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OBJECTIVES: In this systematic review, we aimed to evaluate the clinicopathological and prognosis data of patients with salivary gland myoepithelial carcinoma. MATERIALS AND METHODS: MEDLINE/PubMed, Scopus, and Embase search was performed with the keywords "myoepithelial carcinoma" "malignant myoepithelioma," and "salivary glands." Primary salivary glands myoepithelial carcinoma that fulfilled the World Health Organization diagnostic criteria were included. The Joanna Briggs Institute tool was used to assess the risk of bias. RESULTS: Forty-three studies (71 patients) met the inclusion criteria. The patients showed a mean age of 56.4 ± 19.6 years with no sex predilection. The parotid was the most affected gland (49.3%). The tumor presented as an asymptomatic (65.1%) mass (84%). The most common histological findings were the presence of clear tumor cells (39.7%) and multinodular growth patterns (60.7%). Multivariate analysis showed plasmacytoid cell type (p = 0.010) and solid growth pattern (p = 0.003) were related to decreased disease-free survival. Surgery alone was the most used treatment (53.5%). Patients with a combination of treatments showed a longer disease-free survival (p = 0.049). The 2-year and 5-year overall survival rates were 67.5% and 46.1%, respectively. CONCLUSION: Salivary gland myoepithelial carcinoma showed no sex predilection, with a higher incidence in the parotid gland. Cell type, growth pattern, and treatment type may be related to a lower disease-free survival. Overall, salivary gland myoepithelial carcinoma presented low recurrence and metastasis rates. Registration and protocol: This systematic review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2020 checklist and registered in the International Prospective Register of Systematic Reviews (PROSPERO) database (CRD42022311512).

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RESUMEN: El carcinoma mioepitelial es una rara neoplasia maligna de las glándulas salivales; puede originarse de novo o desarrollarse en un adenoma pleomórfico preexistente o un mioepitelioma. Las características histológicas e inmunohistoquímicas de un caso de carcinoma mioepitelial de glándula parótida originado por la transformación maligna de un adenoma pleomórfico primario previo se investigaron en este trabajo. Se demostró la presencia de mitosis, pleomorfismo nuclear, áreas necróticas, mioepiteliocitos con una marcación con p63 superior al 75 % y un índice proliferativo de 15 %, características que pueden desencadenar un desenlace adverso. Resulta clave el examen periódico de la paciente que dará una probabilidad superior de curación y sobrevida y de investigar eventuales recurrencias o metástasis.

ABSTRACT: Myoepithelial carcinoma is a rare malignant neoplasm of the salivary glands; it may originate de novo or develop into a pre-existing pleomorphic adenoma or myoepithelioma. The histological and immunohistochemical characteristics of a case of myoepithelial carcinoma of the parotid gland originated by the malignant transformation of a previous primary pleomorphic adenoma were investigated in this work. The presence of mitosis, nuclear pleomorphism, necrotic areas, myoepitheliocytes with a p63 labeling greater than 75 % and a proliferative index of 15 % were demonstrated, characteristics that can trigger an adverse outcome. The periodic examination of the patient is essential, which will give a higher probability of cure and survival and to investigate eventual recurrences or metastases.

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Myoepithelial carcinoma, also known as malignant myoepithelioma, is considered an extremely rare (0.45-1%) malignant salivary gland neoplasm. Approximately 100 cases have been reported in the English-language literature on myoepithelial carcinoma. The majority of the myoepitheliomas described in the literature have been benign, and the malignant counterpart is considered rare (<1%). Such a tumor may appear de novo or rarely develop from a preexisting pleomorphic adenoma (<20%), and in exceedingly rare cases (<0.5%), it has arisen from a benign myoepithelioma (i.e., plasmacytoid myoepithelioma). To our knowledge, no case of myoepithelial carcinoma of the parotid gland arising in a plasmacytoid myoepithelioma synchronized with melanoma has been reported to date. The treatment of myoepithelial carcinoma has been mainly surgical, including wide excision with free margins, with or without nodal dissection. The roles of chemotherapy and radiotherapy have not yet been established. We report a case of myoepithelial carcinoma of the parotid gland arising in a plasmacytoid myoepithelioma synchronized with melanoma in a 40-year-old woman. In our case, a complete response was achieved with surgery followed by adjuvant chemotherapy based on carboplatin and paclitaxel concurrent with radiotherapy.

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Myoepithelial tumors of soft tissue are rare mesenchymal neoplasms that overlap with their salivary gland and skin counterparts at both the histopathologic and molecular levels. EWSR1 gene rearrangements with various fusion partners represent a common genetic event in myoepithelial tumors of soft tissue, whether benign or malignant, and may prove useful as a diagnostic tool in difficult cases. However, the number of diagnostic entities with EWSR1 gene rearrangements has grown considerably in recent years, and there is significant morphologic and immunophenotypic overlap amongst this group, underscoring the importance of fusion testing to detect fusion partners that are characteristic of discrete diagnostic entities. Herein, we report a malignant myoepithelial tumor of soft tissue/myoepithelial carcinoma with an undifferentiated round cell morphology arising in a pediatric patient with a EWSR1-ATF1 gene fusion.

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As neoplasias de glândulas salivares são um grupo heterogêneo de lesões que correspondem aproximadamente a 3-6% dos casos de neoplasias de cabeça e pescoço e apresentam características histológicas distintas. A grande variação no padrão histológico das lesões de glândulas salivares tem sido atribuída à presença de células mioepiteliais, que apresentam padrões distintos em cada neoplasia. O objetivo deste trabalho é avaliar a expressão de um painel de proteínas do citoesqueleto, adesão e proliferação celular, sendo elas: actina de músculo liso (AML), calponina, caldesmon, citoceratina 14 (CK14), E-caderina, vimentina, beta-catenina, Ki-67 e p63 em neoplasias malignas das glândulas salivares utilizando a técnica de imunoistoquímica (IHQ). Foram selecionadas retrospectivamente, um total de 15 amostras, sendo 08 amostras de carcinoma ex-adenoma pleomórfico, 04 de carcinoma mioepitelial, 03 de adenocarcinoma de células basais. Os casos foram analisados e os resultados, obtidos através da imumoistoquímica, comparados com os dados demográficos, clínicos e patológicos. A expressão das proteínas foi analisada qualitativamente e semi-quantitativamente, sendo classificada em negativo, positivo focal, positivo difuso ou positivo abundante. A actina de músculo liso (AML) foi observada em dois casos de adenocarcinoma de células basais (ACCB), três de carcinoma mioepitelial (CAME) e três de carcinoma ex-adenoma pleomórfico (CXAP). A calponina foi expressa em dois casos, tanto de ACCB quanto de CAME e em três casos de CXAP. A proteína caldesmon foi observada em dois casos de ACCB, os quatros casos de CAME e sete casos de CXAP. Três casos de ACCB e de CAME e cinco de CXAP apresentaram expressão de CK14. A E-caderina foi observada em todos os casos dos três tipos tumorais, assim como a beta-catenina. A proteína vimentina foi expressa em todos os casos de ACCB e CAME, e predominantemente nos casos de CXAP. Poucos casos, tanto de ACCB, CAME e CXAP apresentaram positividade para Ki-67. A proteína p63 foi observada em todos os casos de ACCB e CAME, sendo pouco expressa em CXAP. A análise de clusterização hierárquica demonstrou a formação de dois clusters, sendo um deles predominantemente composto por CXAP. A comparação da expressão das proteínas com as características demográficas, clínicas e patológicas demonstrou associação entre a perda de expressão das proteínas CK14 e p63 e a ocorrência de metástase. Os resultados sugerem que a expressão das proteínas beta-catenina, E-caderina, caldesmon e vimentina apresentou-se de forma muito similar, sugerindo um perfil equivalente de expressão entre essas neoplasias derivadas do ducto intercalar. Já a ausência de expressão de AML e calponina parece estar associada à separação dos grupos na análise de clusterização, sugerindo que deve ser considerada como um fator na diferenciação tumoral.

Salivary gland neoplasms are a heterogeneous group of lesions that account for approximately 3-6% of head and neck tumors, with distinct histological characteristics. The variation in the histological pattern of salivary gland lesions has been attributed to the presence of myoepithelial cells, that present different patterns in each neoplasm. The aim of the study was to evaluate the expression of a panel of cytoskeletal, cell adhesion and cell proliferation proteins, namely: smooth muscle actin (SMA), calponin, caldesmon, cytokeratin 14 (CK14), E-cadherin, vimentin, beta- catenin, Ki-67 and p63 in salivary glands malignant neoplasms, using immunohistochemical technique (IHC). Fifteen samples werer etrospectively selected, being 08 carcinoma ex-pleomorphic adenoma samples, 04 myoepithelial carcinoma samples, 03 basal cell adenocarcinoma samples. The immunohistochemical results were analyzed and compared with demographic, clinical and pathological data. Protein expression were qualitatively and semi-quantitatively analyzed and classified as negative and positive (focal, diffuse, or abundant). Smooth muscle actin (SMA) was observed in two cases of basal cell adenocarcinoma (BCAC), three cases of myoepithelial carcinoma (MECA) and three cases of carcinoma ex-pleomorphic adenocarcinoma (CXPA). Calponin was expressed in two cases of BCAC, two cases of MECA, and three cases of CXPA. Caldesmon protein was observed in two cases of BCAC, four cases of MECA and seven cases of CXPA. Three cases of BCAC and MECA and five of CXPA presented CK14 expression. E-cadherin was observed in all cases of the three tumor types, as well as beta-catenin. Vimentin protein was expressed in all BCAC and MECA cases, and predominantly in CXPA cases. Few cases of BCAC, MECA and CXPA were positive for Ki-67. p63 protein was observed in all cases of BCAC and MECA, and present low expression in CXPA. The hierarchical clustering analysis demonstrated the formation of two clusters, one of them being predominantly composed of CXPA. Protein expression comparison with demographic, clinical and pathological characteristics demonstrated an association between loss of expression of CK14 and p63 proteins and the occurrence of metastasis. The results suggest that the expression of beta-catenin, E-cadherin, caldesmon and vimentin proteins was very similar, suggesting an equivalent expression profile among these neoplasms derived from the intercalated duct of the salivary gland. The absence of SMA and calponin expression seems to be associated with the separation of groups in the clustering analysis, suggesting that it should be considered as a factor in tumor differentiation

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Chromosomal instability, leading to aneuploidy, is one of the hallmarks of human cancers. USP44 (ubiquitin specific peptidase 44) is an important molecule that plays a regulatory role in the mitotic checkpoint and USP44 loss causes chromosome mis-segregation, aneuploidy and tumorigenesis in vivo. In this study, it was investigated the immunoexpression of USP44 in 28 malignant salivary gland neoplasms and associated the results with DNA ploidy status assessed by image cytometry. USP44 protein was widely expressed in most of the tumor samples and no clear association could be established between its expression and DNA ploidy status or tumor size. On this basis, it may be concluded that the aneuploidy of the salivary gland cancers included in this study was not driven by loss of USP44 protein expression.

Resumo Instabilidade cromossômica acarretando aneuploidia é um dos fatores marcantes de neoplasias malignas humanas. USP44 (peptidase específica de ubiquitina 44) é uma importante molécula que exerce um papel regulador no ciclo celular e sua perda pode acarretar em segregação cromossômica deficiente, aneuploidia e desenvolvimento de tumores in vivo. Neste estudo, investigou-se a expressão imuno-histoquímica da proteína USP44 em 28 neoplasias malignas de glândulas salivares, associando-se os resultados com o estado de ploidia do DNA avaliado por citometria de fluxo. A proteína USP44 apresentou ampla expressão na maioria das amostras avaliadas e não foi observada associação entre a expressão protéica e o estado de ploidia do DNA ou extensão do tumor. Baseando-se nos resultados, concluiu-se que a aneuploidia das neoplasias malignas de glândulas de salivares incluídas neste estudo não foi influenciada pela perda de expressão da proteína USP44.

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Salivary gland myoepithelial carcinoma (MC) or malignant myoepithelioma is a rare entity. MC usually presents as a slow-growing painless mass arising in the parotid gland, but may involve other salivary glands. This tumour may be particularly locally aggressive, but its clinical and biological features are not yet fully understood. MC may arise from pre-existing benign lesions, such as pleomorphic adenomas or benign myoepitheliomas, or may arise de novo. It usually affects patients over 50 years old, with no gender preference. Because it is often asymptomatic, the presentation and diagnosis can be delayed by months, even years. The current WHO classification considers MC to be an intermediate- to high-grade malignancy. Other published data suggest it is likely to be a high-grade neoplasm, consistent with its aggressive behaviour. Its epidemiology, histopathological features, immunohistochemical profile, clinical behaviour and optimal management are not well understood. Following review of the current literature we aim to address these.

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El carcinoma epitelial-mioepitelial (CEM) de glándulas salivales es un tumor maligno bifásico de bajo grado, que comprende aproximadamente el 1 por ciento de todos los tumores salivales. Se describe un raro caso de CEM sebáceo de glándula parótida en un hombre al que se le realizó parotidectomía izquierda por formación tumoral aparentemente encapsulada. Para su estudio histopatológico e inmunohistoquímico el material se coloreó con H/E, tricrómicos de Massony Dane/Azul alciano, PAS/H, Azul de toluidina, antígeno epitelial de membrana (EMA), citoqueratinas (CK) de bajo peso molecular, a actina de músculo liso (a-SMA) y S-100. Los cortes histológicos mostraron un patrón multinodular con nidos tumorales constituidos por estructuras tubulares delimitadas por: a-células acidófilas luminales cuboideas; b- células claras adluminales. Las células ductales luminales se marcaron positivamente con los anticuerpos para EMA y CK. Se observó inmunorreactividad positiva para a-SMA y S-100 en las células claras. Se observaron áreas de diferenciación sebácea dentro de la masa tumoral. Los estudios inmunohistoquímicos confirman la presencia de células tumorales ductales y mioepiteliales. Además, las células claras mioepiteliales constituyen un elemento importante para el diagnóstico diferencial entre CEM sebáceo y adenocarcinoma sebáceo.

Epithelial myoepithelial carcinoma (EMC) of the salivary glands is an uncommon, low grade, biphasic neoplasm. We reported a rare case of sebaceous EMC of the parotid gland in a man who had a left parotidectomy because of a tumoral formation apparently encapsulated. For the histopathological and immunohistochemical study the material was stained with H/E, Masson and Dane/Alcian blue trichrome, PAS/H, Toluidine blue, epithelial membrane antigen (EMA), cytokeratins (CK) of low molecular weight, a-smooth muscle actin (a-SMA) and S-100. Histologically, the tumor showed a multinodal pattern with tumoral nests constituted by ducts with a double cell lining: a- luminal cuboidal eosinophilic cells; badluminalclear cells. The inner cells were positively marked with antibodies for EMA and CK. It was observed positiveimmunoreactivity for a-SMA and S-100 in clear cells. It was observed areas of sebaceous differentiation inside the tumoral mass. Immunohistochemical studies confirmed ductal tumoral and myoepithelial cells presence. Besides, myoepithelial clear cells aided differential diagnosis between sebaceous EMC and sebaceous adenocarcinoma.

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