Ovarian Sertoli Leydig cell tumours in children and adolescents: An analysis of the European Cooperative Study Group on Pediatric Rare Tumors (EXPeRT)

Dominik T. Schneider, Daniel Orbach, Giovanni Cecchetto, Teresa Stachowicz-Stencel, Bastian Brummel, Ines B. Brecht, Gianni Bisogno, Andrea Ferrari, Yves Reguerre, Jan Godzinski, Ewa Bien, Gabriele Calaminus, Ulrich Göbel, Catherine Patte

Research output: Contribution to journalArticle

Abstract

Objective To analyse ovarian Sertoli-Leydig cell tumours (SLCTs) for potential prognostic markers and their use for treatment stratification. Patients Forty-four patients were included. Patients were prospectively reported to the German MAKEI (Maligne Keimzelltumoren) studies (n = 23), French TGM protocols (n = 10), Italian Rare Tumour Project (TREP) registry (n = 6), and the Polish Pediatric Rare Tumour Study group (n = 5). Tumours were classified according to World Health Organisation (WHO) and staged according to International Federation of Gynecological Oncology (FIGO). Results Median age was 13.9 (0.5-17.4) years. All patients underwent resection by tumour enucleation (n = 8), ovariectomy (n = 17), adenectomy isolated (n = 18) or with hysterectomy (n = 1). FIGO-stage: Ia 24 pts., Ic 17 pts., II/III 3 pts. One patient had bilateral tumours. Four patients (stage Ia: 3, stage Ic: 1) developed a metachronous contralateral tumour. Otherwise, all stage Ia patients remained in complete remission. Among 20 patients with incomplete resection or tumour spread (stage Ic-III), eight relapsed, and five patients died. Eleven patients were initially treated with two to six cycles of cisplatin-based chemotherapy. Of these, seven patients are in continuous remission. Poor histological differentiation was associated with higher relapse rate (5/13) compared to intermediate (3/18) and high differentiation (0/4). Tumours with retiform pattern or heterologous elements showed a high relapse rate, too (5/11). After a median follow-up of 62 months, event-free survival is 0.70 ± 0.07, relapse-free survival 0.81 ± 0.06 and overall survival 0.87 ± 0.05. Conclusions Prognosis of SLCTs is determined by stage and histopathologic differentiation. Complete resection with careful avoidance of spillage is a prerequisite of cure. The impact of chemotherapy in incompletely resected and advanced stage tumours remains to be evaluated.

Original languageEnglish
Pages (from-to)543-550
Number of pages8
JournalEuropean Journal of Cancer
Volume51
Issue number4
DOIs
Publication statusPublished - 2015

Keywords

  • Chemotherapy
  • Ovarian cancer
  • Pediatric oncology
  • Rare tumours
  • Sex cord stromal tumours

ASJC Scopus subject areas

  • Cancer Research
  • Oncology
  • Medicine(all)

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