Posttransplant lymphoproliferative disorder: Significance of central nervous system involvement

Background. Few data exist regarding central nervous system (CNS) involvement in patients with posttransplant lymphoproliferative disorder (PTLD). The purpose of this study was to review the Israel Penn International Transplant Tumor Registry (IPITTR) experience with CNS involvement by PTLD. Methods. Nine hundred ten PTLD cases from the United States were reported to the IPITTR and reviewed for CNS involvement. Results. One hundred thirty-six transplant recipients with PTLD (15 %) had CNS involvement. The highest incidence of CNS involvement occurred in pancreas (3 of 11; 27 %) and kidney transplant recipients (76 of 429; 18 %). Fifteen cases occurred in children and 121 cases in adults. For both children and adults, isolated CNS disease was associated with better survival when compared with multiple-site involvement (children: 29 % vs 0 %; adults: 12 % vs 6 %; P < .05). Three-year survival in PTLD patients with CNS involvement was 9.4 % and without CNS involvement was 49.4 % (P < .01). Radiation therapy alone appeared to provide the best survival rates (25 %). Conclusions. CNS involvement in transplant recipients with PTLD carries an ominous prognosis; however, isolated CNS involvement has a better prognosis than CNS plus extracranial involvement. Radiation therapy alone provides the best results, but this may be a reflection of isolated CNS disease.