Primary Lymphoepithelioma-Like Carcinoma of Ocular Adnexa: Clinicopathologic Features and Treatment

Abstract

Background and Methods: Lymphoepithelioma-like carcinoma (LELC) is a rare malignancy in ocular adnexa. Here, we report 4 patients with LELC and review 11 patients reported in the literature. Clinical profiles, association with Epstein–Barr virus (EBC), treatment, and outcomes are analyzed. Results: Lacrimal glands and the lacrimal drainage system, eyelid, and conjunctiva are potential primary sites for LELC. The tumours are characterized histologically by nests of undifferentiated malignant cells surrounded by lymphoid infiltrates. Infection with EBC was confirmed in LELC of ocular adnexa, and that association seemed to be restricted to Asian populations. Results from our centre uniformly showed expression of EBC-encoded small rnas in primary tumour, locally recurrent tumour, and metastatic lymph nodes. This disease had a tendency to relapse regionally. Postoperative radiotherapy seems to improve disease-free survival. Tumours appear to be sensitive to radiotherapy and chemotherapy based on cisplatin and 5-fluorouracil. At our centre, 3 patients were still living at 22, 33, and 76 months after surgery. One patient died of distant metastasis after a survival of 38 months. Conclusions: Lymphoepithelioma-like carcinoma is a heterogenous entity among ocular adnexal malignancies. Multimodality treatment provides a better chance at survival. Further investigation is required to achieve a better understanding of the biologic behavior of this entity and of its optimal treatment.