The objective of the study is to document the clinical, histopathological and immunological findings of epithelial myoepithelial carcinoma emec which is an uncommon neoplasm. Biomorphic epithelial myoepithelial carcinomas are similar to acc in their staining of outer clear cells with myoepithelial markers in conjunction with vimentincytokeratins. K5k14positive cells contribute to salivary glandlike. Epithelialmyoepithelial carcinoma of the parotid gland. One week after the onset of the hiccups, he had been seen at an urgent care center. Myoepithelial carcinoma of the breast mec, a lesion composed purely of malignant myoepithelial cells, remains a rarely reported phenomenon. There is an inner layer of ductlining cells and an outer layer of clear myoepithelial cells. Though the name for this tumor was coined by donath et al. Myoepitheliomas mostly occur in salivary glands, as well as in breast, skin, and lung. Primary myoepithelial carcinoma of palate world journal.
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. 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. Pdf download for mammary epithelialmyoepithelial carcinoma. Primary pulmonary myoepithelial carcinomas are exceedingly rare, with only five known prior cases reported in the. Sox10 is a novel marker of acinus and intercalated duct. The breast myoepithelial cell is the cinderella of mammary biology. Epithelialmyoepithelial carcinoma of salivary gland. Epithelial myoepithelial carcinoma is a rare tumor which makes up about 0. Epithelial myoepithelial carcinoma emca is a lowgrade malignant tumour. Clear cell predominance within myoepithelial carcinoma is rare but well described. Pulmonary epithelial myoepithelial carcinoma pemc is a rare subset of salivary glandtype tumors of the lung. We report a 61yearold patient with breast myoepithelial carcinoma who developed locoregional and distant metastases and received many chemotherapy. The mammary myoepithelial cell was named the cinderella of mammary cell biology in light of the earlier focus on the luminal cell.
Donath and coworkers 233 are usually credited with calling attention to this tumor in 1972. Unusual epithelialmyoepithelial carcinoma in palate case. The clear cells are often relatively large and usually predominate. 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.
Epithelial myoepithelial carcinoma is regarded as a lowgrademalignancy tumor and the treatment protocol involves wide surgical excision with secure clear margins. It is generally composed of variable proportions of two cell types. These may be positive for alpha smooth muscle actin and can contract and expel the secretions of exocrine glands. The importance of being a myoepithelial cell breast. Approximately 66% of these tumors occur in a part of the salivary gland, known as the parotid gland. Epithelial myoepithelial carcinoma emc was first described by danath et al. The tumor may also very rarely originate in minor salivary glands of the base of the tongue. 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. Epithelial myoepithelial carcinoma of the parotid gland author.
Pathology outlines epithelial myoepithelial carcinoma. Three additional cases of typical emc with transition to highgrade carcinoma are presented. 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. We now need to understand more about their origin and to reconsider their place in the complex process of mammary morphogenesis. They were incorporated into the world health organization classification of soft tissue tumors in 2002. A ct scan showed a fairly well defined heterogeneous lesion with smooth margins and slight enhancement. Epithelial myoepithelial carcinoma emc is a rare biphasic tumor accounting for less than 2% of all salivary gland malignancies. Here we present an exceptional case of myoepithelial carcinoma and myoepithelioma association. An inner layer of duct lining cells and an outer layer of clear cells, which typically form doublelayered ductlike structures. A 54yearold woman presented with an abnormal lung shadow on a. Ephitelial myoepithelial carcinoma, oral cavity, soft palate, immunohistochemistry. Epithelial myoepithelial carcinoma emc is a rare malignant salivary gland neoplasm that most commonly occurs in the parotid gland, but can also arise in. Primary salivary glandtype tumors of the lung are rare. As per literature, it involves the parotid gland most frequently and rarely, the minor salivary glands.
Although its contribution to benign and some malignant pathologies is recognised, it has been largely neglected in molecular and biological studies. The outer layer of myoepithelial cells merge with foci of spindled. Pdf epithelialmyoepithelial carcinoma emc is a rare biphasic tumor accounting for. Myoepithelial carcinoma of the breast treated with surgery. Epithelial myoepithelial carcinoma emc is extremely rare compared to other carcinomas, and is considered a low malignancy neoplasm. It is also known as a malignant myoepithelial tumor. Myoepithelial carcinomas exhibit a wide spectrum of cytomorphologic features and diverse clinical outcomes. Hunt, in diagnostic immunohistochemistry third edition, 2011. 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. Epithelialmyoepithelial carcinoma of the tracheaa rare. In the present report we describe a case of this unusual parotid gland neoplasia, radiologically simulating a benign lesion. Myoepithelial cells in pathology pubmed central pmc.
Epithelial myoepithelial carcinoma in the base of the tongue. Epithelial cancer definition of epithelial cancer by. We present a rare case of emc arising in the submandiblar gland. Pdf epithelialmyoepithelial carcinoma of the parotid gland. Myoepithelial carcinoma is an uncommon, malignant, soft tissue tumor of myoepithelial cells. Epithelialmyoepithelial carcinoma arising from the. Combined epithelialmyoepithelial lesions of the breast. 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. Potentially, the malignant elements can be a highgrade carcinoma, myoepithelial carcinoma, biphasic carcinosarcoma, or a pure sarcoma. E12, cytokeratin 14, p63, s100, calponin, smooth muscle actin, and musclespecific actin. Epithelial myoepithelial carcinoma emca is a rare salivary gland. Linkpage citation a 49yearold, hivpositive man with a cd4 count of 81 cellsmicrol presented with a 3week history of hiccupping. Myoepithelial carcinoma an overview sciencedirect topics. It is similar to salivary gland myoepithelial tumors showing same histological features morphology.
We strongly recommend that you talk with a trusted. 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. Oncocytic and apocrine epithelial myoepithelial carcinoma. Most of the reports emphasized diagnosis and pathology but not biological behavior and treatment. Primary epithelialmyoepithelial carcinoma of the lung. Although rare cases of highgrade emca have been reported, 3,58 most. Epithelialmyoepithelial carcinoma emc is regarded as a rare lowgrade malignant tumor of the salivary gland, accounting for 0. Epithelial myoepithelial carcinoma emca is a salivary tumor with dual cell population. We describe an unusual salivary neoplasm, an epithelial myoepithelial carcinoma emec of the parotid gland, that occurred in a 64yearold man. Simple excision should be avoided because epithelial myoepithelial carcinoma often has a destroyed epithelium 8, 10. It presents as a slowly growing, asymptomatic small size mass. Epithelial myoepithelial tumors of the lung are rare neoplasms whose biological behavior and clinical course still remain to be defined.
Due to rarity of its occurrence, histogenesis and clear cut therapeutic. Epithelialmyoepithelial carcinoma emc is a lowgrade, malignant biphasic salivary tumor of probable intercalated duct origin that comprises 1% to 2% of all salivary neoplasms. While the histologic, immunohistochemical, and even ultrastructural features have been well described, a definite diagnosis of mec based on cytology. 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. 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. Combined myoepithelial carcinoma and myoepithelioma in. 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 pleomorphic adenomas, the myoepithelial elements blend into this. Because of its rarity and unproven malignant potential, the optimal therapy for pemc has not been defined. Myoepithelial cell definition of myoepithelial cell by. They are found in the sweat glands, mammary glands, lacrimal glands, and salivary glands. Epithelial myoepithelial carcinomas are rare neoplasms usually arising from the salivary glands. They are predominantly found in the parotid gland and represent approximately 1% of salivary gland tumours. 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.
Epithelialmyoepithelial carcinoma lester dr thompson, md. The mammary myoepithelial cell cinderella or ugly sister. Notably, metastatic renal cell carcinoma is positive for both vimentin and cd10, but not s100 protein. Epithelial myoepithelial carcinoma emc is a tumour consisting of various proportions of two cell types which typically form ductlike structures. 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. Infiltrating myoepithelial carcinoma of the breast, a case.
Soft tissue myoepithelial carcinoma and myoepithelioma are rare entities, part of myoepithelial tumors. Epithelialmyoepithelial carcinoma an overview sciencedirect. Mr images showed a lesion with intermediate t1weighted signal intensity and a relatively high t2weighted signal intensity. 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. It is observed in a wide age category of children and adults. Myoepitheliomas are tumors arising from myoepithelial cells mainly or exclusively. 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. Myoepithelial carcinoma malignant myoepithelioma of the breast is a rare tumor, for which only a limited number of reports have been published. Epithelialmyoepithelial carcinoma emca is a salivary tumor with dual cell.
Epithelialmyoepithelial carcinoma genetic and rare. Epithelialmyoepithelial carcinoma with highgrade transformation of. The patient underwent biopsies demonstrating the diagnosis of epithelial myoepithelial carcinoma of the base of tongue and a pet scan. Epithelial myoepithelial carcinoma of head and neck region. Epithelial myoepithelial carcinoma emc of the salivary gland is a lowgrade carcinoma with a predilection for the parotid gland. Case epithelialmyoepithelial carcinoma of the report base.
Epithelialmyoepithelial carcinoma of salivary glands. Epithelialmyoepithelial carcinoma of the base of tongue. Epithelial myoepithelial carcinoma the tumor is composed of two cell types which form ductlike structures. Herein we report the morphological features of myoepithelial carcinoma in a 74yearold female clinically presenting with a parotid mass. 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. 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. Epithelialmyoepithelial carcinoma emc is a lowgrade, malignant biphasic. To the best of our knowledge, such an association has never been. Clear cell carcinoma consists exclusively of luminaltype epithelial cells, whereas myoepithelial carcinoma shows only cells that demonstrate myoepithelial differentiation. Mammary myoepithelial cells have recently been described as natural tumour suppressors. Despite several commonalities between these entities, various features distinguish.
Myoepithelial carcinoma and epithelialmyoepithelial. Epithelial myoepithelial carcinoma emc is a rare biphasic tumor of the salivary gland. A hybrid carcinoma of epithelialmyoepithelial carcinoma and adenoid cystic carcinoma in maxillary sinus article pdf available in journal of korean medical science 193. As a result of their morphologic heterogeneity, they can be confused easily with many tumours.
359 401 139 816 1446 237 156 348 41 1488 1188 170 449 1248 66 1248 1155 305 844 1509 1455 1461 571 1037 971 1449 1047 277 773 408 467 1345 1492 1318 17 731 632 611 676 637 998 1050 568