Epithelialmyoepithelial carcinoma arising from the. Notably, metastatic renal cell carcinoma is positive for both vimentin and cd10, but not s100 protein. We now need to understand more about their origin and to reconsider their place in the complex process of mammary morphogenesis. The objective of the study is to document the clinical, histopathological and immunological findings of epithelial myoepithelial carcinoma emec which is an uncommon neoplasm. To the best of our knowledge, such an association has never been. Clear cell predominance within myoepithelial carcinoma is rare but well described. Rare, low grade tumor with epithelial and myoepithelial components and frequent local recurrence also called glycogen rich adenoma clear cell pattern, myoepithelioma spindle cell and plasmacytoid patterns, myoepithelial carcinoma if only myoepithelial differentiation and marked cytologic atypia. Here we present an exceptional case of myoepithelial carcinoma and myoepithelioma association. Despite several commonalities between these entities, various features distinguish.
Although rare cases of highgrade emca have been reported, 3,58 most. The patient underwent biopsies demonstrating the diagnosis of epithelial myoepithelial carcinoma of the base of tongue and a pet scan. Combined epithelialmyoepithelial lesions of the breast. We strongly recommend that you talk with a trusted. Herein we report the morphological features of myoepithelial carcinoma in a 74yearold female clinically presenting with a parotid mass. Hunt, in diagnostic immunohistochemistry third edition, 2011. These may be positive for alpha smooth muscle actin and can contract and expel the secretions of exocrine glands. Primary myoepithelial carcinoma of palate world journal. Due to rarity of its occurrence, histogenesis and clear cut therapeutic. Epithelial myoepithelial carcinoma emca is a rare malignant tumour that typically arises in a salivary gland and consists of both an epithelial and myoepithelial component. Myoepithelial carcinoma of the breast treated with surgery. Approximately 66% of these tumors occur in a part of the salivary gland, known as the parotid gland. Epithelial myoepithelial carcinoma emc is a rare malignant salivary gland neoplasm that most commonly occurs in the parotid gland, but can also arise in.
It is also known as a malignant myoepithelial tumor. Epithelial myoepithelial carcinoma emc is a tumour consisting of various proportions of two cell types which typically form ductlike structures. The lowgrade element must show areas in which both epithelial and myoepithelial cells are distributed in an organized fashion and have a low proliferative rate. Donath and coworkers 233 are usually credited with calling attention to this tumor in 1972. They are predominantly found in the parotid gland and represent approximately 1% of salivary gland tumours. Oncocytic and apocrine epithelial myoepithelial carcinoma. Potentially, the malignant elements can be a highgrade carcinoma, myoepithelial carcinoma, biphasic carcinosarcoma, or a pure sarcoma. We present a rare case of emc arising in the submandiblar gland. Epithelialmyoepithelial carcinoma emc of salivary gland is a malignant epithelial tumor, composed of two distinct cell types, affecting either the major or minor salivary glands. Epithelial myoepithelial carcinoma emca is a lowgrade malignant tumour. Epithelialmyoepithelial carcinoma emc is regarded as a rare lowgrade malignant tumor of the salivary gland, accounting for 0. We report a 61yearold patient with breast myoepithelial carcinoma who developed locoregional and distant metastases and received many chemotherapy. Myoepithelial carcinoma is an uncommon, malignant, soft tissue tumor of myoepithelial cells.
Epithelial myoepithelial carcinoma emc was first described by danath et al. Myoepithelial carcinoma is a rare malignant cancerous tumor that usually occurs in the salivary glands in the mouth, but can also occur in skin and soft tissues. Epithelial myoepithelial carcinoma emc is a rare biphasic tumor of the salivary gland. Epithelial myoepithelial carcinoma emc is a lowgrade, malignant biphasic salivary tumor of probable intercalated duct origin that comprises 1% to 2% of all salivary neoplasms. Rare, low grade tumor with epithelial and myoepithelial components and frequent local recurrence also called glycogen rich adenoma clear cell pattern, myoepithelioma spindle cell and plasmacytoid patterns, myoepithelial carcinoma if only myoepithelial differentiation and.
Mr images showed a lesion with intermediate t1weighted signal intensity and a relatively high t2weighted signal intensity. As per literature, it involves the parotid gland most frequently and rarely, the minor salivary glands. The importance of being a myoepithelial cell breast. Epithelialmyoepithelial carcinoma emc is a lowgrade, malignant biphasic.
It is similar to salivary gland myoepithelial tumors showing same histological features morphology. Epithelial myoepithelial carcinoma the tumor is composed of two cell types which form ductlike structures. Introduction epithelial myoepithelial carcinoma emc is defined by the world health organization who classification in 1991 as a rare lowgrade maligvary glands tumors 1,2. Epithelial myoepithelial carcinoma is regarded as a lowgrademalignancy tumor and the treatment protocol involves wide surgical excision with secure clear margins. The reason for this has been the perception that its role in normal physiology is confined to lactation and the belief that most breast cancers arise from luminal epithelial cells. A 54yearold woman presented with an abnormal lung shadow on a. Pdf epithelialmyoepithelial carcinoma emc is a rare biphasic tumor accounting for. Primary salivary glandtype tumors of the lung are rare and include mucoepidermoid carcinoma, adenoid cystic carcinoma, acinic cell carcinoma, oncocytoma, epithelial myoepithelial carcinoma, benign myoepithelioma, and mixed tumors of both benign and malignant nature 116.
Epithelial myoepithelial carcinoma, with a biphasic pattern of ducts lined with inner and outer layers of epithelial and myoepithelial cells, may contain prominent foci of clear myoepithelial cells without the inner ductal layer. Infiltrating myoepithelial carcinoma of the breast, a case. Primary pulmonary myoepithelial carcinomas are exceedingly rare, with only five known prior cases reported in the. The mammary myoepithelial cell cinderella or ugly sister. Epithelial cancer definition of epithelial cancer by. Epithelial myoepithelial carcinoma emca is a salivary tumor with dual cell population. The clear cells are often relatively large and usually predominate.
The breast myoepithelial cell is the cinderella of mammary biology. Myoepitheliomas are tumors arising from myoepithelial cells mainly or exclusively. Myoepithelial carcinomas exhibit a wide spectrum of cytomorphologic features and diverse clinical outcomes. Myoepithelial carcinoma and epithelialmyoepithelial. As a result of their morphologic heterogeneity, they can be confused easily with many tumours.
Myoepithelial carcinoma malignant myoepithelioma of the breast is a rare tumor, for which only a limited number of reports have been published. Case epithelialmyoepithelial carcinoma of the report base. Epithelial myoepithelial carcinoma emc is a rare biphasic tumor accounting for less than 2% of all salivary gland malignancies. 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. Primary salivary glandtype tumors of the lung are rare. These tumours have been named in a lot of different ways, including adenomyoepithelioma, epithelial myoepithelial tumour, epithelial myoepithelial carcinoma or epithelial myoepithelial tumour of uncertain malignant potential. An inner layer of duct lining cells and an outer layer of clear cells, which typically form doublelayered ductlike structures. Epithelial myoepithelial carcinoma is a rare tumor which makes up about 0. Although its contribution to benign and some malignant pathologies is recognised, it has been largely neglected in molecular and biological studies. Because of its rarity and unproven malignant potential, the optimal therapy for pemc has not been defined.
Epithelial myoepithelial carcinoma of head and neck region. Epithelialmyoepithelial carcinoma emc is a lowgrade, malignant biphasic salivary tumor of probable intercalated duct origin that comprises 1% to 2% of all salivary neoplasms. Epithelial myoepithelial carcinomas are rare neoplasms usually arising from the salivary glands. Epithelialmyoepithelial carcinoma with highgrade transformation of. Epithelialmyoepithelial carcinoma emca is a salivary tumor with dual cell. While the histologic, immunohistochemical, and even ultrastructural features have been well described, a definite diagnosis of mec based on cytology. K5k14positive cells contribute to salivary glandlike. Pdf download for mammary epithelialmyoepithelial carcinoma. Pathology outlines epithelial myoepithelial carcinoma. Epithelialmyoepithelial carcinoma of salivary glands. The tumor may also very rarely originate in minor salivary glands of the base of the tongue. Sox10 is a novel marker of acinus and intercalated duct. Epithelial myoepithelial carcinoma emc is extremely rare compared to other carcinomas, and is considered a low malignancy neoplasm.
Myoepithelial carcinoma of the breast mec, a lesion composed purely of malignant myoepithelial cells, remains a rarely reported phenomenon. Mammary myoepithelial cells have recently been described as natural tumour suppressors. Epithelialmyoepithelial carcinoma of the parotid gland. It is observed in a wide age category of children and adults. It presents as a slowly growing, asymptomatic small size mass. Pulmonary epithelial myoepithelial carcinoma pemc is a rare subset of salivary glandtype tumors of the lung. Epithelialmyoepithelial carcinoma of the tracheaa rare. Soft tissue myoepithelial carcinoma and myoepithelioma are rare entities, part of myoepithelial tumors. Epithelial myoepithelial carcinoma in the base of the tongue.
A hybrid carcinoma of epithelialmyoepithelial carcinoma and adenoid cystic carcinoma in maxillary sinus article pdf available in journal of korean medical science 193. Myoepithelial carcinoma an overview sciencedirect topics. Epithelialmyoepithelial carcinoma of salivary gland. Epithelial myoepithelial carcinoma emc of the salivary gland is a lowgrade carcinoma with a predilection for the parotid gland. It is generally composed of variable proportions of two cell types.
Primary epithelialmyoepithelial carcinoma of the lung. In pleomorphic adenomas, the myoepithelial elements blend into this. Myoepithelial cell definition of myoepithelial cell by. Epithelial myoepithelial tumors of the lung are rare neoplasms whose biological behavior and clinical course still remain to be defined. Linkpage citation a 49yearold, hivpositive man with a cd4 count of 81 cellsmicrol presented with a 3week history of hiccupping. We describe an unusual salivary neoplasm, an epithelial myoepithelial carcinoma emec of the parotid gland, that occurred in a 64yearold man.
There is an inner layer of ductlining cells and an outer layer of clear myoepithelial cells. Epithelialmyoepithelial carcinoma lester dr thompson, md. Epithelial myoepithelial carcinoma emca is a rare salivary gland. Pdf epithelialmyoepithelial carcinoma of the parotid gland. Myoepithelial cells in pathology pubmed central pmc. Epithelial myoepithelial carcinoma of the parotid gland author. Unusual epithelialmyoepithelial carcinoma in palate case.
Given a malignant neoplasm of the tonsil, tonsillectomy was always the first choice of treatment. Simple excision should be avoided because epithelial myoepithelial carcinoma often has a destroyed epithelium 8, 10. Myoepithelial carcinoma malignant myoepithelioma may show a combination of spindled and epithelioid cells arranged in small nests and cords with glandlike features, but a multilobulated growth. Most of the reports emphasized diagnosis and pathology but not biological behavior and treatment. Myoepithelial cells sometimes referred to as myoepithelium are cells usually found in glandular epithelium as a thin layer above the basement membrane but generally beneath the luminal cells. Though the name for this tumor was coined by donath et al. Three additional cases of typical emc with transition to highgrade carcinoma are presented. One week after the onset of the hiccups, he had been seen at an urgent care center. Ephitelial myoepithelial carcinoma, oral cavity, soft palate, immunohistochemistry. A ct scan showed a fairly well defined heterogeneous lesion with smooth margins and slight enhancement. Epithelial myoepithelial carcinoma emc of salivary gland is a malignant epithelial tumor, composed of two distinct cell types, affecting either the major or minor salivary glands. Introduction epithelial myoepithelial carcinoma emc is defined by the world health organization who classification in 1991 as a rare lowgrade malignant neoplasm and account for than 1% of salivary glands tumors 1,2.
Ame, first described by hamperl in 1970, 33 is a biphasic neoplasm composed of ductal and myoepithelial cells that is identical to epithelial myoepithelial carcinoma of the salivary gland and lacks the features of other specific diagnostic categories of related neoplasms. In the present report we describe a case of this unusual parotid gland neoplasia, radiologically simulating a benign lesion. The mammary myoepithelial cell was named the cinderella of mammary cell biology in light of the earlier focus on the luminal cell. The relatively recently recognized and rarely encountered malignant myoepithelial tumor ie, myoepithelial carcinoma was first described by stromeyer et al 11 in 1975 and has been included in the world health organization classification of salivary gland tumors since 1991. Epithelialmyoepithelial carcinoma of the base of tongue. E12, cytokeratin 14, p63, s100, calponin, smooth muscle actin, and musclespecific actin. They are found in the sweat glands, mammary glands, lacrimal glands, and salivary glands. They were incorporated into the world health organization classification of soft tissue tumors in 2002. Biomorphic epithelial myoepithelial carcinomas are similar to acc in their staining of outer clear cells with myoepithelial markers in conjunction with vimentincytokeratins. Combined myoepithelial carcinoma and myoepithelioma in. Epithelialmyoepithelial carcinoma an overview sciencedirect. Myoepitheliomas mostly occur in salivary glands, as well as in breast, skin, and lung.
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