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Consensus Recommendations from EXPeRT/PARTN-ER Groups for the Diagnosis and Therapy of Sex Cord Stromal Tumors in Children and Adolescents
  • +13
  • Dominik T. Schneider,
  • Daniel Orbach,
  • Tal Ben-Ami,
  • Ewa Bien,
  • Gianni Bisogno,
  • Ines Brecht,
  • Giovanni Cecchetto,
  • Andrea Ferrari,
  • Jan Godziński,
  • Dragana Janic,
  • Ricardo Lopez,
  • Apostolos Pourtsidis,
  • Jelena Roganovic,
  • Kris Ann Schultz,
  • Teresa Stachowicz-Stencel,
  • Brice Fresneau
Dominik T. Schneider
Klinikum Dortmund
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Daniel Orbach
Institut Curie
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Tal Ben-Ami
Kaplan Medical Center
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Ewa Bien
Medical University, Gdansk
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Gianni Bisogno
Division of Hematology/Oncology
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Ines Brecht
University of Tübingen
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Giovanni Cecchetto
Universita degli Studi di Padova Scuola di Medicina e Chirurgia
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Andrea Ferrari
Istituto Nazionale Tumori, Milano
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Jan Godziński
Marciniak Hospital
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Dragana Janic
University of Belgrade, University of Children's Hospital
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Ricardo Lopez
Cruces University Hospital
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Apostolos Pourtsidis
Panagiotis and Aglaia Kyriakou Children's Hospital
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Jelena Roganovic
Clinical Hospital Center Rijeka
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Kris Ann Schultz
University of Minnesota
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Teresa Stachowicz-Stencel
Medical University of Gdansk
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Brice Fresneau
GUSTAVE ROUSSY
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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.

Peer review status:ACCEPTED

14 Feb 2021Assigned to Editor
14 Feb 2021Submission Checks Completed
14 Feb 2021Submitted to Pediatric Blood & Cancer
16 Feb 2021Reviewer(s) Assigned
18 Feb 2021Review(s) Completed, Editorial Evaluation Pending
02 Mar 2021Editorial Decision: Accept