Embryonal rhabdomyosarcoma completely resected at diagnosis: The European paediatric Soft tissue sarcoma Study Group RMS2005 experience
| Autor: | Christophe Bergeron, Meriel Jenney, Federica De Corti, Soledad Gallego, Hans Merks, Heidi Glosli, Andrea Ferrari, Dominique Ranchère-Vince, Gian Luca De Salvo, Ilaria Zanetti, Julia Chisholm, Véronique Minard-Colin, Timothy Rogers, Gianni Bisogno, Adriana Rose, Christine Devalck, Sima Ferman, Peter Mudry, Myriam Weyl Ben Arush, Daniela Sejnova, Maja Cesen, J. Hans Merks |
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| Rok vydání: | 2021 |
| Předmět: |
Male
0301 basic medicine Cancer Research medicine.medical_treatment 0302 clinical medicine Risk Factors Antineoplastic Combined Chemotherapy Protocols Rhabdomyosarcoma Rhabdomyosarcoma Embryonal Prospective Studies Stage (cooking) Child media_common Soft tissue sarcoma Ifosfamide Prognosis Combined Modality Therapy Survival Rate Oncology Vincristine Child Preschool 030220 oncology & carcinogenesis Dactinomycin Female Childhood cancer medicine.drug medicine.medical_specialty Adolescent 03 medical and health sciences Internal medicine medicine Humans media_common.cataloged_instance European union Cyclophosphamide Retrospective Studies Chemotherapy business.industry Infant medicine.disease 030104 developmental biology Doxorubicin Embryonal rhabdomyosarcoma business Follow-Up Studies |
| Zdroj: | European Journal of Cancer. 146:21-29 |
| ISSN: | 0959-8049 |
| Popis: | Rhabdomyosarcoma (RMS) is the most common form of soft tissue sarcoma in children. We report the results of the European paediatric Soft tissue sarcoma Study Group (EpSSG) RMS 2005 study, which prospectively evaluated the reduction of chemotherapy in patients with embryonal RMS (ERMS) after initial surgery.Between October 2005 and December 2016, all patients with localised ERMS with an initial microscopically complete resection (IRS group I) with lymph node-negative (N0) were prospectively enrolled in the low-risk (n = 70, subgroup A; age 10 years and tumour size ≤ 5 cm) or standard-risk group (n = 108, subgroup B; age ≥ 10 years or tumour size 5 cm. Subgroup A received 8 courses of vincristine and dactinomycin (VA) for 22 weeks; subgroup B received 4 courses of VA with ifosfamide (IVA) and 5 courses of VA for 25 weeks.The 5-year event-free survival (EFS) and overall survival (OS) were 90.8% (95% confidence interval [CI]: 85.0-94.4) and 95.7% (95% CI: 90.5-98.1), respectively (n = 178). The EFS and OS were 95.5% (95% CI: 86.8-98.5) and 100% (subgroupA), and 87.8% (95% CI: 79.3-93.0) and 93.0% (95% CI: 84.8-96.8)(subgroup B), respectively. Bearman stage 2 veno-occlusive disease (VOD) occurred in 4 very young patients.VA treatment for 8 courses was effective and well tolerated by the subgroup of patients with low-risk ERMS (group A). Four courses of IVA and 5 courses of VA instead of 9 courses of IVA also has very good results. Careful monitoring for liver toxicity is important in very young patients. European union drug regulating authorities clinical trials EUDRACT No. 2005-000217-35. |
| Databáze: | OpenAIRE |
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