Outcomes in paediatric metastatic rhabdomyosarcoma: Results of The International Society of Paediatric Oncology (SIOP) study MMT-98

Autor: A.B.M. Foot, H.P. McDowell, David Machin, Cécile Giraud, Christophe Bergeron, C. Ellershaw
Rok vydání: 2010
Předmět:
Zdroj: European Journal of Cancer. 46:1588-1595
ISSN: 0959-8049
DOI: 10.1016/j.ejca.2010.02.051
Popis: Results are presented of the SIOP study MMT-98 for paediatric metastatic rhabdomyosarcoma (RMS), which evaluated intensive chemotherapy followed by low intensity 'maintenance' chemotherapy in standard risk patients (SRG). For poor risk patients (PRG), the value of a therapeutic window study, sequential high dose monotherapy to achieve a complete response (CR) followed by low dose maintenance chemotherapy was examined.From November 1998 to 2005, 146 patients aged 6 months to 18 years with metastatic RMS were entered. Forty-five were SRG, i.e. age10 years and no bone marrow or bone involvement. Treatment was a 6-drug regimen with local therapy of surgery and/or radiotherapy followed by maintenance of 9 courses of vincristine, actinomycin D and cyclophosphamide (VAC). One hundred and one patients were PRG, i.e.10 years, or with bone marrow or bone metastases. An upfront window study, high dose monotherapy, local treatment and then VAC maintenance therapy were given.With a median follow-up of 1.52 years, the 3-year event-free survival (EFS) and overall survival (OS) for SRG were 54.92% and 62.14%, respectively, whilst for the PRG 16.17% and 23.17%. The corresponding adverse hazard ratio (HR) for the PRG was HR=2.65 (95% CI 1.63-4.31, p-value0.001) for EFS and HR=2.51 (CI 1.53-4.11, p-value0.001) for OS.SRG patients' EFS and OS were comparable to those of previous studies. For PRG patients there was no improvement in survival.
Databáze: OpenAIRE