原發性中樞神經系統的淋巴瘤並不常見。過去的治療以全腦放射線治療為主，隨著化學藥物
的發展及長期對中樞神經系統淋巴瘤臨床表現的觀察研究，化學治療合併放射線治療使得存活率
逐漸提升，也漸成為治療的主流。而治療引發之晚期神經系統併發症，例如認知功能障礙等，在
近些年也漸受到重視。國際原發性中樞神經系統淋巴瘤合作組織則制定評估治療反應的定義及
準則，以方便國際間之學術交流及合作。本篇研究回顧2000-2005 年有關原發性中樞神經系統
淋巴瘤的文獻發現，目前最適當的治療方式雖仍未達共識，但多數研究肯定以大劑量甲氨喋呤
（methotrexate）為主的聯合化療加上放射線治療為目前最常用且較有效的治療模式，至於脊髓腔內化學治療的角色則仍有爭議。傳統上以單獨全腦放射治療為主的作法，目前已漸式微。未來應進行跨國大型隨機性研究，並將神經精神功能評估列入討論，相信應能找出最佳的治療方式。

Primary central nervous system lymphoma is a rare form of extranodal non-Hodgkin’s lymphoma that is confined to the central nervous system and eye. Whole brain irradiation was the main treatment before 1990. However, Numerous studies examined this disorder in the past 6 years. Novel
treatment regimens continue to be studied for newly diagnosed primary central nervous system lymphoma. As overall survival improved, the burden of late neurotoxicity has become more apparent.
Neuropsychological testing of patients treated with chemoradiation shows significant cognitive deficits that can be attributed to whole brain irradiation. The International Primary Central Nervous System Lymphoma Collaborative Group (IPCG) has proposed guidelines for the baseline evaluation and response criteria for clinical trials of the disease. In conclusion, the optimal treatment for primary central nervous system lymphoma remains undefined. The role of intrathecal chemotherapy remains
unclear. Whole brain irradiation alone become less popular. The most common and probably effective treatment of newly diagnosed disease should include high-dose methotrexate-based chemotherapy plus radiotherapy at the present status. International large-scale prospective randomized studies including prospective neuropsycholgical test will be needed, as it is impossible to draw conclusions from the nonrandomized small series published so far.