Isolated central nervous system post-transplant lymphoproliferative disorder in a pediatric patient: a case report with pathobiological perspective

Isolated central nervous system post-transplant lymphoproliferative disorder (ICNS-PTLD) in pediatric patients is a distinctly rare entity without accepted diagnostic criteria or treatment recommendations. We present a case of an 8-year-old male with a prior history of a kidney transplant who developed and ICNS-PTLD. We highlight the pathobiology and diagnostic features with a brief review of the literature on these rare cases. There is a complex interplay between CD30, Epstein Barr Virus and MYC as part of lymphocyte transformation leading to PTLD. In the appropriate clinical setting, CD30 and EBV positivity along with normal MYC expression are highly predictive of CNS-PTLD over a Primary CNS lymphoma. ICNS-PTLD has only been rarely reported in children. The faithful diagnosis is necessary for prognostication and to accrue data for treatment recommendations.