Epithelial-myoepithelial Carcinoma Research Articles

Nasal epithelial myoepithelial carcinoma: An unusual cause of epiphora, a case report and review of the literature.

Introduction:Epithelial myoepithelial carcinoma (EMC) of the nasal cavity is a rare tumor, and here we describe the first case of EMC of the nasal cavity presenting with epiphora. A case presentation and review of the literature is provided.Methods:A case report is described of a 63-year-old man who presented with unilateral epiphora and was found via a thorough history and physical examination to have a nasal tumor. The physical examination consisted of an ocular examination, including probing and irrigation, and a detailed nasal examination (anterior rhinoscopy, nasal endoscopy). The nasal examination was prompted by the patient's report of concurrent nasal symptoms during history taking. Immunohistochemistry subsequently identified the nasal tumor as EMC. A literature search was performed to gain insights into similar malignancies of the nasal cavity.Results:Eight cases of EMC of the nasal cavity were identified in the literature, none of the patients presented with epiphora. The case presented here resulted in resolution of the patient's symptoms and no evidence of disease after surgical excision.Conclusion:Epithelial myoepithelial is a rare salivary gland malignancy that can arise in the nasal cavity. Unilateral epiphora with concurrent nasal symptoms should prompt nasal cavity examination for the possibility of an obstructive tumor.

A Rare Case of Bronchial Epithelial-Myoepithelial Carcinoma with Solid Lobular Growth in a 53-Year-Old Woman.

Epithelial-myoepithelial carcinoma (EMC) of lung is a minor subset of salivary type carcinoma of lung of known low grade malignancy. Histologically, two-cell components forming duct-like structure with inner epithelial cell layer and outer myoepithelial cell layer are characteristics of EMC. In salivary gland, dedifferentiation of conventional low grade malignancy has been reported and is thought to be related with poor prognosis. However, precise histomorphology and prognostic factors of pulmonary EMC have not been clarified due to its rarity. Herein, we reported a rare case of EMC presented as endobronchial mass in a 53-year old woman, which showed predominant solid lobular growth pattern and lymph node metastases.

A Case of Epithelial-myoepithelial Carcinoma of Parotid Gland that Caused Multiple Cranial Nerve Disorders

A Case of Epithelial-myoepithelial Carcinoma of Parotid Gland that Caused Multiple Cranial Nerve Disorders

多発脳神経障害をきたした耳下腺上皮筋上皮癌例

多発脳神経障害をきたした耳下腺上皮筋上皮癌例

A case of carcinoma ex pleomorphic adenoma with epithelial-myoepithelial carcinoma of the minor salivary gland

A case of carcinoma ex pleomorphic adenoma with epithelial-myoepithelial carcinoma of the minor salivary gland

Global transcriptome and sequenome analysis of formalin‐fixed salivary epithelial–myoepithelial carcinoma specimens

Diverse microarray and sequencing technologies have been widely used to characterize molecular changes in malignant epithelial cells in salivary neoplasms. Such gene expression studies to identify markers and targets in tumor cells are, however, compromised by the cellular heterogeneity of these tumors and by the difficulties to accrue matching controls representing normal salivary glands. Seventeen samples of primary salivary epithelial-myoepithelial carcinoma along with tissue from six normal major salivary glands were microdissected from paraffin-embedded tissue. Pools of RNA from highly enriched preparations of these cell types were subjected to expression profiling using a whole-transcriptome shotgun sequencing experiment. In parallel, extracted genomic DNA was used for the 50 gene hotspot panel sequenome. KRAS mutations in three patients (18%), NRAS mutations in one patient (6%), but no HRAS, MET, PIK3CA, or BRAF mutations. Using strict and conservative criteria, 220 differentially expressed transcripts were found, with 36% up- and 64% downregulated. The transcripts were annotated using NCBI Entrez Gene, and computationally analyzed with the Ingenuity Pathway Analysis program. From these significantly changed expressions, the analysis identified 26 cancer-related transcripts and 16 transcripts related to mitochondrial dysfunction overlapping with three cancer-related genes. These 220 differentially expressed genes including microRNAs provide here a sufficiently large set to specifically define epithelial-myoepithelial carcinoma and to identify novel and potentially important targets for diagnosis, prognosis, and therapy of this cancer.

Cytologic features of sebaceous epithelial‐myoepithelial carcinoma of the salivary gland: A brief report of a rare variant

Diagnostic CytopathologyVolume 43, Issue 8 p. 676-679 Letter to the Editor Cytologic features of sebaceous epithelial-myoepithelial carcinoma of the salivary gland: A brief report of a rare variant Lisa Laskiewicz M.D., Lisa Laskiewicz M.D. Department of Pathology, UMass Memorial Medical Center, Worcester, MassachusettsSearch for more papers by this authorMarjan Mirzabeigi M.D., Marjan Mirzabeigi M.D. Department of Pathology, UMass Memorial Medical Center, Worcester, MassachusettsSearch for more papers by this authorWilliam Faquin M.D., PH.D., William Faquin M.D., PH.D. Department of Pathology, Massachusetts General Hospital, Boston, MassachusettsSearch for more papers by this authorMarie Lithgow M.D., Corresponding Author Marie Lithgow M.D. Department of Pathology, UMass Memorial Medical Center, Worcester, MassachusettsCorrespondence to: Marie Lithgow, MD, Department of Pathology, UMass Memorial Medical Center, Biotech 3, 1 Innovation Drive, Worcester, MA, USA. E-mail: [email protected]Search for more papers by this author Lisa Laskiewicz M.D., Lisa Laskiewicz M.D. Department of Pathology, UMass Memorial Medical Center, Worcester, MassachusettsSearch for more papers by this authorMarjan Mirzabeigi M.D., Marjan Mirzabeigi M.D. Department of Pathology, UMass Memorial Medical Center, Worcester, MassachusettsSearch for more papers by this authorWilliam Faquin M.D., PH.D., William Faquin M.D., PH.D. Department of Pathology, Massachusetts General Hospital, Boston, MassachusettsSearch for more papers by this authorMarie Lithgow M.D., Corresponding Author Marie Lithgow M.D. Department of Pathology, UMass Memorial Medical Center, Worcester, MassachusettsCorrespondence to: Marie Lithgow, MD, Department of Pathology, UMass Memorial Medical Center, Biotech 3, 1 Innovation Drive, Worcester, MA, USA. E-mail: [email protected]Search for more papers by this author First published: 23 November 2014 https://doi.org/10.1002/dc.23235Citations: 4Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Citing Literature Volume43, Issue8August 2015Pages 676-679 RelatedInformation

Iodine-125 brachytherapy for the treatment of a large parotid epithelial-myoepithelial carcinoma in a child

Epithelial-myoepithelial carcinoma (EMC) in the salivary glands is rare. Tumours originating from salivary glands are also rare in children. Radical resection is the main treatment used for EMC in salivary glands. However, this surgery is commonly associated with functional and/or cosmetic deficits. There is also a high percentage of local recurrence after a tumourectomy. We present a typical case of recurrent EMC in the left parotid gland of an 8-year-old girl. The patient was treated with iodine-125 brachytherapy. At the 2-month follow-up, the tumour size was reduced by 80%, and at 1 year, no tumour tissue was detected on positron emission tomography/computed tomography. At the 6-year follow-up, no local recurrence or distant metastasis was found and no complications had occurred. Iodine-125 brachytherapy is a potentially appropriate alternative treatment for EMC in the salivary glands, especially for paediatric patients.

Epithelial‐Myoepithelial Carcinoma of the Salivary Glands: An Analysis of 246 Cases

Objectives: Epithelial-myoepithelial carcinoma (EMC) is a rare neoplasm of the salivary glands. In this study we aim to analyze the clinical features of EMC using national registry data. Methods: Retrospective cohort study. The Surveillance, Epidemiology, and End Results (SEER) database was queried. Data were analyzed with respect to various demographic and clinicopathologic factors. Survival was analyzed using the Kaplan-Meier and Cox proportional hazards models. Results: A total of 246 cases were available for frequency analysis and 189 for survival analysis. Mean age at diagnosis was 63.8 (±15.4) years. EMC affected females more frequently (57.3%). Distant metastases were present in only 4.5% of cases. Overall disease-specific survival (DSS) at 15 years was 80.2%. Patients with low-grade histology had better DSS at 10 years than those with high-grade tumors (96.2% vs 71.0%, P = .0124). Tumors >4 cm exhibited the worst DSS at 15 years (64.5%, P = .0009). In the survival analysis cohort, all but 6 patients underwent surgery, and a total of 78 (41.3%) received adjuvant radiation therapy (RT). However, no survival benefit was noted for patients who received RT when compared with those who did not ( P = .4594). Although a trend toward higher survival with RT (97.1% vs 83.5% without) was observed when high tumor grade and size >4 cm were excluded, it was not statistically significant ( P = .2058). Conclusions: This report represents the largest series of EMC to date. Despite being regarded as a low-grade, indolent tumor, a significant fraction of our cohort underwent RT in addition to surgery, with no apparent added survival benefit.

Recent advances in the diagnostic pathology of salivary carcinomas.

This review concentrates on the most important developments since the WHO classification of 2005. In particular, the identification of specific translocations is revolutionising the way salivary tumours are considered and will have a major impact on future diagnostic practice. This is true so far in four malignancies: mammary analogue secretory, mucoepidermoid, adenoid cystic and hyalinising clear cell carcinomas. In each, the gene rearrangement is found in 80 % or more of cases. Two 2014 publications have added further possible candidates with molecular abnormalities to the list (cribriform adenocarcinoma of the tongue and minor salivary glands and epithelial-myoepithelial carcinoma), but these findings have yet to be confirmed by other investigators. The advances in molecular pathology have also allowed re-evaluation of the morphology; for example, it is now realised that the histological spectrum of hyalinising clear cell carcinoma includes intracellular mucin in over half of cases, as well as tumours with only scanty clear cells. In a separate development, it is now proposed that salivary duct carcinoma can be subdivided along molecular lines, in ways analogous to breast cancer, suggesting new therapeutic prospects in an otherwise highly aggressive malignancy.

설하선에서 발생한 상피-근상피암종 1예

Epithelial-Myoepithelial carcinoma (EMC) is rare salivary gland neoplasm with a propensity to arise from major salivary gland, particularly of the parotid. EMC shows a characteristic biphasic population of epithelium and myoepithelium but shows various cytologic and histologic features, so this low grade malignancy can cause diagnostic confusion during workup and also frequently be misdiagnosed as other high grade salivary malignancy. Therefore histopathologic diagnosis is essential and immunohistochemistry is an important tool when making differential diagnosis. The treatment of choice is wide surgical excision, and long-term follow up is necessary to evaluate local recurrences. We herein report a case of EMC arising in a sublingual gland with a review of the relevant literature. (J Clinical Otolaryngol 2014;25:75-79)

Diagnostic Utility of PLAG1 Immunohistochemical Determination in Salivary Gland Tumors

PLAG1 (pleomorphic adenoma gene 1) is a proto-oncogene whose overexpression is a crucial oncogenic event in salivary gland pleomorphic adenomas (PA), and in carcinoma ex-PA. The aim of the present study is to evaluate the sensitivity and the specificity of PLAG1 as a marker in the differential diagnosis of salivary gland benign and malignant tumors. We examined 101 cases, including 36 PAs, 8 myoepitheliomas, 3 basal cell adenomas, and 1 canalicular adenoma among benign tumors; 16 mucoepidermoid carcinomas, 10 adenoid cystic carcinomas, 8 acinic cell carcinomas, 8 polymorphous low-grade adenocarcinomas, 7 salivary duct carcinoma, and 4 epithelial-myoepithelial carcinoma among malignant tumors. PLAG1 was diffusely positive in 94.4% of PAs and in all myoepitheliomas, although with a lower staining intensity. Among malignant tumors, 2 (25%) polymorphous low-grade adenocarcinomas and 1 salivary duct carcinoma ex-PA were positive. In conclusion, PLAG1 is a marker with good specificity for PA and could be a useful diagnostic adjunct in the diagnosis of salivary gland tumors. In particular, this marker is negative in the most common salivary carcinomas, including adenoid cystic carcinoma, mucoepidermoid carcinoma, and acinic cell carcinoma. However, some mimickers of PA, like polymorphous low-grade adenocarcinoma, may show occasional positivity for PLAG1, thus limiting its diagnostic use. In addition, carcinoma ex-PA shows consistent positivity, and therefore should be considered as a diagnostic possibility in case of a malignant tumor with PLAG1 expression.

Epithelial-myoepithelial carcinoma of the hypopharynx: A rare case.

Epithelial-myoepithelial carcinoma (EMC) is a rare neoplasm, which predominantly arises in the parotid gland. EMC is characterized by two types of cells; myoepithelial and epithelial cells arranged in well-defined tubules. A 48-year-old male with a six-month history of dysphagia presented with a 2-cm-diameter mass in the left posterior wall of the hypopharynx. Histopathological examination revealed that the tumor cells were arranged in duct-like structures with an inner layer of ductal cells and an outer layer of clear cells. Immunohistochemically, the outer layer of clear cells stained positive for calponin, p63 protein, glial fibrillary acidic protein, S-100 protein and smooth muscle actin, which is consistent with a myoepithelial phenotype. The inner layer stained positive for cytokeratin and cytokeratin-7, which is consistent with an epithelial phenotype. The tumor was excised and no recurrence or metastasis was found 27 months following surgery. To the best of our knowledge, this is the first case of EMC described that has arisen from the hypopharynx.

Epithelial-myoepithelial carcinoma of the trachea-a rare entity case report.

Epithelial-myoepithelial tumors of the lung are rare neoplasms whose biological behavior and clinical course still remain to be defined. Epithelial-myoepithelial carcinoma (EMCa) is a low-grade malignant tumour. According to literature, most commonly occurs in salivary glands, particularly in parotic gland, but it can also occur in unusual locations such as breast, lachrymal gland, nose, paranasal sinus, lung, bronchus and, as in our case, trachea. There are no many documented case reports of a primary myoepithelial carcinoma in the trachea. We report a case of a 34-year-old man diagnosed with this unusual location of an epithelial-myoepithelial tumor. The tumour was removed by segmental tracheal resection and end-to-end anastomosis.

Primary Epithelial Myoepithelial Lung Carcinoma

Primary epithelial-myoepithelial carcinoma (EMC) of the lung is an extremely rare neoplasm that originates from submucosal bronchial glands and has been found in the salivary glands, breast tissue, and sweat glands. However, only a few cases in the respiratory tract have been identified. In the literature, most pulmonary EMCs have been reported to have developed endobronchially although a few EMC cases have been presented as intraparenchymatous tumors. We have identified a case of primary EMC that developed in the peripheral lung parenchyma.

Le carcinome myoépithélial de la glande lacrymale

Myoepithelial carcinomas are unusual tumors most often located in salivary glands. It is very rarely located in lacrimal glands; only 5 cases have been reported. We report a sixth case.An 88-year-old male patient presented with diplopia, painless right sided exophthalmia, as well as eyeball deviation due to a tumor located at upper external quadrant of the orbit. A biopsy initially suggested a sarcoma. The pathological analysis of the biopsy allowed diagnosing a myoepithelial carcinoma of the lacrimal gland. Despite the monoblock resection of the tumor, a recurrence was observed 3 months after removal. The patient died 8 months after the initial surgery.This case illustrates the clinical and pathological characteristics of a myoepithelial carcinoma. This tumor has a high grade of malignancy, and is very rarely described in lacrimal glands. The morphological diagnosis of this tumor is difficult with a problematic differential diagnosis with fusiform cells sarcomas (leiomyosarcoma, undifferentiated sarcoma), and epithelial-myoepithelial carcinoma.

Adenoid Cystic Carcinoma Is Frequently Characterized by MYB Rearrangement and May Be Distinguished From Other Salivary Gland Neoplasms Using Fluorescent In Situ Hybridization: Non-Squamous Cell Malignancies of the Head and Neck (thyroid, skin cancers, salivary gland and sinus cancers)

Adenoid cystic carcinoma (ACC) represents approximately 10-15% of salivary gland malignancies and almost universally exhibits a lethal clinical course. ACC is composed of a dual cell population (ductal and myoepithelial cells) and may demonstrate tubular, cribriform, and/or solid architectural patterns. Unfortunately, these histopathologic features are not specific to ACC and can be seen in other salivary gland neoplasms: basal cell adenoma, basal cell adenocarcinoma, epithelial-myoepithelial carcinoma, polymorphous low-grade adenocarcinoma, and adenocarcinoma not otherwise specified (NOS).

Case Study of a Parotid Gland Adenocarcinoma Dedifferentiated from Epithelial-Myoepithelial Carcinoma

Dedifferentiation is defined as high-grade malignant tumor development out of a low-grade malignant tumor. We present an adenocarcinoma tumor of the parotid gland that was dedifferentiated from a low-grade epithelial-myoepithelial carcinoma and was followed up for 3 years. Our patient, a 46-year-old female, presented with a left parotid mass of 20-year duration. Histopathologic results showed that there was only one area of typical epithelial-myoepithelial carcinoma, with foci of poorly differentiated adenocarcinoma (not otherwise specified; NOS) and clear cytoplasm in the parotid gland. Immunohistochemical staining results showed SMA (+), P63 (+), CK8 (+), and S100 (+) on epithelial cells. A review of the literature revealed 22 previously reported cases of dedifferentiated epithelial-myoepithelial carcinoma. In these cases, the malignant tumors that dedifferentiated from epithelial-myoepithelial carcinoma were adenoid cystic carcinoma, actinic cell carcinoma, polymorphous low-grade adenocarcinoma, mucoepidermoid carcinoma, and intraductal carcinoma. In our case, the malignant tumor that dedifferentiated from the epithelial-myoepithelial carcinoma was a poorly differentiated adenocarcinoma. Histopathological results showed that metastases were not seen in the neck-dissection material. As a result, our case will make a contribution to the literature in terms of prognosis, because there are very few reported cases of dedifferentiated adenocarcinoma development arising from epithelial-myoepithelial carcinoma.

High-grade transformation in salivary gland carcinomas

High-grade transformation (HGT) has been described in a variety of salivary gland carcinomas, including acinic cell carcinoma, adenoid cystic carcinoma, epithelial-myoepithelial carcinoma, polymorphous low-grade adenocarcinoma, myoepithelial carcinoma, low-grade mucoepidermoid carcinoma, hyalinizing clear cell carcinoma, and mammary analogue secretory carcinoma, although the phenomenon is a rare event. HGT tumors are composed of conventional carcinomas juxtaposed with areas of HG morphology, usually either poorly differentiated adenocarcinoma or undifferentiated carcinoma, in which the original line of differentiation is no longer evident. The HG component is generally composed of solid nests, sometimes occurring in cribriform pattern of anaplastic cells with large vesicular pleomorphic nuclei, prominent nucleoli and abundant cytoplasm. Frequent mitoses and extensive necrosis are evident. The Ki-67 labeling index is consistently higher in the HG component. p53 abnormalities have been demonstrated in the transformed component in a few examples, but the frequency varies by the histologic type. HER-2/neu over-expression and/or gene amplification is considerably exceptional. The molecular-genetic mechanisms responsible for the pathway of HGT in salivary gland carcinomas largely still remain to be elucidated. Salivary gland carcinomas with HGT have been shown to be more aggressive than conventional carcinomas with a poorer prognosis, suggesting the need for wider resection and neck dissection.

Primary Salivary Gland–Type Lung Cancer: Clinicopathological Analysis of 88 Cases from China

Salivary gland-type cancers are rare lung neoplasms involving mucoepidermoid carcinoma (MEC), adenoid cystic carcinoma (ACC), and epithelial-myoepithelial carcinoma (EMC). Their behavior and prognostic features are not clearly defined because of their low incidence. We retrospectively analyzed the clinicopathologic profiles of these tumors in a large series. Eighty-eight patients confirmed as having primary salivary gland-type lung cancer between May 2001 and January 2013 were included from the archives of two thoracic oncology center institutions in China and retrospectively evaluated. Of the total 88 patients, 69 were MEC, 12 ACC, and seven EMC. Overall survival (OS) at 3, 5, and 10 years was 91.3%, 86%, and 80.6% in all cases, respectively, and disease-free survival (DFS) was 90.1%, 78.6%, and 55%, respectively. No significant difference was found among MEC, ACC, and EMC groups regarding OS (p = 0.518) and DFS (p = 0.082). Tumor-node-metastasis stage, lymph node involvement, intrathoracic invasion, and margin status were found to be related with OS (p = 0.000, 0.029, 0.000, 0.004) and DFS (p = 0.018, 0.042, 0.002, 0.002). Intrathoracic invasion was an independent predictor for OS (hazard ratio [HR], 1.129; p = 0.039) and DFS (HR, 1.071; p = 0.011). For patients with MEC, pathological grade also was an independent predictor of OS (HR, 0.045; p = 0.006) and DFS (HR, 0.067; p = 0.001). Salivary gland-type lung cancers are a group of low-aggressive entities with higher tendency to recurrence/metastasis. Intensive clinical, radiological, and pathological examinations are essential to estimation of the risk stratification and management.