title: Epithelial-Myoepithelial Carcinoma of the Lacrimal Gland 14 Years After En Bloc Resection of a Pleomorphic Lacrimal Gland Adenoma.
abstract: 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.
authors.affiliation: *Division of Ophthalmology and †Department of Pathology, University of São Paulo Medical School, São Paulo SP, Brazil.
authors.forename: Allan C Pieroni
authors.initials: AC
authors.lastname: Gonçalves
authors.forename: Patricia Picciarelli
authors.initials: PP
authors.lastname: de Lima
authors.forename: Mario L R
authors.initials: ML
authors.lastname: Monteiro
mesh_terms: D000199:Actins / Q000378:metabolism; D008949:Adenoma, Pleomorphic / Q000473:pathology* / Q000601:surgery; D014408:Biomarkers, Tumor / Q000378:metabolism; D004847:Epithelial Cells / Q000378:metabolism / Q000473:pathology*; D005134:Eye Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D053552:Keratin-7 / Q000378:metabolism; D007766:Lacrimal Apparatus Diseases / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D008279:Magnetic Resonance Imaging; D008565:Membrane Proteins / Q000378:metabolism; D008875:Middle Aged; D009208:Myoepithelioma / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D016609:Neoplasms, Second Primary; D013508:Ophthalmologic Surgical Procedures; D014057:Tomography, X-Ray Computed
chemical_list: C541116:ACTA2 protein, human; D000199:Actins; D014408:Biomarkers, Tumor; C081876:CKAP4 protein, human; D053552:Keratin-7; D008565:Membrane Proteins