TY  - JOUR
A1  - Sabel, Magnus
A1  - Fleischhack, Gudrun
A1  - Tippelt, Stephan
A1  - Gustafsson, Göran
A1  - Doz, François
A1  - Kortmann, Rolf
A1  - Massimino, Maura
A1  - Navajas, Aurora
A1  - von Hoff, Katja
A1  - Rutkowski, Stefan
A1  - Warmuth-Metz, Monika
A1  - Clifford, Steven C.
A1  - Pietsch, Torsten
A1  - Pizer, Barry
A1  - Linnering, Birgitta
T1  - Relapse patterns and outcome after relapse in standard risk medulloblastoma: a report from the HIT-SIOP-PNET4 study
JF  - Journal of Neurooncology
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.
KW  - High-dose chemotherapy
KW  - Childhood medulloblastoma
KW  - Adolescents
KW  - Primitive neuroectodermal
KW  - Tumors
KW  - Recurrent medulloblastoma
KW  - Childrens-cancer
KW  - Phase-II
KW  - Trial
KW  - Therapy
KW  - Reirradiation
KW  - Medulloblastoma
KW  - Relapse
KW  - Survival
KW  - Treatment
KW  - Clinical trial
KW  - Chemotherapy
KW  - Radiotherapy
KW  - Paediatric
KW  - Secondary tumours
Y1  - 2016
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-187498
VL  - 129
IS  - 3
ER  -