Ovarian Sex Cord Stromal Tumors in Children and Adolescents—The European Standard Clinical Practice Recommendations

As part of the European Cooperative Study Group for Paediatric Rare Tumours initiative, we developed standard clinical practice guidelines for ovarian sex cord stromal tumors, based on comprehensive national and international cohort analyses, literature review, and a final expert consensus conference. Complete tumor resection is the cornerstone of treatment, with meticulous attention to preventing tumor spillage. Risk stratification of adjuvant chemotherapy decisions incorporates tumor stage and critical parameters, including histological differentiation and mitotic rate. Optimal patient management requires treatment within cooperative networks providing centralized histopathological review and comprehensive genetic testing, multidisciplinary tumor board evaluation, and prospective registry enrollment to advance knowledge for these exceptionally rare malignancies.