title: Primary pineal rhabdomyosarcoma successfully treated by high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation: case report.
abstract: Primary intracranial rhabdomyosarcoma is quite rare, and its prognosis is poor compared with that for rhabdomyosarcoma in other organs. The authors present a case of pineal rhabdomyosarcoma successfully managed with multimodal therapy including surgery, chemotherapy, radiation, and high-dose chemotherapy (HDC) followed by autologous peripheral blood stem cell transplantation (HDC/APBSCT). An 8-year-old girl presenting with headache and nausea was referred to the authors' institution. Computed tomography and MRI revealed a pineal tumor associated with obstructive hydrocephalus. Subsequently, an emergent endoscopic tumor biopsy and third ventriculostomy were performed. The patient's symptoms immediately improved. The most likely pathological diagnosis was embryonal rhabdomyosarcoma. Chemotherapy with etoposide, cyclophosphamide, cisplatin, pirarubicin, ifosfamide, actinomycin D, and vincristine was followed by a second-look operation and whole-brain and craniospinal radiation. Because the intraoperative findings and pathological examination of the second operation suggested a definitive diagnosis of rhabdomyosarcoma and the presence of viable residual tumor cells, HDC with etoposide and melphalan was followed by APBSCT. The patient was discharged from the hospital without residual tumor or any neurological deficit. No recurrence was observed at 30 months. This is the first case of primary pineal rhabdomyosarcoma treated with HDC/APBSCT. Although the efficacy of HDC/APBSCT for rhabdomyosarcoma has not been established, the prognosis of primary intracranial rhabdomyosarcoma treated with conventional treatment is quite poor. High-dose chemotherapy followed by APBSCT may contribute to a better prognosis for primary intracranial rhabdomyosarcoma.
authors.affiliation: Departments of 1 Neurosurgery and.
authors.forename: Yukitomo
authors.initials: Y
authors.lastname: Ishi
authors.affiliation: Departments of 1 Neurosurgery and.
authors.forename: Shigeru
authors.initials: S
authors.lastname: Yamaguchi
authors.affiliation: Pediatrics, Hokkaido University Graduate School of Medicine and.
authors.forename: Akihiro
authors.initials: A
authors.lastname: Iguchi
authors.affiliation: Pediatrics, Hokkaido University Graduate School of Medicine and.
authors.forename: Yuko
authors.initials: Y
authors.lastname: Cho
authors.affiliation: Pediatrics, Hokkaido University Graduate School of Medicine and.
authors.forename: Junjiro
authors.initials: J
authors.lastname: Ohshima
authors.affiliation: Department of Surgical Pathology, Hokkaido University Hospital, Sapporo, Hokkaido, Japan.
authors.forename: Kanako C
authors.initials: KC
authors.lastname: Hatanaka
authors.affiliation: Department of Surgical Pathology, Hokkaido University Hospital, Sapporo, Hokkaido, Japan.
authors.forename: Emi
authors.initials: E
authors.lastname: Takakuwa
authors.affiliation: Departments of 1 Neurosurgery and.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Kobayashi
authors.affiliation: Departments of 1 Neurosurgery and.
authors.forename: Shunsuke
authors.initials: S
authors.lastname: Terasaka
authors.affiliation: Departments of 1 Neurosurgery and.
authors.forename: Kiyohiro
authors.initials: K
authors.lastname: Houkin
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D001932:Brain Neoplasms / Q000628:therapy*; D002648:Child; D003131:Combined Modality Therapy / Q000379:methods; D005260:Female; D006801:Humans; D036102:Peripheral Blood Stem Cell Transplantation / Q000379:methods*; D010870:Pineal Gland / Q000000981:diagnostic imaging / Q000187:drug effects / Q000601:surgery; D010871:Pinealoma / Q000000981:diagnostic imaging / Q000628:therapy*; D012208:Rhabdomyosarcoma / Q000000981:diagnostic imaging / Q000628:therapy*; D014182:Transplantation, Autologous / Q000379:methods; D016896:Treatment Outcome
keywords: APBSCT = autologous peripheral blood stem cell transplantation; CBDCA = carboplatin; CDDP = cisplatin; CPA = cyclophosphamide; CR = complete remission; GCT = germ cell tumor; HDC = high-dose chemotherapy; IRS-V = Intergroup Rhabdomyosarcoma Study V; L-PAM = melphalan; SLO = second-look operation; VP-16 = etoposide; blood stem cell transplantation; brain tumor; high-dose chemotherapy; oncology; pineal tumor; rhabdomyosarcoma