Abstract
The HIT-SIOP-PNET4 randomised trial for standard risk medulloblastoma (MB) (2001–2006) included 338 patients and compared hyperfractionated and conventional radiotherapy. We here report the long-term outcome after a median follow up of 7.8 years, including detailed information on relapse and the treatment of relapse. Data were extracted from the HIT Group Relapsed MB database and by way of a specific case report form. The event-free and overall (OS) survival at 10 years were 76 ± 2 % and 78 ± 2 % respectively with no significant difference between the treatment arms. Seventy-two relapses and three second malignant neoplasms were reported. Thirteen relapses (18 %) were isolated local relapses in the posterior fossa (PF) and 59 (82 %) were craniospinal, metastatic relapses (isolated or multiple) with or without concurrent PF disease. Isolated PF relapse vs all other relapses occurred at mean/median of 38/35 and 28/26 months respectively (p = 0.24). Late relapse, i.e. >5 years from diagnosis, occurred in six patients (8 %). Relapse treatment consisted of combinations of surgery (25 %), focal radiotherapy (RT 22 %), high dose chemotherapy with stem cell rescue (HDSCR 21 %) and conventional chemotherapy (90 %). OS at 5 years after relapse was 6.0 ± 4 %. In multivariate analysis; isolated relapse in PF, and surgery were significantly associated with prolonged survival whereas RT and HDSCR were not. Survival after relapse was not related to biological factors and was very poor despite several patients receiving intensive treatments. Exploration of new drugs is warranted, preferably based on tumour biology from biopsy of the relapsed tumour.
| Original language | English |
|---|---|
| Pages (from-to) | 1-10 |
| Number of pages | 10 |
| Journal | Journal of Neuro-Oncology |
| DOIs | |
| Publication status | Accepted/In press - Jul 16 2016 |
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Keywords
- Chemotherapy
- Clinical trial
- Medulloblastoma
- Paediatric
- Radiotherapy
- Relapse
- Secondary tumours
- Survival
- Treatment
ASJC Scopus subject areas
- Clinical Neurology
- Cancer Research
- Oncology
- Neurology
Cite this
Relapse patterns and outcome after relapse in standard risk medulloblastoma : a report from the HIT-SIOP-PNET4 study. / Sabel, Magnus; Fleischhack, Gudrun; Tippelt, Stephan; Gustafsson, Göran; Doz, François; Kortmann, Rolf; Massimino, Maura; Navajas, Aurora; von Hoff, Katja; Rutkowski, Stefan; Warmuth-Metz, Monika; Clifford, Steven C.; Pietsch, Torsten; Pizer, Barry; Lannering, Birgitta; On Behalf Of The Siop-E Brain Tumour Group, Behalf Of The Siop-E Brain Tumour Group.
In: Journal of Neuro-Oncology, 16.07.2016, p. 1-10.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Relapse patterns and outcome after relapse in standard risk medulloblastoma
T2 - a report from the HIT-SIOP-PNET4 study
AU - Sabel, Magnus
AU - Fleischhack, Gudrun
AU - Tippelt, Stephan
AU - Gustafsson, Göran
AU - Doz, François
AU - Kortmann, Rolf
AU - Massimino, Maura
AU - Navajas, Aurora
AU - von Hoff, Katja
AU - Rutkowski, Stefan
AU - Warmuth-Metz, Monika
AU - Clifford, Steven C.
AU - Pietsch, Torsten
AU - Pizer, Barry
AU - Lannering, Birgitta
AU - On Behalf Of The Siop-E Brain Tumour Group, Behalf Of The Siop-E Brain Tumour Group
PY - 2016/7/16
Y1 - 2016/7/16
N2 - The HIT-SIOP-PNET4 randomised trial for standard risk medulloblastoma (MB) (2001–2006) included 338 patients and compared hyperfractionated and conventional radiotherapy. We here report the long-term outcome after a median follow up of 7.8 years, including detailed information on relapse and the treatment of relapse. Data were extracted from the HIT Group Relapsed MB database and by way of a specific case report form. The event-free and overall (OS) survival at 10 years were 76 ± 2 % and 78 ± 2 % respectively with no significant difference between the treatment arms. Seventy-two relapses and three second malignant neoplasms were reported. Thirteen relapses (18 %) were isolated local relapses in the posterior fossa (PF) and 59 (82 %) were craniospinal, metastatic relapses (isolated or multiple) with or without concurrent PF disease. Isolated PF relapse vs all other relapses occurred at mean/median of 38/35 and 28/26 months respectively (p = 0.24). Late relapse, i.e. >5 years from diagnosis, occurred in six patients (8 %). Relapse treatment consisted of combinations of surgery (25 %), focal radiotherapy (RT 22 %), high dose chemotherapy with stem cell rescue (HDSCR 21 %) and conventional chemotherapy (90 %). OS at 5 years after relapse was 6.0 ± 4 %. In multivariate analysis; isolated relapse in PF, and surgery were significantly associated with prolonged survival whereas RT and HDSCR were not. Survival after relapse was not related to biological factors and was very poor despite several patients receiving intensive treatments. Exploration of new drugs is warranted, preferably based on tumour biology from biopsy of the relapsed tumour.
AB - The HIT-SIOP-PNET4 randomised trial for standard risk medulloblastoma (MB) (2001–2006) included 338 patients and compared hyperfractionated and conventional radiotherapy. We here report the long-term outcome after a median follow up of 7.8 years, including detailed information on relapse and the treatment of relapse. Data were extracted from the HIT Group Relapsed MB database and by way of a specific case report form. The event-free and overall (OS) survival at 10 years were 76 ± 2 % and 78 ± 2 % respectively with no significant difference between the treatment arms. Seventy-two relapses and three second malignant neoplasms were reported. Thirteen relapses (18 %) were isolated local relapses in the posterior fossa (PF) and 59 (82 %) were craniospinal, metastatic relapses (isolated or multiple) with or without concurrent PF disease. Isolated PF relapse vs all other relapses occurred at mean/median of 38/35 and 28/26 months respectively (p = 0.24). Late relapse, i.e. >5 years from diagnosis, occurred in six patients (8 %). Relapse treatment consisted of combinations of surgery (25 %), focal radiotherapy (RT 22 %), high dose chemotherapy with stem cell rescue (HDSCR 21 %) and conventional chemotherapy (90 %). OS at 5 years after relapse was 6.0 ± 4 %. In multivariate analysis; isolated relapse in PF, and surgery were significantly associated with prolonged survival whereas RT and HDSCR were not. Survival after relapse was not related to biological factors and was very poor despite several patients receiving intensive treatments. Exploration of new drugs is warranted, preferably based on tumour biology from biopsy of the relapsed tumour.
KW - Chemotherapy
KW - Clinical trial
KW - Medulloblastoma
KW - Paediatric
KW - Radiotherapy
KW - Relapse
KW - Secondary tumours
KW - Survival
KW - Treatment
UR - http://www.scopus.com/inward/record.url?scp=84978906225&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84978906225&partnerID=8YFLogxK
U2 - 10.1007/s11060-016-2202-1
DO - 10.1007/s11060-016-2202-1
M3 - Article
SP - 1
EP - 10
JO - Journal of Neuro-Oncology
JF - Journal of Neuro-Oncology
SN - 0167-594X
ER -