Epithelial-Myoepithelial Carcinoma of the Lacrimal Gland - Case Report and Review of Literature

Abstract

Objective: To describe the clinicopathologic features and outcome of a patient with epithelial-myoepithelial carcinoma of the lacrimal gland who underwent modified lateral orbitotomy with en toto removal of the lesion and adjuvant radiotherapy.

Methods: This is a case report.

Results: A 31-year-old Filipino male seafarer presented with a 2-month history of an enlarging left superotemporal orbital mass and inferonasal displacement of the globe. Magnetic resonance imaging revealed a superotemporal extraconal mass within the lacrimal sac fossa with evidence of osseous infiltration of the superolateral orbital rim. Modified lateral orbitotomy was performed with en toto removal of the lesion and the clinically infiltrated adjacent lateral bony margin. Histopathologic diagnosis of epithelial-myoepithelial carcinoma of the lacrimal gland was made based on the classic and distinct biphasic morphology and was confirmed with immunohistochemistry studies (cytokeratin-7, S-100, and p63). Systemic surveillance using positron emission tomography and computed tomography scan with contrast revealed no evidence of regional or distant metastasis. Adjuvant radiotherapy of the orbital area was performed for increased local control. Twelve months postoperatively, the patient showed no evidence of tumor recurrence.

Conclusion: Epithelial-myoepithelial carcinoma of the lacrimal gland is a rare condition, and this is the first documented case from the Philippines. Accurate diagnosis is necessary for appropriate treatment. It should be included in the differential diagnosis of infiltrative lesions in the lacrimal gland fossa.