Primary central nervous system lymphoma: An Indian Perspective

Context: Primary central nervous system lymphoma (PCNSL) is an unusual entity comprising around 5% of extra nodal lymphomas with a dismal prognosis in spite of a multi-modality treatment protocol involving chemotherapy and radiotherapy. There is sporadic data from this part of the world and hence we took up this study to analyze the outcome with various modalities of treatment. Materials and Methods: We undertook this study between January 2007 and January 2013 on 33 consecutive patients diagnosed with PCNSL to analyze the clinical profile and treatment outcomes of PCNSL at a tertiary care oncology center in South India. Twelve patients received the DeAngelis protocol and the remaining 21 patients received either radiotherapy or a combination of radiotherapy and anthracycline based therapy or steroids. This study also compared the outcomes with protocols of DeAngelis et al. and cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) regimen with/out radiotherapy. Results: A total of 33 patients with PCNSL were studied. Median age was 40 years (range 22-75 years). Male: Female ratio was 3.1:1. All 33 were histologically diffuse large B-cell lymphoma (DLBCL). HIV was positive in 1. The most common presenting symptom was focal neurological deficits in 19 (58%). Cerebrospinal fluid was positive in 2 (6%). The area of involvement was mainly cerebral hemispheres 20 (61%). The treatment protocols followed were DeAngelis in 12, cyclophosphamide, doxorubicin, vincristine and prednisolone with radiotherapy (CHOP + radiation therapy [RT]) in 12, RT alone in 6, CHOP in 2 and 1 received no therapy. On a median follow-up of 17 months, the median overall survival was 15 months (range 1-60 months) with DeAngelis protocol and 12 months (range 8-24 months) with CHOP + RT. Conclusions: DeAngelis protocol has improved the prognosis in patients with PCNSL compared with other protocols as shown in our study. Newer chemotherapy regimens or targeted therapies need to be evaluated to further improve the survival.