title: Successful treatment of an unresectable choroid plexus carcinoma in a patient with Li-Fraumeni syndrome.
abstract: Choroid plexus carcinoma (CPC) is an uncommon central nervous system tumor requiring complete surgical excision for favorable outcome. The authors report the successful treatment of a 2-year-old patient with widely disseminated CPC and Li-Fraumeni syndrome. Following a partial resection of the tumor the patient received chemotherapy consisting of cyclophosphamide, etoposide, and carboplatin. There were no additional surgical procedures and radiation was not administered. Remarkably, the patient remains without evidence of active disease more than 3 years from the completion of therapy. Additional studies are necessary to determine whether this treatment plan can be beneficial to other patients with CPC and whether the patient's p53 mutation had an effect on outcome.
authors.affiliation: Division of Hematology/Oncology, Department of Pediatrics, DeVos Children's Hospital, Grand Rapids, Michigan, USA.
authors.forename: David S
authors.initials: DS
authors.lastname: Dickens
authors.forename: Judith A
authors.initials: JA
authors.lastname: Dothage
authors.forename: Richard L
authors.initials: RL
authors.lastname: Heideman
authors.forename: Edgar T
authors.initials: ET
authors.lastname: Ballard
authors.forename: Paul T
authors.initials: PT
authors.lastname: Jubinsky
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D016190:Carboplatin / Q000627:therapeutic use; D002277:Carcinoma / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery; D002675:Child, Preschool; D016545:Choroid Plexus Neoplasms / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery; D003520:Cyclophosphamide / Q000627:therapeutic use; D005047:Etoposide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D016864:Li-Fraumeni Syndrome / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery; D008279:Magnetic Resonance Imaging; D009362:Neoplasm Metastasis / Q000188:drug therapy*
chemical_list: D005047:Etoposide; D003520:Cyclophosphamide; D016190:Carboplatin