Single-cell transcriptomic analysis uncovers the origin and intratumoral heterogeneity of parotid pleomorphic adenoma / 国际口腔科学杂志·英文版

Pleomorphic adenoma (PA) is the most common benign tumour in the salivary gland and has high morphological complexity. However, the origin and intratumoral heterogeneity of PA are largely unknown. Here, we constructed a comprehensive atlas of PA at single-cell resolution and showed that PA exhibited five tumour subpopulations, three recapitulating the epithelial states of the normal parotid gland, and two PA-specific epithelial cell (PASE) populations unique to tumours. Then, six subgroups of PASE cells were identified, which varied in epithelium, bone, immune, metabolism, stemness and cell cycle signatures. Moreover, we revealed that CD36+ myoepithelial cells were the tumour-initiating cells (TICs) in PA, and were dominated by the PI3K-AKT pathway. Targeting the PI3K-AKT pathway significantly inhibited CD36+ myoepithelial cell-derived tumour spheres and the growth of PA organoids. Our results provide new insights into the diversity and origin of PA, offering an important clinical implication for targeting the PI3K-AKT signalling pathway in PA treatment.

Giant myoepithelial carcinoma of the nuchal region: a case report and literature review / 临床耳鼻咽喉头颈外科杂志

Myoepithelioma, also known as malignant myoepithelioma, is a rare malignant tumor originating from myoepithelial cell. This article reports a patient with a huge tumor in the neck and left elbow who underwent fine needle aspiration under local anesthesia. The pathological diagnosis was a myoepithelioma. Under general anesthesia, giant tumors in the lower neck, posterior cranial fossa, neck, and left elbow were removed, and postoperative pathology showed that they were all myoepithelial tumors. Immunohistochemistry showed AE1/AE3 (+), P63 (+), CK7 (+), CK5 (+), and CD138 (+). The clinical characteristics and diagnosis and treatment process of this case are reported and relevant literature is reviewed.

Diagnóstico diferencial e tratamento do mioepitelioma e adenoma pleomórfico / Differential diagnosis and treatment of mioepithelioma and pleomorphic adenoma

Introdução: As neoplasias de glândulas salivares constituem um grupo de lesões, clínica e morfologicamente diferente, capaz de determinar importantes desafios diagnósticos e terapêuticos. Apresentar dois casos de tumores benignos de glândulas salivares menores, sendo um adenoma e outro mioepitelioma, discutindo o diagnóstico diferencial e a forma de tratamento em comparação com informações da literatura. Relato de caso: Pacientes do sexo feminino, com idades semelhantes, ambas se queixavam que após o uso de uma prótese mal adaptada notou-se o aparecimento de uma lesão assintomática no palato. Diante das características clínicas das lesões, as hipóteses de diagnóstico foram de tumor de glândula salivar menor e adenoma pleomórfico, respectivamente. Diante da ausência de sinais de malignidade, o tratamento proposto foi biópsia excisional com enucleação e curetagem. O diagnóstico histopatológico foi de Tumor de Glândula Salivar Menor (Mioepitelioma Plasmocitóide), e Adenoma Pleomórfico, respectivamente. A enucleação e curetagem se mostraram eficazes e sem sinais de recidiva. Considerações finais: Tanto o adenoma como o mioepitelioma se apresentam clinicamente semelhantes sendo o diagnóstico diferencial realizado através do histopatológico, porém o tratamento conservador de enucleação e curetagem pode ser aplicado em ambas... (AU)

Introduction: The neoplasms of salivary gland constitute a group of lesions clinically and morphologically different, which are able to determine important challenges in diagnostic and therapeutic.To report two cases of benign tumors of the minor salivary glands, adenoma and myoepithelioma. Also, discussing the differential diagnosis and its treatment in comparison with literature information. Case report: Two female patients, with similar ages, both complained about the appearance of an asymptomatic lesion on the palate after using a poorly adapted prosthesis. On the clinical characteristics of the lesions, the hypothetical diagnosis was of minor salivary gland tumor and pleomorphic adenoma, respectively. In the absence of signs of malignancy, the proposed treatment was excisional biopsy with enucleation and curettage. The histopathology diagnosis was begin tumor of minor salivary glands (myoepithelioma plasmacytoid), and pleomorphic adenoma, respectively. The enucleation and curettage were effective and there were no signs of recurrence. Final considerations: Both the adenoma and myoepithelioma are clinically similar and the differential diagnosis is performed by the histopathology exam, but conservative treatment such as enucleation and curettage can be applied to both... (AU)

Spindle cell myoepithelioma of the parotid gland

Myoepithelioma was recognized as a histological distinct entity by the World Health Organization (WHO) in 1991. Myoepithelial cells are believed to be of ectodermal origin. In salivary glands, the myoepithelial cells that surround the intercalated ducts are spindled, which is in contrast to the large stellate ones that envelop the acini. Myoepithelioma is a benign salivary gland tumor that consists entirely of myoepithelial cells. A 53-year-old man presented with a 1-year history of a painless mass originating from the right parotid gland. The mass grew rapidly reaching a size of approximately 6 cm. The patient had no facial paralysis. The authors performed right parotidectomy. Immunohistochemistry study of this tumor showed that it was positive for vimentin, positive for S-100, focally positive for pancytokeratin, and focally positive for p63 and that it had a Ki-67 labeling index (below 10%). Additionally, the tumor was negative for epithelial membrane antigen, negative for actin, negative for desmin, negative for CD34 and negative for anaplastic lymphoma kinase. The authors present a case of benign spindle cell myoepithelioma of the parotid gland in a 53-year-old man diagnosed after immunohistochemistry study, describing its importance, along with a brief review of the literature.

Ectomesenchymal chondromyxoid tumor: a comprehensive updated review of the literature and case report / 国际口腔科学杂志·英文版

Prompted by a unique case of an ectomesenchymal chondromyxoid tumor (ECT) of the palate in a 54-year-old female, we reviewed the English and German literature on this entity until the end of 2016 using PubMed. The search produced 74 lingual cases with a nearly equal sex distribution and a mean age of 39.3 years, and two extra-lingual cases sharing histological and immunohistological features including nodular growth, round, fusiform or spindle-shaped cellular architecture, and chondromyxoid stroma. Immunophenotyping showed the majority of cases to be positive for glial fibrillary acidic protein (GFAP), S-100 protein, glycoprotein CD57, pancytokeratin (AE1/AE3), and smooth muscle actin (SMA); in isolated cases there was molecular-genetic rearrangement or gain of Ewing sarcoma breakpoint region 1 (EWSR1) but no rearrangement of pleomorphic adenoma gene 1 (PLAG1). At present, ectomesenchymal cells that migrate from the neural crest are considered to play a pivotal role in tumor origin. All cases had a benign course, although there were three recurrences. Because of the rarity of this tumor and the need for differential diagnostic differentiation from myoepithelioma and pleomorphic adenoma, both oral surgeons and pathologists should be aware of this entity.

Assinatura imunoistoquíca das células mioepiteliais em neoplasias benignas de glândulas salivares / Immunohistochemical signature of myoepithelial cells in benign neoplasms of salivary glands

Os tumores de glândulas salivares compreendem um grupo heterogêneo de lesões, apresentando diferentes características histológicas e comportamento clínico diverso. A grande variação no padrão histológico das lesões benignas de glândulas salivares muitas vezes é atribuída à presença de células mioepiteliais que apresentam padrões distintos em cada neoplasia: o mioepitelioma (M) é composto predominantemente por células mioepiteliais, o adenoma de células basais (ACB) é composto predominantemente por células epiteliais e o adenoma pleomórfico (AP) composto por variações de células mioepiteliais e epiteliais. O objetivo desse trabalho foi avaliar a expressão de um painel de proteínas do citoesqueleto (citoceratina 14 (CK14), vimentina, actina de músculo liso (AML), calponina e caldesmon), da matriz extracelular (colágeno) e proteína PLAG1, em neoplasias benignas das glândulas salivares por meio de técnica imunoistoquímica. Para tanto foram selecionadas 28 amostras de AP, 3 de M, 5 de ACB e 3 amostras de glândulas salivares histologicamente normais. A proteína CK14 foi predominantemente expressa no adenoma pleomórfico e adenoma de células basais. A AML foi expressa em todos os casos de adenoma pleomórfico e sua expressão foi menos frequente no adenoma de células basais e mioepitelioma. Caldesmon e vimentina foram expressas em todos os casos de AP, M e ACB. A proteína calponina foi expressa em todos os casos de mioepitelioma e adenoma de células basais e frequentemente positiva no adenoma pleomórfico. Colágeno tipo IV foi raramente expresso nos casos de adenoma pleomórfico e no adenoma de células basais. PLAG1 foi expressa na maioria dos tumores. Utilizando análise de clusterização hierárquica não foi possível classificar os diferentes tumores benignos de glândula salivar. A análise estatística demonstrou que a AML é mais expressa em adenoma pleomórfico e que existe uma associação entre a expressão das proteínas calponina e PLAG1. Nossos resultados sugerem que o mioepitelioma, adenoma pleomórfico e adenoma de células basais representam espectros histológicos de um mesmo tumor

Salivary gland tumors comprise a heterogeneous group of lesions, presenting different histological characteristics and diverse clinical behavior. The large variation in the histological pattern of benign lesions of the salivary glands is often attributed to the presence of myoepithelial cells presenting distinct patterns in each neoplasia: myoepithelioma is predominantly composed of myoepithelial cells, basal cell adenoma is composed predominantly of epithelial cells and pleomorphic adenoma composed of variations of myoepithelial and epithelial cells. The aim of this work was to determine a myoepithelial cell signature in benign salivary gland neoplasms using a panel of cytoskeleton proteins (cytokeratin, vimentin, smooth muscle actin, calponin and caldesmon), extracellular matrix protein (collagen) and PLAG1 protein by means of immunohistochemical technique. For this purpose 28 samples of pleomorphic adenoma, 3 of myoepithelioma, 5 of basal cell adenoma and 3 samples of histologically normal salivary glands were selected. CK14 protein was predominantly expressed in pleomorphic adenoma and basal cell adenoma. Smooth muscle actin was expressed in all cases of pleomorphic adenoma and its expression was less frequent in basal cell adenoma and myoepithelioma. Caldesmon and vimentin were expressed in all cases of pelomorphic adenoma, myoepithelioma and basal cell adenoma. Calponin protein was expressed in all cases of myoepithelioma and basal cell adenoma and frequently positive in pleomorphic adenoma. Type IV collagen was rarely expressed in cases of pleomorphic adenoma and basal cell adenoma. PLAG1 was expressed in the majority of the tumors. Using hierarchical clustering analysis it was not possible to classify the different benign tumors of the salivary gland. Statistical analysis demonstrated that smooth muscle actin is more expressed in pleomorphic adenoma and that there is an association between the expression of the calponin and PLAG1 proteins. Our results suggest that myoepithelioma, pleomorphic adenoma, and basal cell adenoma represent histological spectra of the same tumor

Carcinoma parotídeo epitelial-mioepitelial: presentación de un caso y revisión de la literatura / Parotid epithelial-myoepithelial carcinoma: case presentation and literature review

Se presenta caso de paciente de sexo femenino de 53 años de edad con tumor parotídeo izquierdo de larvada evolución, con crecimiento progresivo y otalgia ipsilateral en los últimos meses, estudiado previamente con tomografía de cuello con contraste y resonancia magnética que destacan masa del lóbulo profundo de la parótida de características imagenológicas benignas. Se realizó parotidectomía del lóbulo profundo con resección tumoral preservando el nervio facial casi en su totalidad con excepción de rama marginal, la biopsia de la pieza quirúrgica fue informada como carcinoma epitelialmioepitelial de bajo grado, un tumor infrecuente de las glándulas salivales. Se decidió completar la parotidectomía superficial y realizar vaciamiento ganglionar selectivo lateral ipsilateral, complementando el tratamiento con radioterapia. Además se presenta una revisión de la literatura correspondiente.

We present a case of a 53 years old female patient with a left parotid tumor, with slow evolution, progressive growth and ipsilateral otalgia during later months. She was previously studied by tomography of the neck with contrast and magnetic resonance, which showed the mass of the deep lobe to have benign imaging characteristics. A parotidectomy of deep lobe was performed, with tumoral resection, preserving the facial nerve with the exception of the marginal branch. The biopsy was informed as epithelial-myoephitelial carcinoma, a rare salivary gland tumor. We completed the parotidectomy with neck dissection and Radiotherapy complementary was made. Besides we presented a literature review.

Myoepithelioma in accessory parotid gland: report of a rare case / Mioepitelioma em glândula parótida acessória: relato de um caso raro

Mioepitelioma é um tumor benigno de glândula salivar que acomete, principalmente, a glândula parótida. Apresença de uma glândula parótida acessória é uma variação anatômica e neoplasias nessa estrutura são extremamente raras. Este artigo descreve um caso de mioepitelioma ocorrendo em uma glândula parótidaacessória de uma mulher de 29 anos de idade. No entendimento dos autores este é o sétimo caso relatado em Inglês. A queixa da paciente era umamassa na bochecha, com cinco anos de evolução, apresentando súbito surto de crescimento. A paciente foi submetida à cirurgia por meio de uma abordagemintra-bucal e a histopatologia e a imunohistoquímica revelaram um mioepitelioma.

Myoepithelioma is a benign tumor of the salivary gland that mainly affects the parotid gland. The presence of an accessory parotid gland is an anatomical variation and neoplasms in this structure are extremely rare. This paper describes a case of a myoepithelioma arising in the accessory parotid gland of a 29-year-old woman. To the author´s knowledge this is the seventh case reported in English. The patient´s complaint was about a five year growing mass in the left cheek with a sudden growth outbreak. The patient underwent surgery via an intra-oral approach and the histopathology and the immunohistochemistry disclosed a myoepithelioma.

One nursing case of nasal cavity paranasal sinus and maxillofacial huge myoepithelial carcinoma patient / 临床耳鼻咽喉头颈外科杂志

A patient diagnosed with myoepithelial carcinoma was recently treated in our department. The neoplasm was huge, located in the left maxillofacial region, blocking both eyes, impeding feeding. About one month before admission, the tumor began to bleed frequently, about 100 ml each time, causing headache, dizziness, fatigue, and cold sweats. CT showed the maximum diameter of the tumor was about 23 cm, with uneven density, and maxillofacial bone destruction. MRI revealed internal bleeding and necrosis inside the tumor. After admission, blood routine test showed erythrocyte count 3.64 x 10(12)/L(↓), hemoglobin 106 g/L(↓), hematocrit 0.320 (↓), serum iron 6.2 μmol/L(↓). After surgery, the patient recovered smoothly.

Analysis of the diagnosis and treatment of myoepithelial carcinoma of the parotid gland: report of 17 cases / 中华肿瘤杂志

<p><b>OBJECTIVE</b>Myoepithelial carcinoma (MC) is a rare malignant neoplasm of the salivary gland. The aim of this study was to analyze the diagnosis, treatment and prognosis of MC of the parotid gland.</p><p><b>METHODS</b>The clinicopathological data of 17 cases of MC of the parotid gland treated in our hospital from 1999 to 2013 were analyzed retrospectively. Of all the 17 patients, 9 cases received radical surgery only, 5 cases received postoperative radiotherapy, 2 cases received preoperative radiotherapy, and one case received chemotherapy. The survival rate was calculated by Kaplan-Meier analysis.</p><p><b>RESULTS</b>Among the 17 patients, 11 patients had post-operative recurrence (11/17, 64.7%), Of these 11 cases, 5 cases (45.5%) had recurrence within one year after the first operation. During the follow-up for 12-180 months (median 50 months), six cases died (two patients died of distant metastases and 4 cases died of local recurrence). The overall 1-year, 2-year and 5-year survival rates were 94.1%, 74.2% and 64.9%, and the overall 1-year, 2-year and 5-year recurrence-free survival rates were 70.6%, 48.1% and 40.1%, respectively.</p><p><b>CONCLUSIONS</b>Radical surgery is the main treatment modality for myoepithelial carcinoma of the parotid gland. For the patients with extensive lesions or after palliative surgery, adjuvant radiotherapy or chemotherapy might be helpful. However, its therapeutic efficacy remains to be proved.</p>

Myoepithelioma of nasal cavity: a case report / 临床耳鼻咽喉头颈外科杂志

Myoepithelioma is an uncommon benign tumor that most likely occurs in the salivary gland. Extra-salivary myoepithelioma are sporadic and rare, especially the quantities occur in the sinonasal cavity are fewer in related literature reported. In this paper, we present a rare case of benign myoepithelioma that occurred in the nasal cavity, and the related process of pathogenesis, diagnosis, treatment and prognosis are discussed.

Tumor mioepitelial mixto (paracordoma) / Myoepithelial mixed tumor (parachordoma)

Estudio de caso: Paciente de sexo masculino, de 38 años de edad,con tumor ación indolor a en muslo izquierdo de 3 años de evolución. Esta neoplasia corresponde típicamente a nódulos compuestos por células epiteloides poligonales, claras, que recuerdan a las de la notocorda y una segunda población de células e osinofílicas organizadas en cordones y nidos separados por estroma mixoide, con fenómenos de vacuolización citoplasmática similar a las del cordoma (células tipo fisalíferas).

Case Study: Male patient, 38 years old, with painless swelling in left thigh 3 years evolution. This neoplasm typicall y corresponds tonodules composed of polygonal epithelioid cells, clear, reminiscent of the notochord and a second population of eosinophilic cells arranged incords and nests separated by myxoid stroma with phenomena similar to cytoplasmic vacuolization the chordoma (physaliphorous type cells).

Soft Tissue Malignant Myoepithelioma in the Extremities / 대한골관절종양학회지

PURPOSE: We report the diagnosis, treatment outcomes and prognosis of the patients with soft tissue malignant myoepithelioma in the extremities. MATERIALS AND METHODS: We retrospectively reviewed 6 patients with soft tissue malignant myoepithelioma in the extremities who were treated at our institution between 2008 and 2014. Two patients received unplanned excision at another hospital and remaining 4 patients underwent the biopsy procedures and received wide excision at our hospital. RESULTS: There were 3 men and 3 women with mean age of 41 (33-54) years. The average follow up was 28 (9-45) months. Among the 6 patients, only 4 patients underwent biopsy procedures under the impression of malignant soft tissue sarcoma. Surgical margins for these 4 patients were negative. Two patients who had unplanned excision received another re-excision and one of them showed no residual tumor in the resected specimen. Local recurrences were developed in all patients and distant metastasis in 4 patients. All 4 patients who developed distant metastasis died due to disease progression. Among the 2 patients who developed local recurrence only, one patient has another local recurrence after re-operation and remaining one patient is no evidence of disease for 2 years after resection of locally recurred mass. CONCLUSION: Soft tissue malignant myoepithelioma in the extremities is a rare disease and shows an aggressive behavior. Appropriate biopsy under the impression of soft tissue malignancy is necessary and complete surgical resection with wide margins is the recommended treatment of choice.

Recurrent Benign Myoepithelioma of the Auricle

Myoepithelioma, a generally benign tumor comprised of myoepithlial cells, is an uncommon salivary gland tumor. Myoepithelioma originates primarily in the parotid gland, but several isolated cases have been described. Although myoepithelioma has a benign nature, but there is a potential risk of malignant change and recurrence in case of incomplete resection. We acknowledge that benign myoepithelioma originating from the auricle has not been reported in the English literature. We present a rare case of 27-year-old female who had recurrent benign myoepithelioma originating from the auricle and already had been given twice operations at the other clinic.

Primary myoepithelial carcinoma of the preauricular area in a young female: a rare case report / 临床耳鼻咽喉头颈外科杂志

A female patient with a dark red mass in the of preauricular area for over 3 months was hospitalized. Enhanced CT scan and ultrasonography showed that the mass had clear boundaries and was rich in blood supply. The patient was misdiagnosed as preauricular hemangioma, then the mass was removed as a benign tumor and found irrelevant to parot, SMA(+), Vim(+), S-100(+), indicating myoepithelial carcinoma. So a radical excision was performed to get negative incised margin. The patient didn't undergo postoperative chemotherapy but demonstrated no evidence of recurrence over a 12-month follow-up.

Imprint Cytology of Soft Tissue Myoepithelioma: A Case Study

Soft tissue myoepithelioma is a rare neoplasm composed of myoepithelial cells. Here, we describe the cytologic features of soft tissue myoepithelioma arising on the right forearm in an 18-year-old man. The excised tumor (3.0x1.8x1.5 cm) was well-demarcated, yellow-gray, soft, and myxoid. The cytologic smears showed round to spindle, epithelioid, and plasmacytoid cells in the myxoid background. The nuclei were uniform, round to ovoid, with finely distributed chromatin and eosinophilic or pale cytoplasm. The tumor cells demonstrated immunoreactivity for cytokeratin (AE1/AE3), epithelial membrane antigen, S100 protein, and glial fibrillary acidic protein. Electron microscopy showed intermediate filaments, desmosomes, and basal lamina.

Mioepitelioma intraóseo: presentación de un caso y revisión de la literatura / Intraosseous myoepithelioma: report of a case and review of the literature

Durante su formación, remanentes de glándulas salivales pueden quedarse atrapados en diversas partes del cuerpo, incluyendo el maxilar y la mandíbula. Lo siguiente da lugar a proliferaciones neoplásicas de carácter benigno y maligno. El mioepitelioma es una rara neoplasia de glándulas salivales, cuya frecuencia es poco frecuente. Representa aproximadamente el 1.5 por ciento de todos los tumores de glándulas salivales, mayores y menores. Es aún más infrecuente su localización intraósea, siendo reportados en la literatura menos de veinte casos en esta localización. En este artículo se presenta un caso de mioepitelioma, variante epitelioide, de localización intraósea. El mismo fue tratado mediante maxilectomía de infraestructura con márgenes libres de lesión, obteniendo finalmente buenos resultados estéticos y funcionales.

Mioepitelioma de vagina: neoplasia poco frecuente en esta localización, reporte de un caso / Myoepithelioma located in the vagina of a pregnant woman

Antecedentes: Los mioepiteliomas, son tumores poco frecuentes originados a partir de la proliferación de células mioepiteliales. La mayor parte localizados en las extremidades inferiores y en glándulas salivales. Existen ocasionales informes previos de MEP que involucren la vagina. Objetivo: Describir un caso de mioepitelioma localizado en vagina. Caso clínico: Embarazada de 29 años edad, quien presenta una masa vaginal de un año de evolución, localizada en el himen. El examen histopatológico muestra una lesión compuesta por células fusiformes con positividad inmunohistoquímica para vimentina, citoqueratinas (CK) AE1/ AE3, S100, CD10, receptores de estrógenos y progesterona, Bcl2 y calponina y negatividad para desmina, actina muscular especifica, actina de músculo liso y p63. Con los anteriores hallazgos se interpreta la lesión como un mioepitelioma primario vaginal. Conclusión: Los mioepiteliomas son tumores que requieren para su diagnostico, análisis con técnicas de inmunohistoquímica o ultraestructurales que permitan diferenciarlos de otros tumores.

Background: The myoepitheliomas are rare tumors arising from the proliferation of myoepithelial cells. Most tumors are located in the lower extremity and salivary glands. There have been only occasional myoepithelial neoplasms previously reported involving the vagina. Objective: Describe a case of myoepithelioma located in the vagina of a pregnant woman. Case report: A 29 years old pregnant female, who about 1 year prior has a vaginal mass, located in the hymen. At histopathological examination shows a mass composed of spindle cells with positivity immunohistochemical studies for vimentin, cytokeratin AE1/AE3, S100, CD10 and calponin and negative for desmin, actinmuscle specific (HHF35), smooth muscle actin and p63. With these findings it was established the diagnosis of myoepithelioma in the vagina. Conclusion: Myoepitheliomas are tumors requiring for diagnostic of immunohistochemistry or ultrastructural techniques that allow its differentiation from other tumors.

Low-grade malignant myoepithelioma arising in a pleomorphic adenoma: a rare case

Malignant myoepithelioma is a very rare salivary gland tumor that can arise de novo or within a preexisting pleomorphic adenoma. We report a case of malignant myoepithelioma most probably arising in a pre-existing pleomorphic adenoma of the left parotid gland. The patient was a 60-year-old man who presented with a multinodular mass lesion over left side of the face and neck. He had undergone removal of a pleomorphic adenoma of the left parotid gland twice [8 and 22 years ago]. Histological examination showed locally concentrated highly invasive myoepithelial cells with bland-looking morphology and no evidence of mitosis or necrosis. Immunohistochemistry confirmed the myoepithelial differentiation [S-100+, SMA+] and a low Ki-67 labeling index [<5%]