Journal
PEDIATRIC BLOOD & CANCER
Volume 62, Issue 1, Pages 16-23Publisher
WILEY
DOI: 10.1002/pbc.25207
Keywords
chemotherapy; embryonal rhabdomyosarcoma; response
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BackgroundResponse (tumour volume reduction) to induction chemotherapy has been used to stratify secondary local and systemic treatment of Intergroup Rhabdomyosarcoma Study Group III (IRSG-III) embryonal rhabdomyosarcoma (RME) in consecutive CWS-trials. To evaluate its actual impact we studied response-related treatment and outcomes. ProcedurePatients with IRSG-III RME <21 years and non-response (NR, <33% volume reduction) in five consecutive CWS-trials were analysed and compared with partial responders (PAR, 33% reduction). The NR was reviewed and sub-classified as Objective Response (OR, <0%-33% reduction) or Stable/Progressive Disease (SPD). ResultsFifty-nine of 529 patients had NR (n=34 OR, n=25 SPD). Primary risk-factors including age, tumour size, and TN-classification did not differ between NR and PAR groups but NR had more patients with unfavourable sites comparatively (P=0.04). There were no differences in primary risk-factors between OR and SPD. Significant factors associated with poor outcome in multivariate analysis were NR, TN-classification, age >10 years, tumour size >5cm and therapy in older trials. After response assessment n=24 NR continued to receive induction chemotherapy, n=32 received other combinations and n=3 no further chemotherapy. Forty-two non-responders were irradiated, and the tumours were completely resected in n=20. After a median follow-up of 8 years, 34 NR are alive. Seventeen of 21 failures leading to disease-related deaths were locoregional. The five-year overall survival rate (OS) was 764% for PAR, 79 +/- 14% for OR, but only 40 +/- 19% for SPD (P<0.001). ConclusionResponse to induction chemotherapy appears to be an important surrogate marker of poor outcome in patients with SPD largely due to ineffective local control. Pediatr Blood Cancer 2015;62:16-23. (c) 2014 Wiley Periodicals, Inc.
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