Primary Central Nervous System Post-transplantation Lymphoproliferative Disorder: an International Primary Central Nervous System Lymphoma Collaborative Group Report

Background: Primary central nervous system (CNS) post-transplantation lymphoproliferative disorder (PCNS-PTLD) is a rare complication of solid organ transplantation. The objectives of this study were to define the clinical, radiologic, and pathologic features of this disease and to explore the impact of treatment on patient outcomes.

Methods: The authors reviewed the databases of participating institutions of the International Primary CNS Lymphoma Collaborative Group for cases of PCNS-PTLD. Thirty-four patients who had pathologically confirmed PCNS-PTLD without evidence of systemic PTLD were investigated retrospectively.

Results: The median time from transplantation to diagnosis of PCNS-PTLD was 4.4 years. Disease usually was multifocal and involved any location of the brain but was most common in the cerebral hemispheres, usually in the subcortical white matter or basal ganglia. Radiographically, all lesions enhanced either homogenously or in a ring-enhancing pattern. Cerebral biopsy was required to establish diagnosis in most patients. Most patients had monomorphic, Epstein-Barr virus (EBV)-positive disease of B-cell origin. Response rates were high regardless of treatment type, and the median survival was 47 months. Age was the only factor predictive of survival.

Conclusions: The current study demonstrated that PCNS-PTLD is typically an EBV-induced B-cell lymphoma that is responsive to treatment with favorable survival in many patients. An aggressive approach to tissue confirmation of diagnosis and treatment with chemotherapy or radiotherapy should be strongly considered.