Consensus Recommendations from EXPeRT/PARTN-ER Groups for the Diagnosis and Therapy of Sex Cord Stromal Tumors in Children and Adolescents
Year: 2021
Type: preprint
Abstract: As part of the European Union-funded project designated PARTN-ER, the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) is continuously developing consensus recommendations in order to harmonize standard care for very rare solid tumors of children and adolescents. This paper presents the internationally recognized recommendations for the diagnosis and treatment of sex cord stromal tumors (SCST). The clinical approach to sex cord stromal tumors of the testis (TSCST) and ovary (OSCST) depends on histological differentiation and tumor stage. Virtually all TSCSTs present as localized non-metastatic tumors, with excellent prognosis after complete resection. In contrast, the prognosis of OSCSTs may be adversely affected by tumor spillage during surgery or presence of metastases. In these cases, cisplatin-based chemotherapy is recommended. Of note, some SCSTs may develop in the context of tumor predisposition syndromes e.g. DICER-1, so that specific follow-up is indicated. SCSTs should be diagnosed and treated according to standardized recommendations that include reference pathology, genetic testing for tumor predisposition syndromes in selected cases, and stratified adjuvant chemotherapy in patients with unfavorable risk profile. To ensure high quality of diagnosis and therapy, patients should be enrolled into prospective registries.
Institutions: Klinikum Dortmund, Université Paris Sciences et Lettres, Institut Curie, Kaplan Medical Center, Gdańsk Medical University, University of Padua, University of Tübingen, Fondazione IRCCS Istituto Nazionale dei Tumori, Wroclaw Medical University, Szpital im Tadeusza Marciniaka, University of Belgrade, University Children's Hospital, Belgrade, Hospital de Cruces, Panagiotis & Aglaia Kyriakou Children's Hospital, University of Rijeka, Klinički Bolnički Centar Rijeka, University of Minnesota, Children's Minnesota, Université Paris-Sud, Université Paris-Saclay, Institut Gustave Roussy
Language: English
Subfield: Surgery
Field: Medicine
Domain: Health Sciences
Open Access status: green
Funder: European Commission
OpenAlex ID: W3141822620