Oncocytic variant of epithelial-myoepithelial carcinoma of the parotid gland: a rare case report and review of the literature

Oncocytic variants of epithelial-myoepithelial carcinoma are exceptionally rare and often difficult to distinguish from other oncocytic or biphasic salivary tumors. This case underscores the diagnostic challenge and highlights the importance of detailed histopathological and immunohistochemical evaluation for accurate classification. A 71-year-old woman presented with a slowly enlarging mass in the superficial lobe of the parotid gland. Imaging demonstrated a well-circumscribed lesion. The patient underwent superficial parotidectomy. Histopathological examination revealed biphasic epithelial and myoepithelial cell differentiation with prominent oncocytic changes. Immunohistochemistry showed CK7 positivity in the ductal epithelial component and p63 and calponin positivity in the myoepithelial layer, confirming the diagnosis of oncocytic variant EMCa. Oncocytic EMCa is a particularly rare subtype and may resemble intraductal carcinoma, pleomorphic adenoma, or other oncocytic neoplasms. Accurate diagnosis requires combined evaluation of morphology and specific immunohistochemical markers. The distinction is critical because oncocytic EMCa generally demonstrates indolent behavior but requires complete excision and close follow-up. This case underscores the value of appropriate IHC panel selection and correct classification to guide management. This case illustrates the diagnostic challenges posed by oncocytic EMCa and emphasizes that careful integration of morphology with targeted immunohistochemistry is essential for accurate diagnosis and clinical decision-making.

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Publication Details

Journal
Journal of Rare Diseases
Published
2026-03-12
DOI
https://doi.org/10.1007/s44162-026-00174-6
Primary Topic
Salivary Gland Tumors Diagnosis and Treatment
Type
article
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article

Oncocytic variant of epithelial-myoepithelial carcinoma of the parotid gland: a rare case report and review of the literature

Murat Topak, Gamzenur Erdoğan, Esin Yiğitbaşı Kılıç, Mesut Öztürk et al.
Journal of Rare Diseases
Salivary Gland Tumors Diagnosis and Treatment
article

Oncocytic variant of epithelial-myoepithelial carcinoma of the parotid gland: a rare case report and review of the literature

Murat Topak, Gamzenur Erdoğan, Esin Yiğitbaşı Kılıç, Mesut Öztürk, Umutcan Musaoğlu, Semra Külekçi Öztürk
article en

Abstract

Oncocytic variants of epithelial-myoepithelial carcinoma are exceptionally rare and often difficult to distinguish from other oncocytic or biphasic salivary tumors. This case underscores the diagnostic challenge and highlights the importance of detailed histopathological and immunohistochemical evaluation for accurate classification. A 71-year-old woman presented with a slowly enlarging mass in the superficial lobe of the parotid gland. Imaging demonstrated a well-circumscribed lesion. The patient underwent superficial parotidectomy. Histopathological examination revealed biphasic epithelial and myoepithelial cell differentiation with prominent oncocytic changes. Immunohistochemistry showed CK7 positivity in the ductal epithelial component and p63 and calponin positivity in the myoepithelial layer, confirming the diagnosis of oncocytic variant EMCa. Oncocytic EMCa is a particularly rare subtype and may resemble intraductal carcinoma, pleomorphic adenoma, or other oncocytic neoplasms. Accurate diagnosis requires combined evaluation of morphology and specific immunohistochemical markers. The distinction is critical because oncocytic EMCa generally demonstrates indolent behavior but requires complete excision and close follow-up. This case underscores the value of appropriate IHC panel selection and correct classification to guide management. This case illustrates the diagnostic challenges posed by oncocytic EMCa and emphasizes that careful integration of morphology with targeted immunohistochemistry is essential for accurate diagnosis and clinical decision-making.

Journal of Rare DiseasesVol. 5(1)
Fatih Sultan Mehmet Eğitim Ve Araştırma Hastanesi (TR)
Peace, Justice and strong institutions
Openalex Percentile: Top 25%
Salivary Gland Tumors Diagnosis and Treatment
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