Lymphoepithelial Carcinoma of the Salivary Gland: A Systematic Review and Single Institution Case Series

Introduction: Lymphoepithelial carcinoma of the salivary gland (LECSG) is a rare malignancy. The purpose of this study is to evaluate the clinical presentation, pathological features, treatment modalities, and outcomes of LECSG, with a focus on the role of Epstein-Barr virus (EBV).

Methods: Retrospective review of patients diagnosed with LECSG at a single institution identified using an institutional pathology search tool. Additionally, a systematic literature review was performed using PubMed. Full text articles in English were included and duplicate cases were removed. Clinical characteristics, histopathological findings, EBV status, treatment approaches, and survival outcomes were analyzed.

Results: There were 286 unique cases of LECSG identified via literature review and an additional 10 within our institution. Mean age was 47.8 years (range 8-92) and 52% were females. LECSG predominantly presented as a slow-growing, painless, unilateral mass. Most tumors were in the parotid gland (70.5%), followed by submandibular gland (16.6%), minor salivary gland (10.8%), and sublingual gland (2.0%). Lymph node metastesis was inconsitently reported but observed in at least 28%. Most cases were in Asian or Inuit patients (68.9%). EBV association was inconsistently reported but positive in 52%, and 89% of these were in endemic populations (Asian or Inuit). EBV positivity was rare in non-endemic cases (10.4%). Treatment primarily involved surgical resection (66.2%), often combined with adjuvant radiotherapy or chemoradiotherapy (50%). The overall prognosis was favorable, with five-year survival rates around 80%, though this varied based on tumor stage and treatment response.

Conclusions: This comprehensive analysis of 296 salivary gland LEC cases provides valuable insights into the clinical behavior, pathological features, and treatment outcomes of this rare malignancy. The study highlights the potential role of EBV in tumor pathogenesis, particularly in endemic populations, and emphasizes the need for further research to establish optimal management strategies for patients with salivary gland LEC.