Epithelial-Myoepithelial carcinoma of the lacrimal gland - Case report and review of literature

Objective@#To describe the clinicopathologic features and outcome of a patient with epithelial-myoepithelial carcinoma of the lacrimal gland who underwent modified lateral orbitotomy with en toto removal of the lesion and adjuvant radiotherapy.@*Methods@#This is a case report.@*Results@#A 31-year-old Filipino male seafarer presented with a 2-month history of an enlarging left superotemporal orbital mass and inferonasal displacement of the globe. Magnetic resonance imaging revealed a superotemporal extraconal mass within the lacrimal sac fossa with evidence of osseous infiltration of the superolateral orbital rim. Modified lateral orbitotomy was performed with en toto removal of the lesion and the clinically infiltrated adjacent lateral bony margin. Histopathologic diagnosis of epithelial-myoepithelial carcinoma of the lacrimal gland was made based on the classic and distinct biphasic morphology and was confirmed with immunohistochemistry studies (cytokeratin-7, S-100, and p63). Systemic surveillance using positron emission tomography and computed tomography scan with contrast revealed no evidence of regional or distant metastasis. Adjuvant radiotherapy of the orbital area was performed for increased local control. Twelve months postoperatively, the patient showed no evidence of tumor recurrence.@*Conclusion@#Epithelial-myoepithelial carcinoma of the lacrimal gland is a rare condition, and this is the first documented case from the Philippines. Accurate diagnosis is necessary for appropriate treatment. It should be included in the differential diagnosis of infiltrative lesions in the lacrimal gland fossa.

A Rare Case of Pulmonary Epithelial-myoepithelial Carcinoma: Case Report and Literature Review / 中国肺癌杂志

BACKGROUND@#Pulmonary epithelial-myoepithelial carcinoma is a very rare type of salivary gland lung tumor. No standard treatment plan yet. This article intends to analyze the clinical characteristics of pulmonary epithelial-myoepithelial carcinoma and discuss the diagnosis and treatment of pulmonary epithelial-myoepithelial carcinoma.@*METHODS@#The clinical data of a patient with pulmonary epithelial-myoepithelial carcinoma were analyzed and other relevant clinical literatures were reviewed.@*RESULTS@#Epithelial cells immunohistochemically expressed cytokeratin and myoepithelial cells immunohistochemically expressed SMA and S-100. The next-generation sequencing was mainly HRAS gene mutation and the express of PD-L1 protein was negative.@*CONCLUSIONS@#Most of the patients with Pulmonary epithelial-myoepithelial carcinoma have a good prognosis. Diagnosis mainly depends on microscopic examination and immunohistochemistry. The treatment of pulmonary epithelial-myoepithelial carcinoma is mainly surgical resection. The effect of radiotherapy and chemotherapy is not clear.

Epithelial-Myoepithelial Carcinoma of the Inferior Turbinate: A Case Report

DNA Aneuploidy in Malignant Salivary Gland Neoplasms is Independent of USP44 Protein Expression

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.

A retrospective study of the treatment for epithelial-myoepithelial carcinoma / 中华放射肿瘤学杂志

Objective To evaluate the risk of locoregional recurrence (LRR) and the influencing factors for long-term survival in patients with epithelial-myoepithelial carcinoma (EMCa).Methods A retrospective analysis was performed for 18 EMCa patients, who received initial therapy or initial adjuvant therapy in our hospital from 1999 to 2015, to investigate their survival.Among these patients, 8(44%) underwent surgery alone, 9(50%) received adjuvant radiotherapy, and 1(6%) received radical concurrent chemoradiotherapy.Locoregional recurrence-free survival (LRFS) and overall survival (OS) rates were compared between these groups.The Kaplan-Meier mtthod was used to calculated survival rates and log-rank test was used to compare the LRFS.Results With a median follow-up time of 46 months, 5 patients developed LRR, and the 5-year LRFS and OS rates were 69% and 93%, respectively.The patients treated with radiotherapy had a significantly higher 5-year LRRFS rate than those not treated with radiotherapy (71% vs.57%, P=0.569).Conclusions LRR is the main failure mode of EMCa treatment, and further improving local control is the key to improved survival.

Epithelial-myoepithelial carcinoma of salivary glands, report of 23 cases / 中国耳鼻咽喉头颈外科

OBJECTIVE Epithelial-myoepithelial carcinoma (EMC) is a rare malignant neoplasm of the salivary glands. The aim of the study was to investigate the diagnosis, treatment and prognosis of EMC of the parotid gland.METHODSThe clinical data of 23 cases of EMC of the parotid gland from 1999 to 2013 were analyzed retrospectively. Of all the patients, 13 cases received radical surgery only, 9 cases received postoperative radiotherapy, and one case received postoperative chemotherapy. Kaplan-Meier survival curve was used to analyze the clinical data. RESULTSThe overall survival rate at 5-year was 81.0% and the overall no recurrence survival rate at 5-year was 67.3%. The recurrence rate was 39.1% (9/23). The 5-year no recurrence survival rate of radical surgery group was 55.6%, compared with 80.0% in the combined modality therapy group, there was no significant deference (χ2=2.232, P=0.135).CONCLUSIONThe postoperative recurrence rate of EMC is high. Radical surgery could be the main treatment of EMC, when the surgical margin couldn't be achieved, adjuvant radiotherapy or chemotherapy might be helpful.

Malignant Transformation Of Breast Ductal Adenoma: A Diagnostic Pitfall

We present what is believed to be the first report of heterogeneous carcinoma arising from breast ductal adenoma. A 57-year-old woman presented with a nodule in her right breast. Histological examination of a vacuum-assisted biopsy specimen revealed epithelial tubular proliferation and papillary apocrine epithelium. The myoepithelial cells lining the tubules were confirmed by immunohistochemistry. The nodule had increased in size 18 months later and tumorectomy was performed. The surgical specimen revealed proliferating apocrine epithelium with sheet-like and cribriform architecture within a mammary duct. Some myoepithelial cells showed irregular proliferation around the tubular epithelium. All three components, including apocrine, myoepithelial and glandular cells, showed prominent nuclear atypia and significant mitotic activity. The patient was diagnosed with malignant transformation of ductal adenoma. The malignant potential of ductal adenoma has not previously been discussed, but this heterogeneous carcinoma could represent a serious pitfall in the diagnosis of ductal adenomas.

A Case of Epithelial-Myoepithelial Carcinoma Originating from Minor Salivary Gland of Tongue Base / 대한이비인후과학회지

Epithelial-myoepithelial carcinoma (EMC) is a rare, often low grade malignant neoplasm that occurs most commonly in the parotid gland. However, EMC originating from a minor salivary gland is extremely rare. A 38-year-old female patient was referred to our department for dysarthria and a huge mass at the base of tongue. Transoral resection of the mass was performed using a midline glossotomy approach. The pathological examination revealed epithelial-myoepithelial carcinoma of a minor salivary gland. Sequential adjuvant chemoradiotherapy was performed. After clinical follow-up of 26 months, she has remained free of disease. We report on this rare condition in order to share our experience.

Epithelial-Myoepithelial Carcinoma of External Auditory Canal Evolving from Pleomorphic Adenoma

A 45-year-old female patient visited the clinic due to ear fullness. A 8 mm sized mass was found in the external auditory canal. It was surgically removed and the pathology exam confirmed epithelial-myoepithelial carcinoma (EMC). EMC is glandular epithelial neoplasm of the salivary gland. EMC originating from the external auditory canal is very rare, and only 1 case has been reported so far in the literature. The difference between our case and the one previously published was that the tumor was previously proven as a benign tumor (i.e. pleomorphic adenoma) 5 years ago. Therefore, this is the first case in the literature showing the evolvement of pleomorphic adenoma of the external auditory canal into EMC.

Epithelial-Myoepithelial Carcinoma of External Auditory Canal Evolving from Pleomorphic Adenoma

A 45-year-old female patient visited the clinic due to ear fullness. A 8 mm sized mass was found in the external auditory canal. It was surgically removed and the pathology exam confirmed epithelial-myoepithelial carcinoma (EMC). EMC is glandular epithelial neoplasm of the salivary gland. EMC originating from the external auditory canal is very rare, and only 1 case has been reported so far in the literature. The difference between our case and the one previously published was that the tumor was previously proven as a benign tumor (i.e. pleomorphic adenoma) 5 years ago. Therefore, this is the first case in the literature showing the evolvement of pleomorphic adenoma of the external auditory canal into EMC.

Epithelial-myoepithelial carcinoma on the superficial lobe of the parotid gland: a case report

Epithelial-myoepithelial carcinoma (EMC) is a low-grade malignant salivary gland neoplasm that was first described in 1972. EMC occurs in the older age group, there is a female predilection and mainly involves the parotid gland. Most authors recommend superficial parotidectomy as a treatment for low-grade malignant tumor in the superficial lobe of parotid gland. The treatment of epithelial-myoepithelial tumors typically includes surgical excision aimed at achieving a R0 resection. This paper reports a case of EMC of the parotid gland treated only by a conservational surgical excision. The lesion was exposed by the retromandibular approach and detached. After the parotid gland envelop was exposed, the mass was observed and was easy to remove due to capsulation. The preoperative diagnosis was a pleomorphic adenoma on the left parotid gland. The tumor was removed surgically with a conservative extracapsular dissection. The postoperative diagnosis was EMC, so superficial parotidectomy or radiation therapy was considered. Nevertheless, the patient was observed and no additional treatment was attempted because the patient was old and a successfully excision of the tumor had been achieved.

Epithelial-Myoepithelial Carcinoma of the Parotid Gland: A Case Report

PURPOSE: Epithelial-myoepithelial carcinoma (EMC) represents about the 1% of the malignant neoplasms in the salivary glands and clinically most commonly found localized, well defined and sometimes presents orofacial pain. Treatment of choice is surgical excision. Postoperative radiotherapy can be used when surgical margins are doubtful. We report our experience of EMC of the parotid gland. METHODS: A 78-year-old man presented with a three-year history of a localized, painless, 7 x 6 cm sized recurred tumor in his right preauricular area. He was diagnosed as EMC of the right parotid gland. So a total parotidectomy was performed. In his old medical history, he had a mass in the same area 5 years ago. The diagnosis of pleomorphic adenoma was made and the mass excision was performed at the local clinic without further evaluation. RESULTS: It was unable to visually discriminate between the tumor and the normal tissue. So a total parotidectomy was performed. The patient was got post-operative radiotherapy and was followed up for 9 months. There was no specific evidence of recurrence. CONCLUSION: We present a case of EMC of the parotid gland in right preauricular area, which is uncommon. So we report a uncommon case of EMC to discuss about our experience with relevant journal discussion.

Carcinoma sebáceo/mioepitelial de parótida: análisis histopatológico e inmunohistoquímico / Sebaceous epithelial myoepithelial carcinoma of parotid: histopathological and immunohistochemical analysis

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.

A Case of Epithelial-Myoepithelial Carcinoma in Left Submandibular Gland / 대한이비인후과학회지

Epithelial myoepithelial carcinoma is a rare tumor with slightly less than one percent of salivary gland neoplasm. Histologically, the carcinomas are characterized by a mixture of trabecular structure with outer myoepithelial cells and inner ductal cells, which are confirmed by electron microscopy and immunohistochemistry. Although this neoplasm exhibits high degree of cellular differentiation histologically, it is classified into low grade carcinoma because of its locally infiltrative, destructive growth pattern, and tendency to metastasize. We report a 45-year-old woman with epithelial-myoepithelial carcinoma arising from a submandubular gland, one of the most unusual locations.

Fine-Needle Aspiration Cytology of Epithelial-Myoepithelial Carcinoma of Parotid Gland: A Report of Two Cases / 대한세포병리학회지

Epithelial-myoepithelial carcinoma (EMC) is a rare low grade malignant tumor of the salivary glands and it shows a characteristic biphasic population of epithelium and myoepithelium. It shows various cytologic and histologic features, so making an exact diagnosis is difficult. We report here on two cases of epithelial-myoepithelial carcinoma arising from the parotid gland and we compare the cytologic findings of the aspirated samples with the histologic findings of the tumors. We think the finding of mixed pattern of large, clear myoepithelia and small epithelia is the most valuable finding in the diagnosis of EMC.

Cytologic Features of Epithelial-Myoepithelial Carcinoma Occurring in External Auditory Canal: A Case Report / 대한세포병리학회지

Epithelial-myoepithelial carcinoma (EMC) is a low grade malignant neoplasm that commonly occurs in the parotid gland. Recently, we investigated a case of EMC that occurred in the external auditory canal (EAC) in a 35-year-old male, and this tumor was initially diagnosed as pleomorphic adenoma. The difficulty associated with diagnosing EMC by aspiration cytology arises from both the rare incidence of this tumor and the overlapping spectrum of cytological features found in various salivary gland tumors, such as pleomorphic adenoma, basal cell adenoma and adenoid cystic carcinoma.

Epithelial-myoepithelial carcinoma arising in pleomorphic adenoma of palate

A case of epithelial-myoepithelial carcinoma transformed in pleomorphic adenoma occurring in palate of a 61 years old woman is reported. The tum or was composed of 2 different components, pleomorphic adenoma and epithelial myoepithelial carcinoma, accounting for approximately 40% and 60 % of whole tumor, respectively. As the results of the immunohistopathologic study, epithelial-myoepithelial carcinoma showed multiple tubular or solid nest, which were separated by a basement membrane and considered of variable proportion of 2 cell types, cuboidal epithelial cells positive for cytokeratin and clear myoepithelial cells positive for glial fibrillary acid protein, wheres the myoepithelial nest of pleomorphic adenoma intermingled with hyaline and myxoid stroma. The malignancy was demonstrated by convincing evidence of invasion into the submucosa, although the epithelial-myoepithelial carcinoma com ponent was mostly surrounded by the pleomorphic adenoma componemts. An increased immunoreactivity of proliferating cell nuclear antign in the epithelial myoepithelial carcinoma area in comparison to the pleomorphic adenoma also suggested epithelial-myoepithelial carcinoma arising in a pleomorphic adenoma.

A Case of Epithelial-myoepithelial Carcinoma of the Parotid Gland

Epithelial-myoepithelial carcinoma (EMC) of the salivary gland is a rare tumor that comprises approximately 1% of all salivary gland tumors. It has a distinctive histological appearance comprising ductal structures with an inner epithelial cell component and an outer layer of myoepithelial cells. We report a case of EMC of the parotid gland in a 41- year-old man. He presented left-sided subauricular swelling developed 3-month earlier. Neck CT scans revealed a well-defined mass in the left superficial parotid gland. He underwent superficial parotidectomy and was diagnosed as EMC. He was taken postoperative radiotherapy. There was no evidence of recurrence during a follow-up period of 12 months.

Epithelial-myoepithelial carcinoma of the parotid gland: a case report

Epithelial-myoepithelial carcinoma(EMC) of the salivary glands is a rare tumor first discribed in 1972. The EMC comprises approximately 1% of all salivary gland tumors. It is characterized by tubular and solid growth pattern with a dual cell population including an inner layer of epithelial cells, which is peripherally bounded by a layer of clear myoepithelial cells. It is demonstrated that tumor with solid slowly growing pattern, generally have a higher frequency of local recurrence. We report a case of parotidic EMC in a 30 years old woman with literature review

Epithelial-Myoepithelial Carcinoma of the Parotid Gland: Report of a Case Misinterpreted as Pleomorphic denoma on Fine Needle Aspiration Cytology / 대한세포병리학회지

Epithelial-myoepithelial carcinoma(EMC) is a rare, low grade malignant tumor of the salivary glands. The EMC has a distinctive histological appearance comprising ductal structures with an inner epithelial cell component and an outer layer of myoepithelial cells which show plump clear cytoplasm. The cytologic features of the EMC have been rarely described. A correct cytological diagnosis to this rare tumor is difficult with high false negative rate. We report a case of EMC in which fine needle aspiration cytologic findings were misinterpreted as a pleomorphic adenoma.