Clinical characteristics and treatment analysis of primary ocular adnexal lymphoma

Objective: To study the clinical characteristics and analyze the treatment of primary ocular adnexal lymphoma (POAL). Methods: A retrospective review was performed based on the clinical records of 26 POAL cases, who were treated from June 1999 to June 2009. The clinical manifestations, imaging features, diagnosis approaches and treatment of them were analyzed. Results: The main symptoms were a slowly growing painless mass, exophthalmia and red eyelid. Mostly, imaging finding shows "casting-like". 50 % of POAL was at the orbit. 22 patients were B-cell origin [13 patients were extranodal marginal zone B-cell lymphoma-mucosal-associated lymphoid tissue (MALT) lymphoma], 2 patients were NK/T cell lymphoma. 76.9 % patients were in stage I - II. Surgery, radiotherapy and chemotherapy were most common treatment modalities for POAL. Conclusion: Most POAL were low-grade B-cell lymphoma, with MALT by far the most common type. POAL was easy to be misdiagnosed due to non specific clinical manifestations. Imaging finding was helpful to diagnosis, but could not reliably distinguish between benign and malignant. The invasive biopsy is the main diagnosis approach. Radiotherapy had been the standard treatment for low-grade POAL. High-grade or infiltrating peri-orbit need combine chemotherapy. Immunotherapy and molecular targeted therapy were the direction of future research.