title: Pathology of central nervous system posttransplant lymphoproliferative disorders: lessons from pediatric autopsies.
abstract: Posttransplant lymphoproliferative disorders (PTLD) involving the central nervous system (CNS) in children are uncommon and can prove diagnostically challenging. The clinical and imaging characteristics of CNS PTLD can overlap with those of infection, hemorrhage, and primary CNS tumors. Some cases of CNS PTLD remain clinically unsuspected and are diagnosed postmortem. We report 6 instances of CNS PTLD in children, 2 of which were limited to the CNS and were unsuspected before autopsy. In our autopsy series, PTLD was found outside the CNS in 4 out of 6 cases. Since CNS PTLD has a poor prognosis and the presentation can be subtle, unsuspected, and high grade, it is important to maintain a high index of suspicion and to perform imaging and brain biopsy whenever clinically appropriate. In the presence of leptomeningeal involvement, the diagnosis could be made by cerebral spinal fluid examination.
authors.affiliation: Department of Pathology, Children's Hospital of Wisconsin, Medical College of Wisconsin, 9000 West Wisconsin Avenue, Milwaukee, WI 53226, USA. ggheorghe@mcw.edu
authors.forename: Gabriela
authors.initials: G
authors.lastname: Gheorghe
authors.forename: Oana
authors.initials: O
authors.lastname: Radu
authors.forename: Samuel
authors.initials: S
authors.lastname: Milanovich
authors.forename: Ronald L
authors.initials: RL
authors.lastname: Hamilton
authors.forename: Ronald
authors.initials: R
authors.lastname: Jaffe
authors.forename: James F
authors.initials: JF
authors.lastname: Southern
authors.forename: John A
authors.initials: JA
authors.lastname: Ozolek
mesh_terms: D000293:Adolescent; D001344:Autopsy; D002493:Central Nervous System Diseases / Q000209:etiology* / Q000473:pathology*; D002648:Child; D006801:Humans; D008232:Lymphoproliferative Disorders / Q000209:etiology* / Q000473:pathology*; D016377:Organ Transplantation / Q000009:adverse effects*; D011183:Postoperative Complications / Q000209:etiology / Q000473:pathology