A retrospective study of 40 cases of pathologically established primary central nervous system lymphoma

Objective: To explore the clinical and radiological features, treatment strategy and prognosis of primary central nervous system lymphoma (PCNSL). Methods: Forty pathologically established PCNSL patients were enrolled for retrospective analysis of clinical, radiological and pathological presentations, treatment strategies and survival periods. Results: PCNSL was more common in males of 50-60 years old with an acute or subacute onset. seventy-five percent of the patients presented with solitary lesions and 25% multifocal lesions. Most of the lymphomas were adjacent to the cerebral spinal fluid pathway either in deep structures near ventricle (57.5%) or superficial structures near cortex (32.5%). Almost all were of B cell type, with characteristic phenomenon of "cuff" of tumor cells surrounding the vessels and "stars in sky". The clinical manifestations were nonspecific. Most of the tumors were hypointense on T 1-weighed and hyperintense on T 2-weighed with clear edge and obvious edema. sixty-seven point six percent of the tumors enhanced homogeneously and 13.5% had ring enhancing phenomenon. Patients undergoing high dose methotrexate (HDMTX) based chemotherapy had a higher survival rate (75.0%) than those treated without HDMTX. The mean survival period was only 14.8 months. After relapse, 69.2% of the patients survived less than 6 month. Conclusions: PCNSL has nonspecific clinical manifestations, whose diagnosis relies on stereotactic biopsy. HDMTX based chemotherapy is the first treatment of choice, and the prognosis of PCNSL is poor with a mean survival period of 14.8 months.