RT Journal Article
T1 Pediatric Non-Rhabdomyosarcoma Soft Tissue Sarcomas: Standard of Care and Treatment Recommendations from the European Paediatric Soft Tissue Sarcoma Study Group (EpSSG).
A1 Ferrari, Andrea
A1 Brennan, Bernadette
A1 Casanova, Michela
A1 Corradini, Nadege
A1 Berlanga, Pablo
A1 Schoot, Reineke A
A1 Ramirez-Villar, Gema L
A1 Safwat, Akmal
A1 Guillen Burrieza, Gabriela
A1 Dall'Igna, Patrizia
A1 Alaggio, Rita
A1 Lyngsie Hjalgrim, Lisa
A1 Gatz, Susanne Andrea
A1 Orbach, Daniel
A1 van Noesel, Max M
K1 EpSSG
K1 NRSTS
K1 non-rhabdomyosarcoma soft tissue sarcomas
K1 pediatric
K1 recommendations
K1 treatment
AB This paper describes the standard of care for patients with non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) and the therapeutic recommendations developed by the European paediatric Soft tissue sarcoma Study Group (EpSSG). NRSTS form a very mixed group of mesenchymal extraskeletal malignancies. Their rarity, heterogeneity, and aggressiveness make the management of children and adolescents with these tumors complex and challenging. The overall cure rate for patients with NRSTS is around 70%, but survival depends on several prognostic variables, such as histotype and tumor grade, extent of disease and stage, tumor size, and tumor site. While surgery remains the mainstay of treatment for most of these tumors, a multimodal therapeutic approach including radiotherapy and chemotherapy is required in many cases. The EpSSG NRSTS 2005 study was the first prospective protocol tailored specifically to NRSTS. Together with the ARST0332 study developed by the North-American Soft Tissue Sarcoma Committee of the Children's Oncology Group (COG), the EpSSG NRSTS 2005 study currently represents the benchmark for these tumors, establishing risk-adapted standards of care. The EpSSG has developed common treatment recommendations for the large group of adult-type NRSTS (including synovial sarcoma), and specific treatment recommendations for other particular adult-type histologies (ie, alveolar soft-part sarcoma, clear cell sarcoma and dermatofibrosarcoma protuberans); other highly malignant tumors with a biology and clinical behavior differing from those of adult-type NRSTS (ie, rhabdoid tumors and desmoplastic small round cell tumor); and soft tissue tumors of intermediate malignancy (ie desmoid-type fibromatosis, inflammatory myofibroblastic tumors, and infantile fibrosarcoma). New effective drugs are needed for patients whose NRSTS carries the worst prognosis, ie, those with unresectable tumors, metastases at diagnosis, or relapsing disease. Progress in this area relies on our ability to develop international integrated prospective collaborations, both within existing pediatric oncology networks and, importantly, between the communities of specialists treating pediatric and adult sarcoma.
SN 1179-1322
YR 2022
FD 2022-09-23
LK http://hdl.handle.net/10668/20456
UL http://hdl.handle.net/10668/20456
LA en
DS RISalud
RD Apr 17, 2025