Vall d'Hebron participates in the largest study on non-rhabdomyosarcoma pediatric soft tissue sarcomas

The pediatric surgeon Gabriela Guillén is a co-author in this study of the European Paediatric Soft Tissue Sarcoma Study Group (EpSSG) that lays the foundations for the treatment of this type of tumors.

15/08/2021

Members of the Translational Research Group in Childhood and Adolescent Cancer of the Vall d'Hebron Institute of Research (VHIR) and the Pediatric Oncologic Surgery Unit of the Vall d'Hebron University Hospital have been part of the largest study to date on pediatric non-rhabdomyosarcoma soft tissue sarcomas. This publication lays the foundation for a standardized treatment for non-rhabdomyosarcoma soft tissue sarcomas.

Non-rhabdomyosarcoma soft tissue sarcomas are a group of rare oncologic pathologies with more than 30 subtypes. This situation, in addition to a low incidence in pediatrics, meant that until now there was limited data available on their treatment. In most cases, experience with adult patients was used to establish treatment. With this publication, professionals will be able to define a new treatment scheme based on an appropriate selection of prognostic factors. In other words, treatment will be tailored to the needs of each patient.

The first European protocol

The EpSSG NRSTS 2005 Protocol is the first prospective European protocol specifically dedicated to the treatment of this rare type of sarcoma in children and adolescents. The results of the study, which included patients from more than 100 hospitals in 14 countries, have served to establish the basis for standardized treatment for the first time in children.

The study involved 1,321 patients registered in the NRSTS 2005 Protocol ("Non-Rhabdomyosarcoma Soft Tissue Sarcomas") of which 569 were finally included in the study, constituting the largest pediatric series of this type of tumor published to date. The study analyzes the results of two studies in patients under 21 years of age and with localized disease: the first for adult-type sarcomas and the second for synovial sarcomas. The results of the study, published in the journal The Lancet Child & Adolescent Health, have made it possible to define the new standard of treatment based on the risk of each patient in order to achieve the highest cure rates with the least possible sequelae.

The pediatric surgeon Gabriela Guillén, head of the Pediatric Oncologic Surgery Unit and Researcher of the Translational Research Group in Childhood and Adolescent Cancer at Vall d'Hebron Institute of Research (VHIR), is one of the co-authors of the work and highlights that "this work has shown that, in the case of small tumors in which a complete and oncological surgical resection is achieved, chemotherapy and radiotherapy treatments can be dispensed with".

Effective treatment for patients with high-grade tumors and large masses

Despite the overall good results, the article also highlights that patients with high-grade tumors and large masses have a high risk of metastasis, and therefore a worse prognosis. However, the publication demonstrates that in large, inoperable upfront masses, treatment with neoadjuvant chemotherapy with ifosfamide and doxorubicin allows tumor shrinkage prior to definitive surgery. This strategy aims to make it easier for tumors to be operated on than in previous studies.

Dr. Guillén explains that "the findings of this study, together with the one recently published by the American Childrens' Oncology Group, will make it possible to reduce the toxicity of the treatment in the short and long term, by confirming with certainty the role of surgery as a curative treatment in specific cases. To this end, it is a priority that patients with this type of tumor be treated in reference centers, with multidisciplinary teams specialized in their management. On the other hand, it is still a priority to identify new treatments for patients with poor prognosis, for whom there are still no effective therapies".

Vall d'Hebron is a national reference center CSUR, from Spanish Centres, Servicies and Units of Reference, in pediatric sarcomas and is part of the European Reference Network (ERN) in pediatric cancer "PaedCan", with its multidisciplinary team and high technology formed by the services of Pediatric Oncology, Pediatric Surgery, Orthopedic Surgery, Pathological Anatomy and Radiotherapy.

In addition, the Translational Research Group in Childhood and Adolescent Cancer at VHIR has a laboratory dedicated to the development of new drugs for pediatric sarcomas, led by Dr. Josep Roma, as well as a Precision Medicine program and clinical trials for childhood sarcomas.

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