title: Epstein-Barr virus-associated posttransplant lymphoproliferative disorder involving the central nervous system following autologous hematopoietic stem cell transplantation for neuroblastoma.
abstract: BACKGROUND
  Posttransplant lymphoproliferative disorder (PTLD) is a serious complication following solid organ or hematopoietic stem cell transplantation (HSCT). Although extranodal involvement of PTLD is common, its isolated involvement in the central nervous system (CNS) is extremely rare. To date, primary CNS-PTLD has been reported in 13 patients who underwent allogeneic HSCT, but no cases have been reported in autologous HSCT recipients.
  
  
  METHODS
  Herein, we report the first report of a patient with neuroblastoma that progressed to CNS-PTLD after autologous peripheral blood stem cell transplantation (auto-PBSCT). A 27-month-old boy with stage IV neuroblastoma of the left adrenal gland received auto-PBSCT after intensive chemotherapy, tumor resection, and radiation of tumor bed and regional lymph node. An intracranial tumor in his left parietal lobe was detected by magnetic resonance imaging 99 days posttransplantation, and the tumor was completely resected. The histological diagnosis of the intracranial tumor was diffuse large B-cell lymphoma with latency type III Epstein-Barr virus infection. The patient has maintained tumor free status 3 years after auto-PBSCT.
  
  
  RESULTS
  Given the rarity of CNS-PTLD, there is no consensus on the optimal treatment. Historically, the outcome of CNS-PTLD has been very poor. However, our patient remains free from PTLD after only total resection. The prognosis for PTLD following auto-HSCT may depend upon the underlying malignancy, immune state, EBV immune status, and treatments.
  
  
  CONCLUSIONS
  The outcome of PTLD following auto-HSCT is not necessarily poor prognosis. Further research is required to establish the optimal treatment strategy for CNS-PTLD.
authors.affiliation: Division of Pediatrics and Perinatology, Faculty of Medicine, University of Tottori, 36-1 Nishi-cho, Yonago, 683-8504 Japan ; Division of Molecular Pathology, Faculty of Medicine, University of Tottori, Yonago, Japan.
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Sano
authors.affiliation: Division of Pediatrics and Perinatology, Faculty of Medicine, University of Tottori, 36-1 Nishi-cho, Yonago, 683-8504 Japan.
authors.forename: Masanobu
authors.initials: M
authors.lastname: Fujimoto
authors.affiliation: Division of Pediatrics and Perinatology, Faculty of Medicine, University of Tottori, 36-1 Nishi-cho, Yonago, 683-8504 Japan.
authors.forename: Keisuke
authors.initials: K
authors.lastname: Okuno
authors.affiliation: Division of Pediatrics and Perinatology, Faculty of Medicine, University of Tottori, 36-1 Nishi-cho, Yonago, 683-8504 Japan.
authors.forename: Jun-Ichi
authors.initials: J
authors.lastname: Ueyama
authors.affiliation: Division of Pediatric Surgery, Faculty of Medicine, University of Tottori, Yonago, Japan.
authors.forename: Shuichi
authors.initials: S
authors.lastname: Takano
authors.affiliation: Division of Molecular Pathology, Faculty of Medicine, University of Tottori, Yonago, Japan.
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Hayashi
authors.affiliation: Division of Pediatrics and Perinatology, Faculty of Medicine, University of Tottori, 36-1 Nishi-cho, Yonago, 683-8504 Japan.
authors.forename: Susumu
authors.initials: S
authors.lastname: Kanzaki
keywords: Autologous hematopoietic stem cell transplantation; Central nervous system; Epstein-Barr virus; Neuroblastoma; Posttransplant lymphoproliferative disorder