Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
SHR Neuro Krebs Kardio Lipid Stoffw Microb
Gerber, NU; von Hoff, K; Resch, A; Ottensmeier, H; Kwiecien, R; Faldum, A; Matuschek, C; Hornung, D; Bremer, M; Benesch, M; Pietsch, T; Warmuth-Metz, M; Kuehl, J; Rutkowski, S; Kortmann, RD.
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.
Int J Radiat Oncol Biol Phys. 2014; 89(4):863-871
Doi: 10.1016/j.ijrobp.2014.04.017
Web of Science
PubMed
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- Co-Autor*innen der Med Uni Graz
- Benesch Martin
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- Abstract:
- 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. 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). 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). 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. Copyright © 2014 Elsevier Inc. All rights reserved.
- Find related publications in this database (using NLM MeSH Indexing)
- Adolescent -
- Antineoplastic Agents - therapeutic use
- Brain Neoplasms - drug therapy
- Brain Neoplasms - mortality
- Brain Neoplasms - radiotherapy
- Brain Neoplasms - surgery
- Child -
- Child, Preschool -
- Clinical Protocols -
- Disease-Free Survival -
- Dose Fractionation, Radiation -
- Female -
- Humans -
- Maintenance Chemotherapy - methods
- Male -
- Memory, Short-Term -
- Mental Processes -
- Neoplasm Recurrence, Local -
- Neoplasm, Residual -
- Neuroectodermal Tumors, Primitive - drug therapy
- Neuroectodermal Tumors, Primitive - mortality
- Neuroectodermal Tumors, Primitive - radiotherapy
- Neuroectodermal Tumors, Primitive - surgery
- Neuropsychological Tests -
- Pineal Gland -
- Pinealoma - drug therapy
- Pinealoma - mortality
- Pinealoma - radiotherapy
- Pinealoma - surgery
- Prospective Studies -
- Regression Analysis -
- Survival Rate -
- Young Adult -