Rapid identification of serum/CSF supporting diagnostic markers such as active EBV replication, elevated CSF protein, and positive cytology/flow cytometry is critical.

PCNS-PTLD is a rare complication of solid organ transplantation (SOT) and is a distinct entity from primary CNS lymphoma (PCNSL).  With fewer than 90 cases reported since 1970, there are no treatment standards.  Existing data supports combining reduction of immunosuppression (RI), whole-brain radiotherapy, monoclonal antibody therapy, and systemic/IT chemotherapy.  Treating kidney SOT PTLD is complicated by reduced renal clearance, contrast-dye restrictions, and nephrotoxicity.  Treatment complications and allograft rejection cause early morbidity and mortality. 

Patients achieved complete responses and mOS has not been met.  No patients have relapsed.  One patient with recurrent systemic infection.  Long-term survival in PCNS-PTLD is one distinguishing feature from PCNSL. 

With multiple treatment obstacles in renal-SOT PT early recognition and minimally toxic regimens improve patient outcomes.  This data challenges traditional paradigms in diagnosis and treatment of PTLD, specifically EBV+ disease occurring >6 years post SOT.  We propose that the specialized CNS immune microenvironment permits EBV driving of neoplastic progression, which is exquisitely sensitive to B-cell targeting concurrent with partial restoration of host immunity.   Furthermore, targeted radiotherapy, temozolomide, and partial immunosuppression maintains patient quality-of-life and reduce risk for allograft rejection.