Onkologie. 2016:10(5):224-227 | DOI: 10.36290/xon.2016.048

The rhabdomyosarcoma treatment in children

Ondřej Rohleder, Kristýna Melichárková, Pavel Mazánek, Viera Bajčiová, Peter Múdry

Klinika dětské onkologie LF MU a FN Brno, Dětská nemocnice Brno

The rhabdomyosarcoma is the most frequent soft tissue sarcoma in childhood. Current treatment standards are defined by EpSS

and COG cooperative groups. Risk adapted treatment is adopted for different prognostic groups of patients with long term overall

survival within range of 60–80 %. New treatment options in Europe randomize irinotecan and mTOR inhibitor to standards in

high risk and very high risk patients. Maintenance therapy is scheduled for period of 6-24 months according to risk group. Locoregional

therapy will be randomized as pre – or postsurgery, standard or higher radiotherapy dose for post induction FDG PET

positive patients with tumor size up to 5 cm, all metastatic sites or limited locoregional irradiation for patients with more then

three metastatic sites. Relapsed patient will be in Europe randomized to three arms with addition of doxorubicin or temsirolimus

to vincristine/irinotecan backbone. COG group defines temsirolimus as better option for relapsed patients compared to bevacizumab.

Patients with relapse or high risk metastatic disease should be treated in clinical trial.

Keywords: rhabdomyosarcoma, soft tissue sarcoma, mTOR inhibitor, doxorubicin, irinotecan

Published: November 1, 2016  Show citation