Diagnosis and Treatment of Orbital Malignant Lymphoma: A 14-Year Review at Yamagata University

Abstract

Purpose: With the recent increase in the incidence of orbital malignant lymphoma, it is important to make the appropriate decision on effective treatment. The purpose of the current study was to develop a protocol to treat orbital malignant lymphoma based on evidence from histopathological examination. Methods: In the present study we analyzed the records of 18 cases of orbital lymphoma treated in the Yamagata University Hospital over the past 14 years, including 6 cases of isolated orbital disease and 12 cases of systemic disease. The tentative strategy for selecting the treatment was as follows: the cases at clinical stage I (Ann Arbor criteria) were treated with curative intent, and the cases at stages II–IV were treated with palliative intent. This strategy was evaluated from the viewpoints of efficacy and side effects. Results: Histopathologically, all 6 cases of isolated orbital disease were classified as low-grade malignancy, 3 of the systemic disease cases were classified as low-grade, and 9 were classified as intermediate-grade malignancy. The isolated orbital disease cases at stage I were treated by radiation or chemotherapy. The systemic disease cases at stages II–IV were treated by chemotherapy and/or radiation. The lymphoma disappeared completely in all 6 isolated orbital disease cases. Although the survival rate was 42% in systemic disease cases after a mean period of 15-month observation, the ocular complications decreased with an improved quality of life. Conclusions: It is important to determine the histological features and clinical stages in order to choose the appropriate treatment methods. All the isolated orbital disease cases were treated effectively by radiation or chemotherapy. Our strategy for the treatment of the systemic disease cases contributed to the decrease in ocular complications and the improvement in the quality of life for the patients.