Population Outcomes in Primary Central Nervous System Lymphoma: Provincial Experience and National Survey

Abstract

Introduction: Primary central nervous system lymphoma (PCNSL) is rare and carries a poor prognosis. A retrospective review of Manitoba data for patients diagnosed with PCNSL spanning 1998-2008 was undertaken along with a national survey of PCNSL management. The study’s goal was to benchmark provincial metrics for diagnostic work-up and patient outcomes in PCNSL. Materials and Methods: A retrospective chart review was carried out, and data involving diagnostic workup, treatment, and outcome were collected. A mailed survey regarding PCNSL diagnosis and therapeutic practices was distributed to adult neuro oncology centres in Canada. Results: The age adjusted rate of PCNSL in Manitoba was 0.8 cases per 100,000. Comparing diagnostic tests obtained by Manitobans with PCNSL between 1998-2003 and 2004-2008, the proportion of patients receiving the appropriate testings increased. Curative intent treatment utilizing high-dose methotrexate was offered to 12/72 (17%) patients, and conferred a median overall survival (OS) of 33 months. Non-curative intent therapy or palliation was offered to 83% of patients, who achieved a median OS of 2 months. No significant survival difference was found between patients receiving non-curative chemotherapy or radiotherapy. National survey results demonstrate significant variation in the management of patients with PCNSL across Canada. Discussion: The proportion of patients receiving appropriate diagnostic testing has increased over time. Patients considered for non-curative intent therapy have suboptimal responses and may benefit from novel treatment approaches or more aggressive palliation. National variation in PCNSL management offers an opportunity to develop a national consensus.