Presentation
A patient with a history of liver transplant presents to the Emergency Department with worsening confusion for the past 3 days.
Patient Data
Multiple peripherally enhancing lesions in the bilateral cerebral hemispheres with significant adjacent vasogenic edema and rightward midline shift.
The right frontal lobe lesion was biopsied and found to be consistent with the primary CNS PTLD.
Pathology:
The biopsy pathology of the right frontal lobe mass was found to contain brain tissue with multifocal, polymorphous lymphohistiocytic infiltrates throughout the parenchyma.
Serum Epstein Barr virus: positive
Final Diagnosis: biopsy-proven primary central nervous system post-transplant lymphoproliferative disorder (CNS-PTLD).
Case Discussion
Post-transplant lymphoproliferative disorder is a spectrum of proliferation of lymphoid tissue that ranges from benign hyperplasia to lymphoma. Per the 2016 WHO classification of central nervous system tumors, primary CNS post-transplant lymphoproliferative disorder (CNS-PTLD) falls within the spectrum of immunodeficiency-associated CNS lymphoma. PTLD develops in approximately 2% of patients who receive solid organ and stem cell transplants who are immunosuppressed to avoid rejection. PTLD is four times more common in the pediatric population compared to adults.
Additional case contributors:
Dustin Coleman, DO
Iwan Tjauw, MD
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