Secondary versus recurrent central nervous system lymphoma - similar or different?

Abstract BACKGROUND Although rare, primary central nervous system lymphoma (PCNSL) affects 0.5 per 100,000 individuals. It is estimated that 5% of cases will develop into secondary central nervous system lymphoma (SCNSL) and 2-5% will evolve into recurrent CNS lymphoma (RPCNSL) with the diffuse large B-cell lymphoma (DLBCL) variant of RPCNSL. METHODS This is a retrospective study; data obtained via chart review. RESULTS We compared 3 patients with RPCNSL to 3 patients with SCNSL. All patients were EBV and HIV negative, DLBCL variant. The average age of diagnosis with the first malignancy with RCNSL was 62.7 years old and age at recurrence was 66.7. With SCNSL, the initial age of diagnosis averaged to 72.7 years old and with secondary presentation at an average age of 76. Initial PCNSL treatments for RPCSNL were steroids, RCHOP with radiation, and DeAngelis Protocol. Initial malignancies for patients with SCNSL were renal cell carcinoma in 2 patients and atypical chronic lymphocytic leukemia variant with 17p deletion. All patients with RPCNSL presented with altered mental status and 2/3 SCNSL patients were altered upon presentation; 1 SCNSL patient was asymptomatic. The lesions within the RPCNSL cohort were all single lesions, localized to the frontal and temporal lobes. Within the SCNSL cohort, 2/3 patients had multiple lesions, whereas 1 patient had single lesions. All of the RPCNSL patients were ultimately on palliative care. SCNSL 2/3 were lost to follow-up and 1/3 elected to proceed with further treatment. CONCLUSION Although differing in etiology, there are shared features among SCNSL and RPCNSL. Most relapses are in the central nervous system, a small number of relapses are isolated systemic relapses, and clinical symptoms occur early and vary. Treatment is similar for both, although the prognosis for RPCNSL is very poor.