cases), adenocarcinoma (2 cases), acinic cell carcinoma (2 cases) and mucoepidermoid carcinoma (1 case). No two people are alike, and responses to treatment can vary greatly. The prognosis of Myoepithelial Carcinoma is based on several factors including the stage of the tumor, but is generally guarded. Also, tumor recurrences are not uncommon Malignant rhabdoid tumor and proximal-type epithelioid sarcoma have strikingly similar morphologic and immunohistochemical features. Our findings suggest that the parotid gland were the most common sites for head and neck MC, and this tumor recurs frequently at local sites and metastases occur commonly, the prognosis was poor. Symptoms of MEC usually begin with a painless mass. However, chemo-agent interventions should be considered in the management of MECA patients with metastasis, and the use of cisplatin-based chemotherapy, or ifosfamide and etoposide, has been discussed several studies [2,6,24]. Squamous metaplasia is acceptable in myoepithelial carcinoma. The relationship between clinicopathological features and overall survival (OS) was assessed using statistical analyses. Maintaining a safe surgical margins decreases the incidence of recurrence and has a significant effect on the OS [6,9]. The Kaplan-Meier model was used to estimate actuarial survival. The 19732014 SEER (Surveillance, Epidemiology, and End Results) cancer database was queried for patients treated for epithelial-myoepithelial carcinoma. Ossifying fibromyxoid tumor is generally negative for EMA and keratin. Indeed, histologically, MECA was shown to closely mimic PA; and the diagnosis of MECA was dependent on the expansile nodular lobulated pattern and zonal cellular distribution [2]; patients with this condition show a positive immunohistochemical staining for a AE1/AE3, CAM5.2, S100, calponin, SMA, CK7, P63, CK5/6, and INI1 in the salivary gland and other rare primary sites such as the larynx, lung, vulvar, shoulder, and soft tissues [2,3,[11], [12], [13], [14]]. government site. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). After PSM, the patients did not benefit from additional radiotherapy [135 months (95% CI: 83170) vs. 136 months (95% CI: 95171) months (P=0.8)] (Fig. The median OS was 142 months, while the survival rates at 120 months and 180 months were 53% and 39%, respectively. Figure3 shows the Kaplan-Meier actuarial overall survival by N stage. A total of 468 patients were identified. Chemotherapy (P<0.05), grade (P<0.05), and stage (T, N, M, P<0.05) had a significant effect on CSS both before and after PSM (Fig. Epithelial-myoepithelial carcinoma is an uncommon malignant neoplasm seen most frequently in the salivary glands, representing approximately 1 to 2% of salivary gland tumors. Int J Pediatr Otorhinolaryngol. Whenever possible, it is important for the entire tumor to be removed, with wide margins of healthy tissue surrounding the tumor. Radiotherapy (HR: 0.58, P=0.042) was a favorable factor for OS, and it reduced the mortality risk by 42%. Provided by the Springer Nature SharedIt content-sharing initiative. Mean survival time was 165.5months. Children's Cancer Therapy Development Institute. 18F-FDG PET/CT findings in a rare myoepithelial carcinoma arising in the parapharyngeal space. Other symptoms vary depending on the site of the tumor and may include hoarseness, nasal blockage, bleeding from the nose, pain, headaches, and facial weakness and paralysis. The National Cancer Institute has more information on how chemotherapy is used to treat cancer. Federal government websites often end in .gov or .mil. Kane S.V., Bagwan I.N. Patients have ranged from 6 to 98 years in age with a mean in the early to mid-40s.145,401 As with other benign salivary gland tumors, myoepitheliomas typically present as slowly enlarging, asymptomatic masses, usually 1 to 5 cm in diameter. Vazquez et al. The 3-year and 5-year overall survival rates were 59% and 31.8%, respectively. Overall 5-, 10-, and 20-year survival was 72.7%, 59.5%, and 38.3%, respectively. Most start in the parotid glands. In this case, biomarker testing can find out whether someones cancer has an NTRK gene change that may be treated with an TRK inhibitor, like the drug larotrectinib. The reported molecular features were TGFBR3-PLAG1 fusion and SMARCB1-deficiency [12,[15], [16], [17]], and patients with clear cell MECA may present with other PLAG1 fusions including LIFR-PLAG1, CTNNB1-PLAG1, CHCHD7-PLAG1, EWSR1-ATF1, FGFR1-PLAG1, and CTNNB1-PLAG1 [17,18]. Tumor cells are arranged as sheets, nests, or cords. Multi-dimensional genomic analysis of myoepithelial carcinoma identifies prevalent oncogenic gene fusions. The distant organs that were invaded included the lung, liver, bone, and brain (in descending order of frequency). For example, in some cases of MEC, a gene called EWSR1 joins with a region called KLF15, KLF17, PBX3, PBX1, ATF1, POU5F1, or ZNF444. Epithelial-myoepithelial carcinoma: a population-based survival analysis, https://doi.org/10.1186/s12901-018-0063-2, Surveillance epidemiology and end results, http://creativecommons.org/licenses/by/4.0/, http://creativecommons.org/publicdomain/zero/1.0/. Talk with your doctor to discuss whether biomarker testing for cancer treatment should be part of your care. found that adjuvant radiotherapy decreased the risk of local relapse (P=0.008) but increased the risk of mortality (P=0.04) as shown in the univariate analysis [9]. If you have any questions about how this information may pertain to your or your childs case, please discuss it with your doctor. Copyright 2023 Elsevier B.V. or its licensors or contributors. 136 View chapter Purchase book Salivary Gland Parotid gland was the most common As this period is contained within the 19732010 period, this may have had some effect on the epithelial-myoepithelial carcinoma cases reported during this time period. When stomach cancer is found early, there is a better chance of recovery. Doctors base MEC survival rates on how groups of people with MEC have done in the past. The malignant cell population is exclusively myoepithelial. On the other hand, myoepithelioma/parachordoma and peripheral chordoma may show considerable immunophenotypic overlap in reference to keratin subtypes, EMA, vimentin, S-100 protein, and collagen type IV. Table1 illustrates the demographic data for age, sex, and race of the cohort. However, further studies are required to assess the value of radiotherapy in MECA. In addition, owing to its rarity, this tumor is under-recognized and can be misdiagnosed [2]. Less than 600 cases have been reported in the literature since its initial description in 1972. The aim of this study was to examine demographic, site, stage, and survival factors in patients with epithelial-myoepithelial carcinoma. In the 2015 study [2] patients with low-grade histology and lesions <4cm had significantly better survival vs. patients with high-grade histology and tumors >4cm. All authors have read and agreed to the published version of the manuscript. Your US state privacy rights, Given the above morphologic and molecular similarities, it has been proposed that proximal-type epithelioid sarcoma and malignant rhabdoid tumor are a single entity; however, differences in clinical presentation, the amount of rhabdoid component distribution, and overall survival support keeping these tumors as separate designations. Endod. Ductal structures differentiate epithelial-myoepithelial carcinoma from clear cell myoepithelial carcinoma. The nodal and distant metastasis rates are low. 5). Patients did not appear to benefit from additional chemotherapy, as the median OS times of patients with and without chemotherapy were 26 months (95% CI: 2130) and 152 months (95% CI: 102200) months, respectively. It is interesting to note that between 1973 and 2010 there were 246 salivary gland epithelial-myoepithelial carcinomas reported (an average of approximately 6.6 per year), while in the updated data in the present study a further 70 epithelial-myoepithelial carcinomas of the major salivary glands (parotid + submandibular gland) were recorded in the next 4years from 2010 to 2014 (an average of approximately 17.5 per year). It can happen anywhere on the body. Yokose C., Asai J., Kan S., Nomiyama T., Takenaka H., Konishi E., Goto K., Ansai S.-.I., Katoh N. Myoepithelial carcinoma on the right shoulder: case report with published work review. The diagnostic signs of MECA may be easily overlooked, and they can be misclassified as a benign salivary gland neoplasms such as cellular or myoepithelial-rich pleomorphic adenoma (PA) [2]. Like all tumors with a myoepithelial component, MEC tumors exhibit a wide morphologic and cytologic diversity that is based on four major types of cells, similar to those seen in benign myoepithelioma: spindle, plasmacytoid (hyaline), epithelioid, and clear cell types. [10]. The definition and diagnostic criteria for MECA have been reported in the literature since 1995 [1]. Squamous metaplasia is acceptable in, Non-neuroendocrine carcinomas (excluding sarcomatoid carcinoma) and salivary gland-type tumors of the lung, Gattuso's Differential Diagnosis in Surgical Pathology (Fourth Edition), Diagnostic Surgical Pathology of the Head and Neck (Second Edition), Translocation-Specific Salivary Gland Tumors, Fibroblastic and myofibroblastic tumors of the skin, Diagnostic Atlas of Cutaneous Mesenchymal Neoplasia, Immunohistology of Soft Tissue and Osseous Neoplasms, Diagnostic Immunohistochemistry (Third Edition), Soft tissue myoepitheliomas and their malignant counterparts (, Mesenchymal Lesions of the Vulva and Vagina. This demonstrated that age (p<0.0001), race (p=0.02), AJCC stage (p<0.0001), T (p=0.006), N (p<0.0001), and M (p=0.003) stage, and treatment type (p<0.0001) were significantly associated with survival. Scientists are always working to understand how cancer forms, but it can be hard to prove. The main limitations of the current study were as follows: some details regarding the therapeutic intervention including extent of resection or irradiation, especially neck lymph node dissection and surgical margins, irradiation dose, and chemotherapy regimen were not available. 88.2% of patients had surgery as part or all of the treatment regimen. The factors that showed a significant association in the KM analysis were included in the Cox hazard regression model. Due to the absolute rarity of this cancer, it is poorly understood and no treatment options specific to MEC currently exist. From low power, the lesions are multilobulated with a significant component of stromal matrix that may be variably myxoid, myxochondroid, or hyalinized. White patients represented 78.0% of the total. They develop less often in the MECA, myoepithelial carcinoma. If the tumor grows near the surface of the skin, you will be able to see and feel the tumor. Conventional chemotherapy demonstrated some activity in myoepithelial carcinoma, In their review of 246 cases of salivary gland epithelial-myoepithelial carcinoma from the 19732010 SEER database, Vazquez et al. The low rates of distant metastasis may be attributable to the differences in the histological sub-classification, genetic features, and inadequate imaging examinations. Radiation therapy: Radiation therapy is usually recommended after surgery to kill any remaining tumor cells that are left behind after surgery. Progression-free survival rate. MECA is a rare type of tumor occurring in the parotid and submandibular glands. Lastly, the influence of clinicopathological features on cancer-specific survival (CSS)was analyzed, and only the CSS data of 286 patients were included as those of four cases were not available. Savera A.T., Sloman A., Huvos A.G., Klimstra D.S. ScienceDirect is a registered trademark of Elsevier B.V. ScienceDirect is a registered trademark of Elsevier B.V. Myoepithelial carcinoma of the head and neck: A report of 23 cases and literature review, https://doi.org/10.1016/j.ctrc.2014.08.002. These findings remained significant on multivariate logistic regression analysis, with overall AJCC stage, T, N, and M stage, age>80, black race, and nonsurgical treatment significantly affecting survival. Survival statistics use information collected from large groups of people who may have received different types of treatment. Otolaryngol Head Neck Surg. (Predisposing Factors) Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. GUID:E3F36507-42D6-47EB-BDEC-645B06695E36, Myoepithelial carcinoma, Salivary gland, Surveillance epidemiology and end results, Population, Radiotherapy, Prognosis. Next,103 matched pairs were next investigated after PSM, (Supplementary Table1). We retrospectively analyzed 23 patients diagnosed with MC of the head and neck between 1991 and 2005. The baseline characteristics were similar between the S and S+R groups, except for chemotherapy (P<0.001). Primary tumor site and patient sex were not significantly associated with survival on multivariate analysis. Miyata M., Hasegawa K., Ishikawa K., Kato R., Udagawa Y., Kuroda M. Primary myoepithelial carcinoma of the vulva and review of the literature. Also, the National Cancer Institute has resources to help you understand cancer prognosis. Further studies are needed before the treatment can be used on patients. 9, Changbin West Fourth Street, Xiuying District, Haikou, Hainan 570100, China. In the present study univariate analysis showed that T stage, N stage/presence of nodal metastases, M stage/presence of distant metastases, surgical treatment vs. nonsurgical treatment, overall AJCC stage, race, and age at diagnosis all significantly affected survival. One significant factor may be Hurricane Katrina. At one end of the morphologic spectrum is the rare lesion also known as parachordoma, which is composed of large epithelioid cells with abundant eosinophilic to clear, vacuolated cytoplasm.17,18 The differential diagnosis for this group of lesions primarily centers on extraskeletal myxoid chondrosarcoma and the very rare peripheral chordoma (chordoma periphericum); however, chondroid lipoma, ossifying fibromyxoid tumor, and subcutaneous myxopapillary ependymoma may be considered as well. Table2 illustrates the Tumor (T), Nodal (N), and Metastasis (M) characteristics of the cohort. Patients included in the SEER database had previously consented to participate in any scientific research worldwide. The median progression-free survival for first-line chemotherapy was 9.3 months. Most patients were treated with surgery and postoperative radiotherapy or adjuvant chemotherapy. A patient with stage I cancer who received chemotherapy had an undifferentiated tumor. Figure5 illustrates the Kaplan-Meier actuarial overall survival by primary site. [2] noted that 57.3% of patients were female. Most of the cases are observed in adults A very high preference for males is noted, though both males and females are affected There is no racial or ethnic group predilection observed What are the Risk Factors for Myoepithelial Carcinoma of Lung? The three staging systems were merged, and the outcomes were recorded as T, N, and M stages for analysis. The database was queried for malignant neoplasm, epithelial-myoepithelial carcinoma, any site, any age, and was extracted using SEER*Stat version 8.3.4 (National Cancer Institute, Bethesda, Maryland) and exported into Microsoft Excel 2016 (Microsoft Corporation, Redmond, Washington) for analysis. PubMed Myoepithelial carcinomas account for less than 1% of malignant epithelial salivary neoplasms. Twenty-five percent to 30% of patients succumb to their disease, and histopathologic features do not reliably identify these lethal tumors. Sklov A., Weinreb I., Hyrcza M., Simpson R.H.W., Laco J., Agaimy A., Vazmitel M., Majewska H., Vanecek T., Talarik P., Manajlovic S., Losito S.N., teiner P., Klimkova A., Michal M. Clear cell myoepithelial carcinoma of salivary glands showing EWSR1 rearrangement: molecular analysis of 94 salivary gland carcinomas with prominent clear cell component. Published by Elsevier Ltd.