Treatment of young children with CNS-primitive neuroectodermal tumors/pineoblastomas in the prospective multicenter trial HIT 2000 using different chemotherapy regimens and radiotherapy
Open Access
- 7 December 2012
- journal article
- research article
- Published by Oxford University Press (OUP) in Neuro-Oncology
- Vol. 15 (2), 224-234
- https://doi.org/10.1093/neuonc/nos292
Abstract
Especially in young children, primitive neuroectodermal tumors of the central nervous system (CNS-PNET) and pineoblastomas are associated with an unfavorable outcome, and only a few prospective trials have been conducted thus far. From January 2001 through January 2005, 17 eligible children aged n = 8), ependymoblastoma (n = 1), or pineoblastoma (n = 8) confirmed by central review were prospectively treated in the trial HIT 2000. In nonmetastatic disease (n = 11), up to 5 postoperative cycles of HIT-SKK systemic multiagent chemotherapy (8 months duration), followed by craniospinal radiotherapy (CSI), were given. In metastatic disease (M1-M3, n = 6), treatment consisted of a shorter induction chemotherapy (2–3 months) with carboplatin and etoposide, followed by tandem high-dose chemotherapy (HDCT) in case of good response to induction. During induction and HDCT, patients received intraventricular methotrexate. CSI was applied to all patients with poor response to induction or residual disease after HDCT and was optional for patients with residual disease before HDCT. Five-year event-free survival and overall survival rates ± standard error for all eligible patients were 24% ± 10% and 40% ± 12%, respectively (median follow-up of survivors: 8.3 years). Only one patient with nonmetastatic disease remained free of relapse/progressive disease during induction. Three of 6 patients with metastatic disease responded to induction and received tandem-HDCT, followed by preventive CSI, and remain in continuous complete remission. Short intensive induction chemotherapy followed by tandem-HDCT in young children with CNS-PNET/pineoblastomas seems to be superior to the prolonged and less intensive induction regimen.Keywords
This publication has 33 references indexed in Scilit:
- Treatment of young children with localized medulloblastoma by chemotherapy alone: Results of the prospective, multicenter trial HIT 2000 confirming the prognostic impact of histologyNeuro-Oncology, 2011
- Non-cerebellar primitive neuroectodermal tumors (PNET): Summary of the Milan consensus and state of the art workshop on marrow ablative chemotherapy with hematopoietic cell rescue for malignant brain tumors of childhood and adolescentsPediatric Blood & Cancer, 2010
- Treatment of early childhood medulloblastoma by postoperative chemotherapy and deferred radiotherapyNeuro-Oncology, 2009
- The 2007 WHO Classification of Tumours of the Central Nervous SystemActa Neuropathologica, 2007
- Supratentorial primitive neuroectodermal tumors of the central nervous system frequently harbor deletions of the CDKN2A locus and other genomic aberrations distinct from medulloblastomasGenes, Chromosomes and Cancer, 2007
- Role of Radiotherapy in Supratentorial Primitive Neuroectodermal Tumor in Young Children: Results of the German HIT-SKK87 and HIT-SKK92 TrialsJournal of Clinical Oncology, 2006
- Kurzgefasste Intelligenzdiagnostik bei HirntumorenKindheit und Entwicklung, 2006
- Multiagent Chemotherapy and Deferred Radiotherapy in Infants With Malignant Brain Tumors: A Report From the Children’s Cancer GroupJournal of Clinical Oncology, 2005
- Patterns of treatment failure in infants with primitive neuroectodermal tumors who were treated on CCG-921: A phase III combined modality studyPediatric Blood & Cancer, 2005
- The treatment of malignant brain tumors in infants and very young children: An update of the Pediatric Oncology Group experienceNeuro-Oncology, 1999