title: Adults with CNS primitive neuroectodermal tumors/pineoblastomas: results of multimodal treatment according to the pediatric HIT 2000 protocol.
abstract: Central nervous system primitive neuroectodermal tumors (CNS-PNET) and pineoblastomas (PBL) are rare in adulthood. Knowledge on clinical outcome and the efficacy and toxicities of chemotherapy in addition to radiotherapy is limited. Patients older than 21 years at diagnosis were followed in the observational arm of the prospective pediatric multicenter trial HIT 2000. After surgery, craniospinal irradiation and maintenance or sandwich chemotherapy were recommended. Radiotherapy was normo- (35.2 Gy; tumor region, 55.0 Gy; metastasis, 49.6 Gy) or hyperfractionated (40.0 Gy; tumor bed, 68.0 Gy; metastasis, 50-60 Gy). Maintenance chemotherapy consisted of eight courses (vincristine, lomustine, cisplatin). Sandwich chemotherapy included two cycles of postoperative chemotherapy followed by radiotherapy, and four courses of maintenance chemotherapy. Seventeen patients (CNS-PNET, n = 7; PBL, n = 10), median age 30 years, were included. Eight patients had a postoperative residual tumor and four patients metastatic disease. The median follow-up of ten surviving patients was 41 months. The estimated rates for 3-year progression-free survival (PFS) and overall survival were 68 ± 12 and 66 ± 13%, respectively. PBL compared to CNS-PNET tended towards a better PFS, although the difference was not clear (p = 0.101). Both chemotherapeutic (maintenance, n = 6; sandwich, n = 8) protocols did not differ in their PFS and were feasible with acceptable toxicities. Intensified regimens of combined chemo- and radiotherapy are generally feasible in adults with CNS-PNET/PBL. The impact of intensified chemotherapy on survival should be further assessed.
authors.affiliation: Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Martinistr. 52, 20246, Hamburg, Germany.
authors.forename: Carsten
authors.initials: C
authors.lastname: Friedrich
authors.forename: Klaus
authors.initials: K
authors.lastname: Müller
authors.forename: Katja
authors.initials: K
authors.lastname: von Hoff
authors.forename: Robert
authors.initials: R
authors.lastname: Kwiecien
authors.forename: Torsten
authors.initials: T
authors.lastname: Pietsch
authors.forename: Monika
authors.initials: M
authors.lastname: Warmuth-Metz
authors.forename: Nicolas U
authors.initials: NU
authors.lastname: Gerber
authors.forename: Peter
authors.initials: P
authors.lastname: Hau
authors.forename: Joachim
authors.initials: J
authors.lastname: Kuehl
authors.forename: Rolf D
authors.initials: RD
authors.lastname: Kortmann
authors.forename: André O
authors.initials: AO
authors.lastname: von Bueren
authors.forename: Stefan
authors.initials: S
authors.lastname: Rutkowski
mesh_terms: D000328:Adult; D001932:Brain Neoplasms / Q000401:mortality / Q000628:therapy*; D003131:Combined Modality Therapy / Q000379:methods*; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D018242:Neuroectodermal Tumors, Primitive / Q000401:mortality / Q000628:therapy*; D012189:Retrospective Studies; D055815:Young Adult