title: Brainstem primitive neuroectodermal tumors (bstPNET): results of treatment with intensive induction chemotherapy followed by consolidative chemotherapy with autologous hematopoietic cell rescue.
abstract: We have evaluated the response rate and survival utilizing intensified chemotherapy followed by myeloablative chemotherapy with autologous hematopoietic cell rescue (AuHCR) and adjuvant radiation therapy in six young children with newly diagnosed brainstem primitive neuroectodermal tumors (bstPNET). Following maximum surgical resection of the tumor, patients received high dose induction chemotherapy including vincristine, cisplatin, cyclophosphamide, and etoposide. Eligible patients received a single cycle of myeloablative chemotherapy followed by AuHCR. Two patients survive at least 32 months with stable disease. This approach provides an alternative for young patients with bstPNET who in prior reports have had a uniformly fatal prognosis.
authors.affiliation: The Children's Hospital Los Angeles, New York University Medical Center, Memorial Sloan-Kettering Cancer Center, and Schneider Children's Hospital, USA. jfangusaro@chla.usc.edu
authors.forename: Jason R
authors.initials: JR
authors.lastname: Fangusaro
authors.forename: Rima F
authors.initials: RF
authors.lastname: Jubran
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Allen
authors.forename: Sharon
authors.initials: S
authors.lastname: Gardner
authors.forename: Ira J
authors.initials: IJ
authors.lastname: Dunkel
authors.forename: Mark
authors.initials: M
authors.lastname: Rosenblum
authors.forename: Mark P
authors.initials: MP
authors.lastname: Atlas
authors.forename: Ignacio
authors.initials: I
authors.lastname: Gonzalez-Gomez
authors.forename: Douglas
authors.initials: D
authors.lastname: Miller
authors.forename: Jonathan L
authors.initials: JL
authors.lastname: Finlay
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D016026:Bone Marrow Transplantation; D001925:Brain Damage, Chronic / Q000209:etiology; D001933:Brain Stem; D016190:Carboplatin / Q000008:administration & dosage; D002648:Child; D002675:Child, Preschool; D002945:Cisplatin / Q000008:administration & dosage; D003131:Combined Modality Therapy; D016371:Cranial Irradiation; D003520:Cyclophosphamide / Q000008:administration & dosage; D005047:Etoposide / Q000008:administration & dosage; D006801:Humans; D007223:Infant; D015192:Infratentorial Neoplasms / Q000188:drug therapy* / Q000532:radiotherapy / Q000601:surgery; D002955:Leucovorin / Q000008:administration & dosage; D015080:Mesna / Q000008:administration & dosage; D008727:Methotrexate / Q000008:administration & dosage; D018242:Neuroectodermal Tumors, Primitive / Q000188:drug therapy* / Q000532:radiotherapy / Q000601:surgery; D036102:Peripheral Blood Stem Cell Transplantation; D012074:Remission Induction; D013852:Thiotepa / Q000008:administration & dosage; D014182:Transplantation, Autologous; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D014750:Vincristine; D005047:Etoposide; D003520:Cyclophosphamide; D013852:Thiotepa; D016190:Carboplatin; D015080:Mesna; D002945:Cisplatin; D002955:Leucovorin; D008727:Methotrexate