title: Ovarian Sertoli Leydig cell tumours in children and adolescents: an analysis of the European Cooperative Study Group on Pediatric Rare Tumors (EXPeRT).
abstract: OBJECTIVE
  To analyse ovarian Sertoli-Leydig cell tumours (SLCTs) for potential prognostic markers and their use for treatment stratification.
  
  
  METHODS
  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 24pts., Ic 17pts., II/III 3pts. 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 sixcycles 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.
authors.affiliation: Clinic of Pediatrics, Beurhausstr. 40, D-44137 Dortmund, Germany. Electronic address: dominik.schneider@klinikumdo.de.
authors.forename: Dominik T
authors.initials: DT
authors.lastname: Schneider
authors.affiliation: Department of Paediatric Oncology, Institut Curie, 26 rue d'Ulm, Paris 75231, France. Electronic address: daniel.orbach@curie.net.
authors.forename: Daniel
authors.initials: D
authors.lastname: Orbach
authors.affiliation: Department of Pediatrics, Pediatric Surgery, University of Padua, Via Giustiniani, 3, 35128 Padova, Italy. Electronic address: cecchett@pediatria.unipd.it.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Cecchetto
authors.affiliation: Department of Pediatric Hematology, Oncology and Endocrinology, Medical University Gdansk, 7 Debinki Street, 80-211 Gdansk, Poland. Electronic address: tsten@gumed.edu.pl.
authors.forename: Teresa
authors.initials: T
authors.lastname: Stachowicz-Stencel
authors.affiliation: Clinic of Pediatrics, Beurhausstr. 40, D-44137 Dortmund, Germany.
authors.forename: Bastian
authors.initials: B
authors.lastname: Brummel
authors.affiliation: Pediatric Oncology and Hematology, University Children's Hospital Erlangen, Loschgestrasse 15, D-91054 Erlangen, Germany. Electronic address: ines.brecht@uk-erlangen.de.
authors.forename: Ines B
authors.initials: IB
authors.lastname: Brecht
authors.affiliation: Department of Pediatrics, Pediatric Hematology and Oncology, University of Padua, Via Giustiniani, 3, 35128 Padova, Italy. Electronic address: gianni.bisogno@unipd.it.
authors.forename: Gianni
authors.initials: G
authors.lastname: Bisogno
authors.affiliation: Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Via G. Venezian 1, Milano 20133, Italy. Electronic address: andrea.ferrari@istitutotumori.mi.it.
authors.forename: Andrea
authors.initials: A
authors.lastname: Ferrari
authors.affiliation: Paediatric Department, Service d'oncologie pédiatrique, Centre hospitalo-universitaire, 4 rue Larrey, 49033 Angers Cedex 1, Angers, France. Electronic address: yvreguerre@chu-angers.fr.
authors.forename: Yves
authors.initials: Y
authors.lastname: Reguerre
authors.affiliation: Department of Pediatric Surgery, Marciniak Hospital and Department of Emergency Medicine, Wroclaw Medical University, Poland. Electronic address: jgodzin@dilnet.wroc.pl.
authors.forename: Jan
authors.initials: J
authors.lastname: Godzinski
authors.affiliation: Department of Pediatric Hematology, Oncology and Endocrinology, Medical University Gdansk, 7 Debinki Street, 80-211 Gdansk, Poland.
authors.forename: Ewa
authors.initials: E
authors.lastname: Bien
authors.affiliation: Clinic of Pediatric Hematology and Oncology, University Hospital Münster, Schweitzerstr. 33, D-48129 Münster, Germany. Electronic address: gabriele.calaminus@ukmuenster.de.
authors.forename: Gabriele
authors.initials: G
authors.lastname: Calaminus
authors.affiliation: Center of Pediatrics, Heinrich-Heine-University Düsseldorf, Germany. Electronic address: U.Gobelgoebelu@arcor.de.
authors.forename: Ulrich
authors.initials: U
authors.lastname: Göbel
authors.affiliation: Département d'oncologie pédiatrique, GHU Paris-Sud - CLCC Institut de cancérologie Gustave Roussy, 114 rue Edouard Vaillant, 94805 Villejuif, France. Electronic address: Catherine.PATTE@gustaveroussy.fr.
authors.forename: Catherine
authors.initials: C
authors.lastname: Patte
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D009367:Neoplasm Staging; D010051:Ovarian Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D018310:Sertoli-Leydig Cell Tumor / Q000401:mortality / Q000473:pathology / Q000628:therapy*
keywords: Chemotherapy; Ovarian cancer; Pediatric oncology; Rare tumours; Sex cord stromal tumours