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.