Epithelial-myoepithelial Carcinoma Research Articles

Pulmonary epithelial-myoepithelial carcinoma: a clinicopathologic and immunohistochemical study of 5 cases

Pulmonary epithelial-myoepithelial carcinomas are rare low-grade malignant neoplasms with histologic features similar to their salivary gland counterparts. We report a series of 5 cases and describe their clinical, morphological, and immunohistochemical features. The patients included 3 men and 2 women whose ages ranged from 38 to 56 years. All patients had obstructing endobronchial lesions that ranged from 2.5 to 5.0 cm in greatest dimension. The tumors were completely resected by simple lobectomy or pneumonectomy. In one patient, the tumor infiltrated perinodal soft tissue of a peribronchial lymph node with intranodal extension and metastasis within the same lymph node. The follow-up in these patients ranged from 4 to 12 months. The histologic appearance of these tumors varied, but all shared the common feature of a biphasic proliferation of epithelial (strong cytokeratin-positive; actin and S-100-negative) and myoepithelial (strong actin and S-100 and focal weak cytokeratin-positive) cells with formation of bilayered ductlike structures. The focal resemblance to other salivary gland-type tumors may cause diagnostic difficulties, particularly in small endobronchial biopsies. Although little is known about their biologic potential due to limited follow-up data, these tumors when in the lung clearly have the capacity to infiltrate and metastasize and therefore should be designated as epithelial-myoepithelial carcinoma. At present, it appears that treatment by complete surgical resection with negative margins alone is appropriate and adequate.

Synchronous bilateral epithelial–myoepithelial carcinoma of the parotid gland: case report and review of the literature

Synchronous bilateral malignancy in the parotid glands is extremely rare. The English literature reveals nine case reports. The most common synchronous bilateral malignancies are acinic cell carcinoma. Epithelial–myoepithelial carcinoma is an uncommon neoplasm comprising 1% of all salivary gland tumours. In this case report, we describe, to our best of knowledge, the first case of a patient with a synchronous bilateral epithelial–myoepithelial carcinoma of the parotid gland. The clinical histopathological and immunohistochemical peculiarities are elucidated. Imaging studies like ultrasonography are mandatory for both parotid glands and upper necks in the clinical presence of a unilateral parotid gland tumour.

Sebaceous Epithelial-Myoepithelial Carcinoma of the Salivary Gland: Clinicopathologic and Immunohistochemical Analysis of 6 Cases of a New Histologic Variant

Epithelial-myoepithelial carcinoma (EMC) of the salivary glands is an uncommon, low-grade malignant tumor. A recent report demonstrates sebaceous differentiation in this tumor even though its significance has never been documented as a precise histologic variant. Six cases of EMC exhibiting sebaceous differentiation (sebaceous EMC) of the parotid gland were analyzed for their clinicopathologic features and immunohistochemical characteristics. In addition, primary salivary sebaceous carcinomas were also examined for comparison. In our series, the incidence of sebaceous EMC was 0.2% among 3012 cases of parotid gland tumors and 14.3% of all EMC cases. The 6 patients comprised 2 men and 4 women, age ranging from 77 to 93 years (mean, 83.7 y). Neither cervical lymph node nor distant organ metastases were found in any cases of sebaceous EMC and no patients died of disease, though local recurrences developed in 1 patient. Conversely, cervical lymph node metastasis was detected in 2 of 3 patients with sebaceous carcinoma, 1 of whom died of disease at 12 months. Histologically, all 6 tumors had an area of sebaceous differentiation admixed with features of bilayered ductal structures typical of EMC. A component of sebaceous differentiation was distributed diffusely in 4 tumors and focally in 2. Cytologic atypia of sebaceous EMCs was lesser than that of sebaceous carcinomas. Immunohistochemically, putative myoepithelial markers such as alpha-smooth muscle actin, calponin, p63, cytokeratin 14, S-100 protein, and vimentin were highly expressed in sebaceous EMC. However, the expression of the latter 4 markers was also observed in primary sebaceous carcinomas, whereas these tumors were all negative for alpha-smooth muscle actin and calponin. Positive immunoreactivity for epithelial membrane antigen, adipophilin, and perilipin confirmed sebaceous differentiation in EMC. These results indicate that sebaceous EMC is a low-grade malignancy, similar to conventional EMC. Our data also suggest that immunohistochemical examination of specific myoepithelial markers is helpful in distinguishing sebaceous EMC from sebaceous carcinoma, which may occasionally be associated with an aggressive clinical course.

Oncocytic epithelial-myoepithelial carcinoma: an evolving new variant with comparative immunohistochemistry

Oncocytic epithelial-myoepithelial carcinoma: an evolving new variant with comparative immunohistochemistry

Dedifferentiated epithelial-myoepithelial carcinoma of the parotid gland: a rare case report of immunohistochemical analysis and review of the literature

Dedifferentiation of salivary gland neoplasms is a rare event, unlike bone and soft part sarcomas, which was first described by Stanley et al. in 1988. An additional case of dedifferentiated epithelial-myoepithelial carcinoma (EMC) is reported here. The patient was a 70-year-old Japanese man who requested examination of the rapid growth of a mass in the right parotid region, which he had first noticed 25 years previously. Clinical examination showed an ill-circumscribed, 6.8 x 4.7 x 7.0-cm lesion. Histologically, most parts of the lesion were high-grade carcinoma (HGC) with sheetlike and nestlike growth of markedly atypical cells and comedonecrosis, whereas the minor part consisted of typical EMC. The outer clear cells of EMC were positive for alpha-smooth muscle actin (ASMA), p63, cytokeratin (CK) 14, and vimentin, and the inner ductal cells of EMC were positive for CKs and epithelial membrane antigen. HGC was negative for ASMA, CK14, and vimentin, but diffusely positive for p53 protein and cyclin D1. The Ki-67 labeling index of EMC was 11.5%, whereas that of HGC was 67.1%. These findings and a review of literature indicate that HGC arose from preexisting EMC, and this phenomenon is the dedifferentiation of EMC. Dedifferentiated EMC is extremely rare.

Clear cell predominant epithelial-myoepithelial carcinoma of the hard palate — role of immunohistochemistry

Epithelial-myoepithelial carcinoma (EMC) arising from the minor salivary gland of the hard palate is very rare. A 34-year-old lady presented with a nodular swelling in the hard palate. Histopathologically, a diagnosis of clear cell predominant EMC was suggested. Immunohistochemistry demonstrated the biphasic nature of the tumor and confirmed the diagnosis of EMC.

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

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.

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

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.

Bilateral Epithelial-Myoepithelial Carcinoma: An Extraordinary Tumor of the Paranasal Sinuses

A 54-year-old woman presented with a 6-month history of swelling on the right side of her face. On physical examination she was found to have a huge mass in the right nasal cavity. Magnetic resonance images of the paranasal sinuses revealed a soft tissue mass in the right maxillary sinus and a mass was also seen in the left maxillary sinus. On histopathologic examination, the tumor had a lobular structure with infiltrating margins. Two cell types, an outer layer of myoepithelial and an inner layer of duct-like cells, were found. On immunohistochemical examination, myoepithelial cells stained positively for calponin, p63, GFAP, S-100 protein, alpha-smooth muscle actin cytokeratin-14. The tumors were resected completely and no recurrence or metastasis was found 30 months after surgery. We describe here an unusual case of epithelial-myoepithelial carcinoma (EMC) arising from the paranasal sinuses. This is the first case report in the literature describing bilateral EMC in the maxillary sinuses.

Epithelial-myoepithelial parotid carcinoma after kidney transplantation

The occurrence of a second malignant neoplasm (SMN) in patients who have been submitted to kidney transplantation is increasing and causes concern; parotid carcinoma is rarely reported after transplantation and may be related to long-term chemotherapy.Salivary gland carcinomas displaying exclusively myoepithelial differentiation—myoepithelial carcinomas (EMC) are rare, being less than 1% of all salivary gland tumours. EMC arises most commonly in the parotid gland and usually occurs in women. Their histopathologic features, immunohistochemical profile and clinical behaviour remain controversial.

Epithelial-myoepithelial carcinoma arising in pleomorphic adenoma of palate

Epithelial-myoepithelial carcinoma arising in pleomorphic adenoma of palate

Epithelial–myoepithelial carcinoma arising in the nasal cavity

This study documents a case of an epithelial–myoepithelial carcinoma (EMC) in the left nasal cavity. A 70-year-old woman who presented with recurrent epistaxis of the left nasal nostril of 3 months duration was found to have a polypoid tumor in the left nasal cavity. A computed tomography (CT) scan revealed a tumor to occupy the left inferior and middle nasal cavity which had destroyed the inferior nasal turbinate, and a horizontal scan showed the tumor to occupy the middle and posterior nasal cavity. Since the tumor was connected to the lateral wall of the left nasal cavity with a narrow stalk, the tumor was excised by peeling the mucosa from the wall of the left nasal cavity. Based on the histological and immunohistochemical findings, the tumor was diagnosed to be an EMC. The follow-up at 12 months after the operation showed no evidence of recurrence.

Carcinome épithélial–myoépithélial de la cavité nasale

Objectives Epithelial–myoepithelial carcinoma is a rare tumor of the salivary glands with an incidence of less than 1%. Most cases arise in the parotid gland. Extraoral location is exceptional. The purpose of this study was to describe a case with nasal location, which is exceptionally reported in the literature. Materials and methods We report a case of epithelial–myoepithelial carcinoma arising in the nasal cavity of a 54-year-old woman. Results The woman presented with right recurrent epistaxis and on nasal endoscopic examination was found to have a polypoid tumor in the right nasal cavity. The CT-scan demonstrated a soft tissue mass without extension to the rhinopharynx or bony destruction. Histopathologic examination revealed the tumor to consist of a mixture of a solid, tubular, and trabecular structures with a double-layered arrangement of inner eosinophilic cells and outer clear cells. Dual differentiation toward myoepithelial and epithelial cells was confirmed immunohistochemically. Conclusion The occurrence of epithelial–myoepithelial carcinoma in the nasal cavity is possible. In published cases, no recurrence or metastasis has been reported in this location.

Epithelial-myoepithelial carcinoma of the parotid gland, unusual malignancy radiologically simulating a benign lesion: case report

BackgroundUltrasound (US), Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) are widely used in the clinical diagnosis of parotid gland tumors and their efficacy in identifying benign lesions is well documented. However, problems arise when facing some malignant lesions. Only few cases of salivary gland low grade malignant tumors have been previously reported in the literature complete with the radiological features.Case presentationWe here describe a case of epithelial-myoepithelial carcinoma (EMC) of the parotid gland, a low grade malignant tumor, with spread to an intraparotid lymph node and with CT and MRI findings mimicking a benign lesion.ConclusionAll the images revealed sharply outlined profiles and a homogeneous enhancement of the nodule, suggesting a benign tumor and demonstrating that a radiological evaluation of the lesion alone may be unsatisfactory and misleading in the diagnosis of salivary gland tumours, especially in the case of low grade malignant tumors, such as EMC.

Update on Sinonasal Adenocarcinoma: Classification and Advances in Immunophenotype and Molecular Genetic Make-Up

Adenocarcinomas of various types comprise 10% to 20% of all primary malignant neoplasms of the nasal cavity and paranasal sinuses [1]. Salivary Gland-Type Adenocarcinomas Adenocarcinomas of salivary-gland type are uncommon constituting 5–10% of sinonasal adenocarcinomas. They are thought to originate from seromucus glands of the nasal cavity and paranasal sinuses as well as the surface epithelium. Histologically, these carcinomas are similar to those originating from major and minor salivary glands [2]. The most common type is adenoid cystic carcinoma (Fig. 1) usually occurring in the maxillary sinus and nasal cavity [3]. Long-term prognosis is poor and patients usually die due to local spread with no metastasis. Adenocarcinoma Not Otherwise Specified may exhibit many high-grade, poorly differentiated growth patterns, and should be considered as a diagnostic entity for many high-grade salivary gland-type adenocarcinomas of the sinonasal tract. These tumors have a poor prognosis [1, 4]. Fig. 1 Adenoid cystic carcinoma of nasal cavity. Classic cribriform and cystic pattern with bland nuclear appearance. Nasal surface epithelium is at the top left corner. H-E-stain x250 Other types of salivary adenocarcinomas are rare in the sinonasal tract but cases of acinic cell carcinoma, mucoepidermoid carcinoma (Fig. 2) [5], epithelial-myoepithelial carcinoma, basal cell adenocarcinoma, salivary duct carcinoma, polymorphous low-grade adenocarcinoma (terminal tubulus adenocarcinoma) [6], and carcinoma-ex-pleomorphic adenoma have been reported. The possibility of specific types of salivary gland-type adenocarcinomas should be taken into account when considering the diagnosis of a sinonasal adenocarcinoma. Table 1 lists the occurrence of different types of salivary gland-type tumors in the sinonasal tract. Fig. 2 Low-grade mucoepidermoid carcinoma of nasal cavity. Islands of intermediate cells contain mucous cysts and few epidermoid cells. Nasal surface epithelium at left. H-E stain x250 Table 1 Occurrence of sinonasal tumors of salivary gland type (modified from reference #5) Non-Salivary Gland-Type Adenocarcinomas The WHO classification of non-salivary gland-type adenocarcinomas [7] follows a classical distinction into intestinal-type adenocarcinoma and non-intestinal-type adenocarcinoma. WHO further subdivides the latter into low-grade and high-grade carcinoma [7]. Another approach first pools the various types of non-salivary gland-type sinonasal adenocarcinomas together and distinguishes them into six histological groups: low-grade, papillary, colonic, solid, mucinous, and mixed types [8]. In this presentation, the WHO classification of 2005 is used.

Advances in salivary gland pathology

This review summarizes the new findings on salivary gland pathology under the following categories: immunohistochemistry; molecular genetics; newly recognized tumour types; known tumour entities with new findings; and progression of salivary gland tumours. In the application of immunohistochemistry, CD117 can aid in highlighting the luminal cell component of various salivary gland tumours, whereas p63 or maspin can aid in highlighting the abluminal cell component. A high Ki67 index remains the most useful marker to predict adverse outcome in salivary gland carcinoma. Specific chromosomal translocations are recognized in pleomorphic adenoma (with translocation involving PLGA1 or HMGA2 gene) and mucoepidermoid carcinoma (with MECT1-MAML2 gene fusion). Newly recognized entities include: sclerosing polycystic adenosis (with recent molecular evidence supporting its neoplastic nature), sclerosing mucoepidermoid carcinoma with eosinophilia, keratocystoma, adenoma with additional stromal component (lymphadenoma, lipoadenoma and adenofibroma), cribriform adenocarcinoma of the tongue and signet ring adenocarcinoma of minor salivary gland. Known tumour entities with new findings include: salivary duct carcinoma (with newly recognized mucinous, micropapillary and sarcomatoid variants), intraductal carcinoma (with controversies in terminology), mucoepidermoid carcinoma (with newly proposed grading parameters and oncocytic variant), epithelial-myoepithelial carcinoma (with newly recognized morphological variants), small cell carcinoma (with most cases being related to Merkel cell carcinoma), extranodal marginal zone B-cell lymphoma (with specific chromosomal translocation) and chronic sclerosing sialadenitis (being a component of IgG4-related sclerosing disease). Progression of salivary gland tumours can take the form of malignant transformation of a benign tumour, progression from low-grade to high-grade carcinoma, dedifferentiation, or stromal invasion of an in situ carcinoma.

Recurrent Bronchial Epithelial-Myoepithelial Carcinoma After Local Therapy

We present a rare case of recurrent multiple lesions of bronchial epithelial-myoepithelial carcinoma in a 74-year-old man treated with local resection. Two cellular types were found: epithelial cells and myoepithelial cells. The patient remains asymptomatic at 4-years of follow-up, supporting the fact that epithelial-myoepithelial carcinoma is a tumor of low-grade malignancy.

Bronchial Epithelial-Myoepithelial Carcinoma

An epithelial-myoepithelial tumor is an extremely rare pulmonary neoplasm. Only 21 cases have been reported to date. This report presents a case of left main bronchial epithelial-myoepithelial carcinoma in a 43-year-old woman. No mediastinal lymph nodes were enlarged in computed tomographic scan, and no tumor growth was noted beyond the bronchial cartilage layer by endobronchial ultrasound imaging. This report highlights the usefulness of endobronchial ultrasound imaging for determining the depth of tumor invasion and choosing an alternative approach to surgical resection.

Epithelial myoepithelial carcinoma: A case report of the rare neoplasm

Epithelial myoepithelial carcinoma: A case report of the rare neoplasm

Epithelial-Myoepithelial Carcinoma: A Review of the Clinicopathologic Spectrum and Immunophenotypic Characteristics in 61 Tumors of the Salivary Glands and Upper Aerodigestive Tract

To further define the clinicopathologic spectrum of epithelial-myoepithelial carcinoma (EMCa), we report the gross, histologic, and immunophenotypic characteristics of 61 tumors seen within a 30-year-period. The mean age at presentation was 60.9 years, with a female predominance (1.5:1). The most common sites were parotid (62.1%), sinonasal mucoserous glands (10.3%), palate (8.6%), and submandibular (8.6%). Most EMCas showed a characteristic nodular/multinodular growth pattern and classic biphasic tubular histology. However, new morphologies in EMCa such as ancient change (8.2%), "Verocay"-like change (3.3%), and sebaceous differentiation (13.1%) were noted. Specific histologic variants were dedifferentiated EMCa (3.3%), oncocytic EMCa (8.2%), EMCa ex pleomorphic adenoma (1.6%), double-clear EMCa (3.3%), and EMCa with myoepithelial anaplasia (3.3%). All cytokeratin cocktails selectively highlighted the epithelial component well. Of the myoepithelial markers, p63, smooth muscle actin and vimentin performed best. Bcl-2 and c-kit were frequently positive (66.7% and 69.2%, respectively). p53 was highly expressed only in 1 dedifferentiated EMCa. The recurrence rate was 36.3% (median disease-free survival 11.34 y), but death was rare with 5-year and 10-year disease-specific survivals of 93.5% and 81.8%, respectively. The most important univariate predictors of recurrence were margin status (log rank P=0.006), angiolymphatic invasion (P=0.002), tumor necrosis (P=0.004), and myoepithelial anaplasia (P=0.038). Thus, EMCa is generally a low-grade tumor with a broader morphologic spectrum than previously thought, with several key features predictive of recurrence. Immunohistochemistry can aid diagnosis by highlighting the biphasic nature of the tumor.