Highlights
- •Developing research for non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) is challenging.
- •Recent tailored studies by cooperative groups have defined a standard of care for NRSTS.
- •A multimodal risk-adapted treatment approach is mandatory for patients with NRSTS.
- •International prospective dedicated programs with adults are needed.
Abstract
The current article focuses on non-rhabdomyosarcoma soft tissue sarcoma (NRSTS), the
heterogeneous group of mesenchymal tumours different from rhabdomyosarcoma that may
affect children and adolescents, with clinical behaviour varying from relatively benign
to highly malignant. This review represents the effort of the international scientific
paediatric community within the context of the INternational Soft Tissue SaRcoma ConsorTium
(INSTRuCT), a project founded by the leadership of three large cooperative groups
– Children's Oncology Group, Cooperative Weichteilsarkom Studiengruppe and European
paediatric Soft tissue sarcoma Study Group – with the main goal to pool expertise
and resources on a broader international level in order to improve knowledge of soft
tissue sarcomas of children, adolescents and young adults.
This article describes the current standard treatment approach in NRSTS, with a focus
on the controversies and challenges in the management of these tumours. Developing
research projects and clinical protocols for NRSTS has always been challenging, supporting
the need to develop international integrated prospective dedicated programs, including
paediatric NRSTS experts together with the adult sarcoma community. INSTRuCT provides
a unique mechanism to increase international collaboration by agreeing on a common
language, developing consensus standards to guide diagnosis and treatment, comparing
clinical trial results across cooperative groups, and through a shared data dictionary
providing answers to questions that can only be addressed by a larger data set.
Keywords
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Article info
Publication history
Published online: April 28, 2022
Accepted:
March 18,
2022
Received in revised form:
March 9,
2022
Received:
February 4,
2022
Identification
Copyright
© 2022 Elsevier Ltd. All rights reserved.
