Research Article| Volume 51, ISSUE 4, P543-550, March 2015

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

Published:December 13, 2014DOI:



      To analyse ovarian Sertoli-Leydig cell tumours (SLCTs) for potential prognostic markers and their use for treatment stratification.


      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).


      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.


      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.


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        • Bisogno G.
        • Ferrari A.
        • Bien E.
        • Brecht I.B.
        • Brennan B.
        • Cecchetto G.
        • et al.
        Rare cancers in children – the EXPeRT initiative: a report from the European Cooperative Study Group on Pediatric Rare tumors.
        Klin Padiatr. 2012; 224: 416-420
        • Cecchetto G.
        • Ferrari A.
        • Bernini G.
        • Alaggio R.
        • Collini P.
        • Virgone C.
        • et al.
        Sex cord stromal tumors of the ovary in children: a clinicopathological report from the Italian TREP project.
        Pediatr Blood Cancer. 2011; 56: 1062-1067
        • De Corti F.
        • Sarnacki S.
        • Patte C.
        • Mosseri V.
        • Baranzelli M.C.
        • Martelli H.
        • et al.
        Prognosis of malignant sacrococcygeal germ cell tumours according to their natural history and surgical management.
        Surg Oncol. 2012; 21: e31-e37
        • Finan M.A.
        • Roberts W.S.
        • Kavanagh J.J.
        Ovarian Sertoli-Leydig cell tumor: success with salvage therapy.
        Int J Gynecol Cancer. 1993; 3: 189-191
        • Gui T.
        • Cao D.
        • Shen K.
        • Yang J.
        • Zhang Y.
        • Yu Q.
        • et al.
        A clinicopathological analysis of 40 cases of ovarian Sertoli-Leydig cell tumors.
        Gynecol Oncol. 2012; 127: 384-389
        • Hennes E.
        • Zahn S.
        • Lopes L.F.
        • Schonberger S.
        • Leuschner I.
        • Gobel U.
        • et al.
        Molecular genetic analysis of bilateral ovarian germ cell tumors.
        Klin Padiatr. 2012; 224: 359-365
        • Hersmus R.
        • Stoop H.
        • van de Geijn G.J.
        • Eini R.
        • Biermann K.
        • Oosterhuis J.W.
        • et al.
        Prevalence of c-KIT mutations in gonadoblastoma and dysgerminomas of patients with disorders of sex development (DSD) and ovarian dysgerminomas.
        PLoS One. 2012; 7: e43952
        • Hofmann M.
        • Schlegel P.G.
        • Hippert F.
        • Schmidt P.
        • von-Schweinitz D.
        • Leuschner I.
        • et al.
        Testicular sex cord stromal tumors: analysis of patients from the MAKEI study.
        Pediatr Blood Cancer. 2013;
        • Olson T.A.
        • Schneider D.T.
        • Brecht I.B.
        • Ferrari A.
        Rare tumors: a different perspective on oncology.
        in: Schneider D.T. Brecht I.B. Olson T.A. Ferrari A. Rare tumors in children and adolescents. Springer, 2012: 3-15
        • Rio Frio T.
        • Bahubeshi A.
        • Kanellopoulou C.
        • Hamel N.
        • Niedziela M.
        • Sabbaghian N.
        • et al.
        DICER1 mutations in familial multinodular goiter with and without ovarian Sertoli-Leydig cell tumors.
        JAMA. 2011; 305: 68-77
        • Roth L.M.
        • Anderson M.C.
        • Govan A.D.
        • Langley F.A.
        • Gowing N.F.
        • Woodcock A.S.
        Sertoli-Leydig cell tumors: a clinicopathologic study of 34 cases.
        Cancer. 1981; 48: 187-197
        • Schneider D.T.
        • Calaminus G.
        • Gobel U.
        Diagnostic value of alpha 1-fetoprotein and beta-human chorionic gonadotropin in infancy and childhood.
        Pediatr Hematol Oncol. 2001; 18: 11-26
        • Schneider D.T.
        • Calaminus G.
        • Wessalowski R.
        • Pathmanathan R.
        • Selle B.
        • Sternschulte W.
        • et al.
        Ovarian sex cord-stromal tumors in children and adolescents.
        J Clin Oncol. 2003; 21: 2357-2363
        • Schneider D.T.
        • Janig U.
        • Calaminus G.
        • Gobel U.
        • Harms D.
        Ovarian sex cord-stromal tumors–a clinicopathological study of 72 cases from the Kiel Pediatric Tumor Registry.
        Virchows Arch. 2003; 443: 549-560
        • Schneider D.T.
        • Terenziani M.
        • Cecchetto G.
        • Olson T.A.
        Gonadal and extragonadal germ cell tumors, sex cord stromal tumors and rare gonadal tumors.
        in: Schneider D.T. Brecht I.B. Oslon T.A. Ferrari A. Rare tumors in children and adolescents. Springer, Heidelberg2012: 327-402
        • Schultz K.A.
        • Pacheco M.C.
        • Yang J.
        • Williams G.M.
        • Messinger Y.
        • Hill D.A.
        • et al.
        Ovarian sex cord-stromal tumors, pleuropulmonary blastoma and DICER1 mutations: a report from the International Pleuropulmonary Blastoma Registry.
        Gynecol Oncol. 2011; 122: 246-250
        • Schultze-Florey R.E.
        • Graf N.
        • Vorwerk P.
        • Koscielniak E.
        • Schneider D.T.
        • Kratz C.P.
        DICER1 syndrome: a new cancer syndrome.
        Klin Padiatr. 2013; 10: 10-15
        • Sigismondi C.
        • Gadducci A.
        • Lorusso D.
        • Candiani M.
        • Breda E.
        • Raspagliesi F.
        • et al.
        Ovarian Sertoli-Leydig cell tumors. A retrospective MITO study.
        Gynecol Oncol. 2012; 125: 673-676
        • Slade I.
        • Bacchelli C.
        • Davies H.
        • Murray A.
        • Abbaszadeh F.
        • Hanks S.
        • et al.
        DICER1 syndrome: clarifying the diagnosis, clinical features and management implications of a pleiotropic tumour predisposition syndrome.
        J Med Genet. 2011; 48: 273-278
        • Young R.H.
        • Perez-Atayde A.R.
        • Scully R.E.
        Ovarian Sertoli-Leydig cell tumor with retiform and heterologous components. Report of a case with hepatocytic differentiation and elevated serum alpha-fetoprotein.
        Am J Surg Pathol. 1984; 8: 709-718
        • Young R.H.
        • Scully R.E.
        Ovarian Sertoli-Leydig cell tumors with a retiform pattern: a problem in histopathologic diagnosis. A report of 25 cases.
        Am J Surg Pathol. 1983; 7: 755-771
        • Young R.H.
        • Scully R.E.
        Ovarian Sertoli-Leydig cell tumors. A clinicopathological analysis of 207 cases.
        Am J Surg Pathol. 1985; 9: 543-569