uu.seUppsala University Publications
Change search
ReferencesLink to record
Permanent link

Direct link
Tumour volume reduction after neoadjuvant chemotherapy impacts outcome in localised embryonal rhabdomyosarcoma
Paediatrics 5 (oncology, hematology, immunology), Olgahospital Klinikum Stuttgart, Germany.
Paediatrics 5 (oncology, hematology, immunology), Olgahospital Klinikum Stuttgart, Germany.
Show others and affiliations
2015 (English)In: Pediatric Blood & Cancer, ISSN 1545-5009, E-ISSN 1545-5017, Vol. 62, no 1, 16-23 p.Article in journal (Refereed) Published
Abstract [en]

BACKGROUND: Response (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.

PROCEDURE: Patients 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).

RESULTS: Fifty-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 >5 cm 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 76 ± 4% for PAR, 79 ± 14% for OR, but only 40 ± 19% for SPD (P < 0.001).

CONCLUSION: Response to induction chemotherapy appears to be an important surrogate marker of poor outcome in patients with SPD largely due to ineffective local control.

Place, publisher, year, edition, pages
2015. Vol. 62, no 1, 16-23 p.
National Category
Pediatrics Hematology
URN: urn:nbn:se:uu:diva-240257DOI: 10.1002/pbc.25207ISI: 000345319300005PubMedID: 25263634OAI: oai:DiVA.org:uu-240257DiVA: diva2:776085
Available from: 2015-01-06 Created: 2015-01-06 Last updated: 2015-01-08Bibliographically approved

Open Access in DiVA

No full text

Other links

Publisher's full textPubMed

Search in DiVA

By author/editor
Ljungman, Gustaf
By organisation
In the same journal
Pediatric Blood & Cancer

Search outside of DiVA

GoogleGoogle Scholar
The number of downloads is the sum of all downloads of full texts. It may include eg previous versions that are now no longer available

Altmetric score

Total: 280 hits
ReferencesLink to record
Permanent link

Direct link