Primary Central Nervous System Lymphoma: An Experience of a Regional Cancer Center from India

Background: Primary central nervous system lymphoma (PCNSL) is a rare malignant non-Hodgkin's lymphoma and it accounts for 1% of all intracranial tumors. Only a few PCNSL studies have been reported from India, and hence, we conducted the retrospective study in our institute to analyze the main clinical features at diagnosis and the treatment results in PCNSL. Materials and Methods: We retrospectively reviewed the medical records of 32 patients with PCNSL who were treated from 2003 to 2014. A complete history was recorded, and the physical examination included neurological assessment. Baseline investigations such as complete blood count, blood biochemistry, chest X-ray, HIV serology, and two-dimensional echocardiography were also done. Contrast-enhanced computed tomography (CT) of the brain or magnetic resonance imaging, positron-emission tomography (if affordable), cerebrospinal fluid (CSF) analysis for cytology, histopathological diagnosis (following stereotactic or open biopsy), CT of the thorax and abdomen, and bone marrow biopsy were done. All patients received whole-brain radiotherapy (WBRT) (36–50 Gy) followed by cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) regimen for six cycles. Clinical features and the treatment results were analyzed. Results: A total of 32 patients with PCNSL were studied. The median age was 50 years (range: 32–70 years). Male: female ratio was 2.5:1. All the 32 patients were histologically diffuse large B-cell lymphoma. The most common presenting symptom was headache in 20 (62.5%) patients. The area of involvement was mainly cerebral hemispheres in 22 (68.8%) patients. Multifocal involvement was seen in 22 (68.8%) patients. CSF cytology was negative in all patients. All patients received WBRT (36–50 Gy) followed by CHOP regimen for six cycles. All patients were compliant to radiation as well as chemotherapy. On a median follow-up of 18 months, the median overall survival was 14 months (range: 1–80). Conclusions: Breaking the blood–brain barrier with radiotherapy followed by CHOP chemotherapy results in reasonable outcome in patients of PCNSL, especially in the Indian setting where general built and tolerance is low- to high-dose methotrexate regimens. Newer chemotherapy regimens or targeted therapies need to be evaluated to further improve the survival.