title: An Infant With Medulloepithelioma Successfully Treated by High-dose Chemotherapy Followed by Autologous Peripheral Blood Stem Cell Transplantation Without Radiotherapy.
abstract: Medulloepithelioma of the central nervous system (CNS) is a rare primitive neuroectodermal tumor characterized by highly malignant behavior occurring in early childhood. Few cases have been reported and optimal management remains unknown. Here, we report a case of CNS medulloepithelioma successfully treated with high-dose chemotherapy (HDCTX) followed by autologous stem cell transplantation (auto-SCT) without radiotherapy. At the last follow-up, 3.0 years after onset, the patient was alive with no sign of relapse and normal development. To the best of our knowledge, this is the first reported case of long-term survival of CNS medulloepithelioma treated by HDCTX/auto-PBSCT without radiotherapy.
authors.affiliation: *Department of Pediatrics, Jichi Children's Medical Center Tochigi, Jichi Medical University School of Medicine †Diagnostic Pathology, Jichi Medical University School of Medicine ∥Department of Pediatric Neurosurgery, Jichi Children's Medical Center Tochigi, Jichi Medical University School of Medicine, Shimotsuke ‡Department of Human Pathology, Gunma University Graduate School of Medicine §Department of Pathology, Gunma University Hospital, Maebashi, Japan.
authors.forename: Tomomi
authors.initials: T
authors.lastname: Hayase
authors.forename: Akira
authors.initials: A
authors.lastname: Morimoto
authors.forename: Yuta
authors.initials: Y
authors.lastname: Kawahara
authors.forename: Masaki
authors.initials: M
authors.lastname: Yagi
authors.forename: Nobuyuki
authors.initials: N
authors.lastname: Kanai
authors.forename: Sumito
authors.initials: S
authors.lastname: Nobusawa
authors.forename: Junko
authors.initials: J
authors.lastname: Hirato
authors.forename: Akira
authors.initials: A
authors.lastname: Gomi
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002066:Busulfan / Q000008:administration & dosage; D002675:Child, Preschool; D003131:Combined Modality Therapy; D004305:Dose-Response Relationship, Drug; D006801:Humans; D060828:Induction Chemotherapy; D008297:Male; D008558:Melphalan / Q000008:administration & dosage; D018242:Neuroectodermal Tumors, Primitive / Q000473:pathology / Q000628:therapy*; D036102:Peripheral Blood Stem Cell Transplantation; D011379:Prognosis; D012074:Remission Induction; D014182:Transplantation, Autologous
chemical_list: D002066:Busulfan; D008558:Melphalan