Consensus Recommendations from EXPeRT/PARTN-ER Groups for the Diagnosis
and Therapy of Sex Cord Stromal Tumors in Children and Adolescents
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.