Primary Mucoepidermoid Carcinoma of the Lacrimal Apparatus

PURPOSE: In this study, we evaluated the clinicopathologic and molecular characteristics of lacrimal apparatus mucoepidermoid carcinoma (MEC) to define its typical diagnostic features. DESIGN: Retrospective observational case series. METHODS: Institutional pathology records between 2011 and 2021 were searched for all cases of lacrimal apparatus MEC. RESULTS: A total of 2 male and 6 female patients ranging in age from 18 to 83 years (median 56, mean 54) were included. Six lacrimal apparatus MECs were found in the lacrimal gland, and 2 cases occurred in the lacrimal sac and nasolacrimal duct. Histologically, there were 6 cases of conventional MEC, 1 clear-cell variant of MEC, and 1 oncocytic variant of MEC for a total of 8 cases. There were 3 low-grade cases and 5 high-grade cases. All 8 cases were evaluated via immunohistochemistry, and the results were positive (scores 1-4) for pankeratin, 34betaE12, p63, p40, CK7, CK8, and CK19, with a relatively higher expression of p63 observed in high-grade MEC. The presence of human papillomavirus (HPV) type 6 DNA was found in 4 patients. MAML2 fluorescence in situ hybridization was positive for MAML2 rearrangement in 3 lacrimal gland tumors (2 low-grade and 1 high-grade). Six tumors were managed with radical resection, and 2 patients underwent orbital exenteration. Postoperative radiation therapy was delivered to 6 patients, and chemotherapy was administered to 1 patient. CONCLUSIONS: MECs of the lacrimal apparatus are rare tumors, and the rate of MAML2 translocations is lower than that in salivary MECs. Lacrimal gland and lacrimal sac MECs may not be of the same sub-types intrinsically because of the difference in MAML2 translocation, anatomy, and clinical course. The etiologic function of HPV type 6 infection should be explored in lacrimal apparatus MECs. Radical surgery is the treatment of choice. The description of these unique findings may assist in the definitive diagnosis of and improve our understanding of lacrimal apparatus MEC. ((C) 2022 The Authors. Published by Elsevier Inc.