Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
SHR Neuro Krebs Kardio Lipid Stoffw Microb
Mynarek, M; Pizer, B; Dufour, C; van Vuurden, D; Garami, M; Massimino, M; Fangusaro, J; Davidson, T; Gil-da-Costa, MJ; Sterba, J; Benesch, M; Gerber, N; Juhnke, BO; Kwiecien, R; Pietsch, T; Kool, M; Clifford, S; Ellison, DW; Giangaspero, F; Wesseling, P; Gilles, F; Gottardo, N; Finlay, JL; Rutkowski, S; von Hoff, K.
Evaluation of age-dependent treatment strategies for children and young adults with pineoblastoma: analysis of pooled European Society for Paediatric Oncology (SIOP-E) and US Head Start data.
Neuro Oncol. 2017; 19(4):576-585
Doi: 10.1093/neuonc/now234
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- Co-Autor*innen der Med Uni Graz
- Benesch Martin
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- Abstract:
- Pineoblastoma is a rare pineal region brain tumor. Treatment strategies have reflected those for other malignant embryonal brain tumors. Original prospective treatment and outcome data from international trial groups were pooled. Cox regression models were developed considering treatment elements as time-dependent covariates. Data on 135 patients with pineoblastoma aged 0.01-20.7 (median 4.9) years were analyzed. Median observation time was 7.3 years. Favorable prognostic factors were age ≥4 years (hazard ratio [HR] for progression-free survival [PFS] 0.270, P < .001) and administration of radiotherapy (HR for PFS 0.282, P < .001). Metastatic disease (HR for PFS 2.015, P = .006), but not postoperative residual tumor, was associated with unfavorable prognosis. In 57 patients <4 years old, 5-year PFS/overall survival (OS) were 11 ± 4%/12 ± 4%. Two patients survived after chemotherapy only, while 3 of 16 treated with craniospinal irradiation (CSI) with boost, and 3 of 5 treated with high-dose chemotherapy (HDCT) and local radiotherapy survived. In 78 patients aged ≥4 years, PFS/OS were 72 ± 7%/73 ± 7% for patients without metastases, and 50 ± 10%/55 ± 10% with metastases. Seventy-three patients received radiotherapy (48 conventionally fractionated CSI, median dose 35.0 [18.0-45.0] Gy, 19 hyperfractionated CSI, 6 local radiotherapy), with (n = 68) or without (n = 6) chemotherapy. The treatment sequence had no impact; application of HDCT had weak impact on survival in older patients. Survival is poor in young children treated without radiotherapy. In these patients, combination of HDCT and local radiotherapy may warrant further evaluation in the absence of more specific or targeted treatments. CSI combined with chemotherapy is effective for older non-metastatic patients. © The Author(s) 2016. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com
- Find related publications in this database (using NLM MeSH Indexing)
- Adolescent -
- Adult -
- Antineoplastic Agents - therapeutic use
- Brain Neoplasms - drug therapy
- Brain Neoplasms - mortality
- Brain Neoplasms - radiotherapy
- Brain Neoplasms - therapy
- Child -
- Child, Preschool -
- Combined Modality Therapy -
- Databases, Factual -
- Disease-Free Survival -
- Europe -
- Female -
- Humans -
- Infant -
- Male -
- Pineal Gland - drug effects
- Pineal Gland - pathology
- Pineal Gland - radiation effects
- Pinealoma - drug therapy
- Pinealoma - mortality
- Pinealoma - radiotherapy
- Pinealoma - therapy
- Prospective Studies -
- Treatment Outcome -
- United States -
- Young Adult -
- Find related publications in this database (Keywords)
- high-dose chemotherapy
- pediatric
- pineoblastoma
- radiotherapy
- treatment