Apr 18, 2020 epithelial myoepithelial carcinoma is a cancer of the parotid salivary duct, with the most common occurrence in elderly people. However, epithelialmyoepithelial carcinoma with highgrade transformation emc with hgt is extremely rare, therefore it is easily to be inappropriately diagnosed and treated. Epithelialmyoepithelial carcinoma of the tongue base. Infiltrating myoepithelial carcinoma of the breast, a case. Wed like to understand how you use our websites in order to improve them. Epithelialmyoepithelial carcinoma of external auditory canal. Abstract we encountered two cases of epithelial myoepithelial carcinoma emc, a rare lowgrade malignant tumor of the parotid gland. As per literature, it involves the parotid gland most frequently and rarely, the minor salivary glands. Because of its rarity and unproven malignant potential, the optimal therapy for pemc has not been defined. Primary myoepithelial carcinoma of the lung is a very rare tumor arising from the salivary glands of the respiratory epithelium. Click on the link to view a sample search on this topic. Epithelialmyoepithelial carcinoma is a rare neoplasm accounting for approximately 1% of all salivary gland neoplasms.
Pdf epithelialmyoepithelial carcinoma with highgrade. Epithelialmyoepithelial carcinoma is a cancer of the parotid salivary duct, with the most common occurrence in elderly people. This tumour may be particularly locally aggressive, but its clinical and biological features are not yet fully understood. Epithelialmyoepithelial carcinoma emc is a rare salivary gland malignancy associated with an overall good prognosis. Epithelialmyoepithelial carcinoma of high grade transformation. Epithelialmyoepithelial carcinoma of external auditory. Epithelialmyoepithelial carcinoma emc is a tumour consisting of various proportions of two cell types which typically form ductlike structures.
Of all intrinsic lacrimal gland masses, 28% are epithelial neoplasms. The breast myoepithelial cell is the cinderella of mammary biology. Epithelialmyoepithelial carcinoma emc is a lowgrade, malignant biphasic salivary tumor of intercalated duct origin that comprises 12% of all salivary neoplasms. Myoepithelial carcinoma with contralateral invasive. An analogous lung tumour exists see epithelial myoepithelial carcinoma of lung. Primary lung myoepithelial carcinomas are rare neoplasms arising from the salivary glands of the respiratory epithelium. Myoepithelial carcinoma and epithelialmyoepithelial. Pleuromediastinal epithelialmyoepithelial carcinomas. Epithelialmyoepithelial carcinoma of salivary gland.
We present an adenocarcinoma tumor of the parotid gland that was dedifferentiated from a lowgrade epithelial myoepithelial carcinoma and was followed up for 3 years. 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. Epithelialmyoepithelial carcinoma with highgrade transform. The p27kip1 protein plays a fundamental role in the development of these neoplasms. Epithelialmyoepithelial carcinoma is an rare tumour occurring in the salivary glands, most commonly in the parotid gland, with a reported incidence of between 0. Linkpage citation a 49yearold, hivpositive man with a cd4 count of 81 cellsmicrol presented with a 3week history of hiccupping. 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. Our patient, a 46yearold female, presented with a left parotid mass of 20year duration. These tumours have been named in a lot of different ways, including adenomyoepithelioma, epithelialmyoepithelial tumour, epithelialmyoepithelial carcinoma or epithelialmyoepithelial tumour of uncertain malignant potential. Epithelialmyoepithelial carcinoma is an uncommon neoplasm comprising 1% of all salivary gland tumours.
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. They are found in the sweat glands, mammary glands, lacrimal glands, and salivary glands. Histologically, emc is characterized by two types of cells arranged in welldefined tubules. Epithelialmyoepithelial carcinoma emc is a lowgrade, malignant biphasic salivary tumor of probable intercalated duct origin that comprises 1% to 2% of all salivary neoplasms. Breast adenomyoepithelioma is an unusual tumor characterized by a biphasic proliferation of epithelial and myoepithelial cells. Pulmonary epithelial myoepithelial carcinoma pemc is a rare subset of salivary glandtype tumors of the lung. Report of a case makoto toida, dds, dmsc, and kuniyasu shimokawa, md, dmsci epithelialmyoepithelial carcinoma emc is a rare salivary gland tumor that was established as a distinct diagnostic entity by the. A 54yearold woman presented with an abnormal lung shadow on a. Abstractadenomyoepithelioma ame is a rare benign tumor composed of myoepithelial cells. Objective of this study is to highlight the importance of considering clinical and radiological findings in parotid tumors before operating tumors with high suspicion of malignancy though reported as benign on histopathology. Three additional cases of typical emc with transition to highgrade carcinoma are presented.
We strongly recommend that you talk with a trusted. 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. Pdf epithelialmyoepithelial carcinoma of the parotid gland. Mc usually presents as a slowgrowing painless mass arising in the parotid gland, but may involve other salivary glands. Layfield, epithelialmyoepithelial cell carcinoma of the parotid gland. Epithelial myoepithelial carcinoma of the parotid gland author. This article is from journal of the korean surgical society, volume 81. Epithelialmyoepithelial carcinoma emc is a rare neoplasm, first described in 1972 by donath et al. 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. These may be positive for alpha smooth muscle actin and can contract and expel the secretions of exocrine glands. In 1991, myoepithelial cell carcinoma was added to the second edition of the world health organizations classification of malignant salivary gland tumours. In this case report, we describe, to our best of knowledge, the first case of a patient with a synchronous bilateral epithelialmyoepithelial carcinoma of the parotid gland. Hunt, in diagnostic immunohistochemistry third edition, 2011.
Most breast adenomyoepitheliomas are considered to be benign or to have a lowgrade malignant potential, characterized by propensity for local recurrence. The treatment for metastatic myoepithelial carcinoma usually begins with surgery to remove the main tumor. Epithelial cancer definition of epithelial cancer by. We report a rare case of epithelial myoepithelial carcinoma, which developed in the subglottic region. Given the rare occurrences and reports of these tumors, appropriate recommendations for resection are difficult to formulate. Myoepithelial carcinoma is an uncommon, malignant, soft tissue tumor of myoepithelial cells. Approximately 66% of these tumors occur in a part of the salivary gland, known as the parotid gland. 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. Abstracta 45yearold female patient visited the clinic due to ear fullness. It is generally composed of variable proportions of two cell types. Jan 20, 2012 the objective of the study is to document the clinical, histopathological and immunological findings of epithelial myoepithelial carcinoma emec which is an uncommon neoplasm. Myoepithelial carcinoma of the lung european journal of.
Myoepithelial cells are a dynamic barrier to epithelial. It is observed in a wide age category of children and adults. An inner layer of duct lining cells and an outer layer of clear cells, which typically form doublelayered ductlike structures. The hallmark of the histology of epithelial myoepithelial carcinoma emc is the presence of a. Characterized by biphasic proliferation of ductlike epithelial cells with surrounding myoepithelial cells terminology previous designations include adenomyoepithelioma, epithelial myoepithelial tumor, epithelial myoepithelial tumor of unknown malignant potential, pneumocytic adenomyoepithelioma hum pathol 2009. An analogous lung tumour exists see epithelialmyoepithelial carcinoma of lung. Pdf epithelialmyoepithelial carcinoma of parotid gland. The normal mammary gland is organized around a branched ductal network arranged in an epithelial bilayer, with an inner luminal and outer myoepithelial cell layer adriance et al. Epithelialmyoepithelial carcinoma with high grade transform. A ct scan showed a fairly well defined heterogeneous lesion with smooth margins and slight enhancement. Mc may arise from preexisting benign lesions, such as. Two cases of epithelialmyoepithelial carcinoma are presented, one arising in the pleura and the other in the anterior mediastinum.
E12, cytokeratin 14, p63, s100, calponin, smooth muscle actin, and musclespecific actin. Primary myoepithelial carcinoma of palate world journal of. The background may contain abundant isolated clear cells and naked nuclei, potentially masking the biphasic pattern of the tumor. 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. For language access assistance, contact the ncats public information. Dedifferentiation is defined as highgrade malignant tumor development out of a lowgrade malignant tumor. The pertinent literature is restricted to a few pathological reports, and hardly any clinical examinations have been performed. Sep 10, 2018 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. Salivary gland myoepithelial carcinoma springerlink. The patient underwent biopsies demonstrating the diagnosis of epithelial myoepithelial carcinoma of the base of tongue and a pet. Although its contribution to benign and some malignant pathologies is recognised, it has been largely neglected in molecular and biological studies. They are predominantly found in the parotid gland and represent approximately 1% of salivary gland tumours. Epithelial myoepithelial carcinoma is regarded as a lowgrademalignancy tumor and the treatment protocol involves wide surgical excision with secure clear margins. Ris zotero endnote bibtex medlars procite refworks reference manager mendeley.
Epithelial myoepithelial carcinoma of the tracheaa rare. This article is from korean journal of audiology, volume 16. Because of the rarity of this tumor, we describe one case of epithelial myoepithelial carcinoma arising in the bronchus intermedius and presenting as an endobronchial mass. Epithelial myoepithelial carcinoma of head and neck region. Epithelial myoepithelial carcinoma emca is a lowgrade malignant tumour. There is limited evidence about the usefulness of chemotherapy in treating myoepithelial carcinoma, and there are no standard treatment guidelines. We report a rare case of epithelialmyoepithelial carcinoma, which developed in the subglottic region. Epithelialmyoepithelial carcinoma an overview sciencedirect.
Myoepitheliomas are tumors arising from myoepithelial cells mainly or exclusively. Donath and coworkers 233 are usually credited with calling attention to this tumor in 1972. Pathology outlines epithelial myoepithelial carcinoma. We report herein on a case of carcinoma ex pleomorphic adenoma with epithelialmyoepithelial carcinoma of the minor salivary gland. Typical microscopic findings involve the dual composition of an inner eosinophilic ductal cell layer and an outer clear myoepithelial cell layer. Though the name for this tumor was coined by donath et al. Nov 18, 2016 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. They are predominantly found in the parotid gland 1 and represent approximately 1% of salivary gland tumours. Epithelialmyoepithelial carcinoma emc is a rare biphasic tumor of the salivary gland.
There is limited evidence in the literature on their prognosis in the base of the tongue but other cases have resolved without recurrence. Epithelialmyoepithelial carcinoma of the parotid gland. Epithelial myoepithelial carcinoma of parotid gland request pdf. Mr images showed a lesion with intermediate t1weighted signal intensity and a relatively high t2weighted signal intensity. Ephitelial myoepithelial carcinoma, oral cavity, soft palate, immunohistochemistry. Epithelial myoepithelial carcinoma is a rare tumor which makes up about 0. Regarding the genetic study of myoepithelial cell carcinoma, the study by antonescu et al, which included 66 cases, showed that the ews rnabinding protein 1 ewsr1 gene rearrangement was a common event in myoepithelial tumours arising outside of salivary glands, as found in 45% of cases. Epithelial myoepithelial carcinoma is an extremely rare disease that usually occurs in the parotid gland but can occur in a variety of sites such as the nasal cavity, paranasal sinus, and base of the tongue.
Epithelialmyoepithelial carcinoma genetic and rare. Epithelialmyoepithelial carcinoma is an extremely rare disease that usually occurs in the parotid gland but can occur in a variety of sites such as the nasal cavity, paranasal sinus, and base of the tongue. The mammary myoepithelial cell cinderella or ugly sister. Pulmonary epithelialmyoepithelial carcinoma pemc is a rare subset of salivary glandtype tumors of the lung. Epithelialmyoepithelial carcinoma is regarded as a lowgrademalignancy tumor and the treatment protocol involves wide surgical excision with secure clear margins. Emc accounts for 1% of epithelial salivary gland tumors, with the majority of cases of emc arising in the parotid gland. Oct 30, 20 breast adenomyoepithelioma is an unusual tumor characterized by a biphasic proliferation of epithelial and myoepithelial cells. 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.
Pubmed is a searchable database of medical literature and lists journal articles that discuss epithelialmyoepithelial carcinoma. The presence of cancer cells beyond the myoepithelium defines invasive breast cancer, yet the role of the myoepithelium during invasion remains unclear. A hybrid carcinoma of epithelialmyoepithelial carcinoma and adenoid cystic carcinoma in maxillary sinus. It is a hybrid tumour, composed of two different tumour entities, each of which conforms to a defined tumour category 4, 5 and based upon the salivary gland register at the. Radiation therapy can be used to reduce the chance that the tumor could return in the same location. It is similar to salivary gland myoepithelial tumors showing same histological features morphology. Simple excision should be avoided because epithelialmyoepithelial carcinoma often has a destroyed epithelium 8, 10. Here we present the cytologic findings from a case of infiltrating myoepithelial carcinoma of the breast in a 52yearold female and. Primary epithelialmyoepithelial carcinoma of the lacrimal. In the present report we describe a case of this unusual parotid gland neoplasia, radiologically simulating a benign lesion.
Epithelialmyoepithelial carcinoma of the base of tongue with. Donath and coworkers are usually accredited with calling attention to this tumor in 1972. Myoepithelial cells in these cases constitute the basal cell layer of an epithelium that harbors the epithelial progenitor. Primary epithelial myoepithelial carcinoma of lung. Both patients were men aged 25 and 68 years, respectively, who had chest pain and dyspnea. The ewsr1 gene is located on chromosome band 22q12 and. Epithelialmyoepithelial carcinoma in the base of the tongue.
Primary myoepithelial carcinoma of the lung is an extremely rare malignant tumor, probably originating from the bronchial minor salivary gland, with only 8 patients reported in the. Although rare cases of highgrade emca have been reported, 3,58 most. It is also known as a malignant myoepithelial tumor. Epithelialmyoepithelial carcinoma with highgrade transformation of. A case report and comparison of cytologic features with other stromal, epithelial and myoepithelial cell containing lesions of the salivary glands, diagnostic cytopathology, vol. Although classified as lowgrade neoplasms, these tumors have a significant rate of recurrence and distant metastasis. Presence of myoepithelial cells in a hyperplastic tissue proves the benignity of the gland and, when absent, indicates cancer. Ephitelialmyoepithelial carcinoma, oral cavity, soft palate, immunohistochemistry. In the case of wound healing, myoepithelial cells reactively proliferate. Epithelial myoepithelial carcinoma is a rare malignant tumor that ismore commonly encountered in salivary glands and comprises 1% of all salivarygland tumors. The mammary epithelium is composed of an inner luminal and surrounding myoepithelial cell layer. 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.
Synchronous bilateral epithelialmyoepithelial carcinoma. Fineneedle aspiration of epithelialmyoepithelial carcinoma of the. Epithelial myoepithelial carcinoma emc is a rare and lowgrade malignant neoplasm that was first described by donath et al in 1972. The few cases that have surfaced firmly document the histopathology of this tumor, but its cytologic characteristics seemingly have been described in only one other report. Dear sir, myoepithelial cell carcinoma, first described by stromeyer et al in 1975, is defined as a malignant carcinoma that primarily arises from the parotid gland. If you have problems viewing pdf files, download the latest version of adobe reader. A 78yearold korean woman visited our hospital complaining of hoarseness, which had. Epithelial myoepithelial carcinoma emc is regarded as a rare lowgrade malignant tumor of the salivary gland, accounting for 0. However, epithelial myoepithelial carcinoma with highgrade transformation emc with hgt is extremely rare, therefore it is easily to be inappropriately diagnosed and. Simple excision should be avoided because epithelial myoepithelial carcinoma often has a destroyed epithelium 8, 10. Myoepithelial cells and the basement membrane separate luminal epithelial cells from the stroma. Salivary gland myoepithelial carcinoma mc or malignant myoepithelioma is a rare entity.
Other localized forms of epithelial carcinoma include acinar adenocarcinoma, which affects the prostate. 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. Primary epithelial myoepithelial carcinoma of lung is a rare entity and is thought to arise from the submucosal bronchial glands distributed throughout the lower respiratory tract. Introduction epithelial myoepithelial carcinoma is an rare tumour occurring in the salivary glands, most commonly in the parotid gland, with a reported incidence of between 0. 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 extremely rare compared to other carcinomas, and is considered a low malignancy neoplasm.
Malignant changes arising in this lesion are extremely rare and may involve one or both cellular components. Epithelial myoepithelial carcinomas are rare neoplasms usually arising from the salivary glands. There is an inner layer of ductlining cells and an outer layer of clear myoepithelial cells 52. Epithelialmyoepithelial carcinoma emc is regarded as a rare lowgrade malignant tumor of the salivary gland, accounting for 0.
Epithelial myoepithelial carcinoma emc and myoepithelial carcinoma mc are. Epithelial myoepithelial carcinoma emca is a salivary tumor with dual cell population. Introduction epithelialmyoepithelial 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. Case study of a parotid gland adenocarcinoma dedifferentiated. Pubmed is a searchable database of medical literature and lists journal articles that discuss epithelial myoepithelial carcinoma. Infiltrating myoepithelial carcinoma remains a rarely encountered lesion of the breast. Epithelialmyoepithelial carcinoma emc is a rare type of malignant tumor of salivary gland accounting for less. Unusual epithelialmyoepithelial carcinoma in palate case. We describe an unusual salivary neoplasm, an epithelialmyoepithelial carcinoma emec of the parotid gland, that occurred in a 64yearold man. Epithelialmyoepithelial 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 parotid gland author. Epithelialmyoepithelial carcinoma arising from the. One week after the onset of the hiccups, he had been seen at an urgent care center. Myoepitheliomas mostly occur in salivary glands, as well as in breast, skin, and lung.
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