Dominik T Schneider1, Daniel Orbach2, Giovanni Cecchetto3, Teresa Stachowicz-Stencel4, Bastian Brummel5, Ines B Brecht6, Gianni Bisogno7, Andrea Ferrari8, Yves Reguerre9, Jan Godzinski10, Ewa Bien11, Gabriele Calaminus12, Ulrich Göbel13, Catherine Patte14. 1. Clinic of Pediatrics, Beurhausstr. 40, D-44137 Dortmund, Germany. Electronic address: dominik.schneider@klinikumdo.de. 2. Department of Paediatric Oncology, Institut Curie, 26 rue d'Ulm, Paris 75231, France. Electronic address: daniel.orbach@curie.net. 3. Department of Pediatrics, Pediatric Surgery, University of Padua, Via Giustiniani, 3, 35128 Padova, Italy. Electronic address: cecchett@pediatria.unipd.it. 4. Department of Pediatric Hematology, Oncology and Endocrinology, Medical University Gdansk, 7 Debinki Street, 80-211 Gdansk, Poland. Electronic address: tsten@gumed.edu.pl. 5. Clinic of Pediatrics, Beurhausstr. 40, D-44137 Dortmund, Germany. 6. Pediatric Oncology and Hematology, University Children's Hospital Erlangen, Loschgestrasse 15, D-91054 Erlangen, Germany. Electronic address: ines.brecht@uk-erlangen.de. 7. Department of Pediatrics, Pediatric Hematology and Oncology, University of Padua, Via Giustiniani, 3, 35128 Padova, Italy. Electronic address: gianni.bisogno@unipd.it. 8. Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Via G. Venezian 1, Milano 20133, Italy. Electronic address: andrea.ferrari@istitutotumori.mi.it. 9. Paediatric Department, Service d'oncologie pédiatrique, Centre hospitalo-universitaire, 4 rue Larrey, 49033 Angers Cedex 1, Angers, France. Electronic address: yvreguerre@chu-angers.fr. 10. Department of Pediatric Surgery, Marciniak Hospital and Department of Emergency Medicine, Wroclaw Medical University, Poland. Electronic address: jgodzin@dilnet.wroc.pl. 11. Department of Pediatric Hematology, Oncology and Endocrinology, Medical University Gdansk, 7 Debinki Street, 80-211 Gdansk, Poland. 12. Clinic of Pediatric Hematology and Oncology, University Hospital Münster, Schweitzerstr. 33, D-48129 Münster, Germany. Electronic address: gabriele.calaminus@ukmuenster.de. 13. Center of Pediatrics, Heinrich-Heine-University Düsseldorf, Germany. Electronic address: U.Gobelgoebelu@arcor.de. 14. 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.
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 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.
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 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: Kris Ann P Schultz; Anne K Harris; Dominik T Schneider; Robert H Young; Jubilee Brown; David M Gershenson; Louis P Dehner; D Ashley Hill; Yoav H Messinger; A Lindsay Frazier Journal: J Oncol Pract Date: 2016-10 Impact factor: 3.840
Authors: Kris Ann P Schultz; Anne K Harris; Michael Finch; Louis P Dehner; Jubilee B Brown; David M Gershenson; Robert H Young; Amanda Field; Weiying Yu; Joyce Turner; Nicholas G Cost; Dominik T Schneider; Douglas R Stewart; A Lindsay Frazier; Yoav Messinger; D Ashley Hill Journal: Gynecol Oncol Date: 2017-10-14 Impact factor: 5.482
Authors: Kris Ann P Schultz; Gretchen M Williams; Junne Kamihara; Douglas R Stewart; Anne K Harris; Andrew J Bauer; Joyce Turner; Rachana Shah; Katherine Schneider; Kami Wolfe Schneider; Ann Garrity Carr; Laura A Harney; Shari Baldinger; A Lindsay Frazier; Daniel Orbach; Dominik T Schneider; David Malkin; Louis P Dehner; Yoav H Messinger; D Ashley Hill Journal: Clin Cancer Res Date: 2018-01-17 Impact factor: 12.531
Authors: Simone de Campos Vieira Abib; Chan Hon Chui; Sharon Cox; Abdelhafeez H Abdelhafeez; Israel Fernandez-Pineda; Ahmed Elgendy; Jonathan Karpelowsky; Pablo Lobos; Marc Wijnen; Jörg Fuchs; Andrea Hayes; Justin T Gerstle Journal: Ecancermedicalscience Date: 2022-02-17