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Int J Radiat Oncol Biol Phys. 2014 Jul 15;89(4):863-71. doi: 10.1016/j.ijrobp.2014.04.017.

Treatment of children with central nervous system primitive neuroectodermal tumors/pinealoblastomas in the prospective multicentric trial HIT 2000 using hyperfractionated radiation therapy followed by maintenance chemotherapy.

Author information

  • 1Department of Pediatric Oncology, University Children's Hospital, Zurich, Switzerland. Electronic address: nicolas.gerber@kispi.uzh.ch.
  • 2Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
  • 3Department of Pediatric Oncology, University of Wuerzburg, Wuerzburg, Germany.
  • 4Institute of Biostatistics and Clinical Research, University of Muenster, Germany.
  • 5Department of Radiation Oncology, Medical Faculty, Heinrich Heine University of Duesseldorf, Duesseldorf, Germany.
  • 6Department of Radiotherapy and Radio-Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
  • 7Institute for Radiation Therapy and Special Oncology, Hannover Medical School, Hannover, Germany.
  • 8Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical University of Graz, Graz, Austria.
  • 9Department of Neuropathology, University of Bonn, Bonn, Germany.
  • 10Department of Neuroradiology, University of Wuerzburg, Wuerzburg, Germany.
  • 11Department of Radiation Oncology, University of Leipzig, Leipzig, Germany.

Abstract

PURPOSE:

The prognosis for children with central nervous system primitive neuroectodermal tumor (CNS-PNET) or pinealoblastoma is still unsatisfactory. Here we report the results of patients between 4 and 21 years of age with nonmetastatic CNS-PNET or pinealoblastoma diagnosed from January 2001 to December 2005 and treated in the prospective GPOH-trial P-HIT 2000-AB4.

METHODS AND MATERIALS:

After surgery, children received hyperfractionated radiation therapy (36 Gy to the craniospinal axis, 68 Gy to the tumor region, and 72 Gy to any residual tumor, fractionated at 2 × 1 Gy per day 5 days per week) accompanied by weekly intravenous administration of vincristine and followed by 8 cycles of maintenance chemotherapy (lomustine, cisplatin, and vincristine).

RESULTS:

Twenty-six patients (15 with CNS-PNET; 11 with pinealoblastoma) were included. Median age at diagnosis was 11.5 years old (range, 4.0-20.7 years). Gross total tumor resection was achieved in 6 and partial resection in 16 patients (indistinct, 4 patients). Median follow-up of the 15 surviving patients was 7.0 years (range, 5.2-10.0 years). The combined response rate to postoperative therapy was 17 of 20 (85%). Eleven of 26 patients (42%; 7 of 15 with CNS-PNET; 4 of 11 with pinealoblastoma) showed tumor progression or relapse at a median time of 1.3 years (range, 0.5-1.9 years). Five-year progression-free and overall survival rates (± standard error [SE]) were each 58% (± 10%) for the entire cohort: CNS-PNET was 53% (± 13); pinealoblastoma was 64% (± 15%; P=.524 and P=.627, respectively).

CONCLUSIONS:

Postoperative hyperfractionated radiation therapy with local dose escalation followed by maintenance chemotherapy was feasible without major acute toxicity. Survival rates are comparable to those of a few other recent studies but superior to those of most other series, including the previous trial, HIT 1991.

TRIAL REGISTRATION:

ClinicalTrials.gov NCT00303810.

Copyright © 2014 Elsevier Inc. All rights reserved.

PMID:
24969797
[PubMed - indexed for MEDLINE]
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