Epithelial-Myoepithelial Carcinoma of the Lacrimal Gland 14 Years After En Bloc Resection of a Pleomorphic Lacrimal Gland Adenoma

• Published in: Ophthalmic plastic and reconstructive surgery Vol. 32; no. 2; pp. e42 - e44
• Main Authors: Gonçalves, Allan C Pieroni, de Lima, Patricia Picciarelli, Monteiro, Mario L R
• Format: Journal Article
• Language: English
• Published: United States 01-03-2016
• Subjects: Actins - metabolism; Adenoma, Pleomorphic - pathology; Adenoma, Pleomorphic - surgery; Biomarkers, Tumor - metabolism; Epithelial Cells - metabolism; Epithelial Cells - pathology; Eye Neoplasms - metabolism; Eye Neoplasms - pathology; Eye Neoplasms - surgery; Female; Humans; Immunohistochemistry; Keratin-7 - metabolism; Lacrimal Apparatus Diseases - metabolism; Lacrimal Apparatus Diseases - pathology; Lacrimal Apparatus Diseases - surgery; Magnetic Resonance Imaging; Membrane Proteins - metabolism; Middle Aged; Myoepithelioma - metabolism; Myoepithelioma - pathology; Myoepithelioma - surgery; Neoplasms, Second Primary; Ophthalmologic Surgical Procedures; Tomography, X-Ray Computed
• ISSN: 0740-9303; 1537-2677
• DOI: 10.1097/IOP.0000000000000225

Fourteen years after en bloc resection of an orbital pleomorphic adenoma, a 61-year-old female patient developed progressive left proptosis on the same side. A CT scan revealed a round, well-circumscribed, heterogeneously enhancing mass in the lacrimal fossa of the left orbit without calcification or bone erosion. An MRI scan showed a lesion that was hypointense on T1-weighted images and heterogeneously isointense on T2-weighted images. The tumor was completely resected through combined anterior and lateral orbitotomy. On histopathology, the specimen was identified as epithelial-myoepithelial carcinoma. After surgery, the patient received adjuvant therapy with irradiation of the orbit. At a 36-month follow-up evaluation, the patient remained tumor-free. Epithelial-myoepithelial carcinoma, a very uncommon tumor of the orbit, apparently behaves like a low-grade malignancy and is associated with good survival rates. However, a good prognosis for survival is tentative at best, in part because of the rarity of the lesion.