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Sertoli–Leydig cell tumor: a clinicopathological analysis in a comprehensive, national cohort
  1. Pernille Bekker1,
  2. Anne Regitze Miland-Samuelsen1,
  3. Maja Patricia Smerdel2,
  4. Tine Henriksen Schnack3,
  5. Finn Friis Lauszus1 and
  6. Sven Hoedt Karstensen1
  1. 1Department of Gynaecology and Obstetrics, Sygehus Sønderjylland i Aabenraa, Aabenraa, Denmark
  2. 2Department of Clinical Genetics, Lillebaelt Hospital-University Hospital of Southern Denmark, Vejle, Denmark
  3. 3Gynecology and Obstetrics, Odense University Hospital, Odense, Denmark
  1. Correspondence to Dr Finn Friis Lauszus, Department of Gynaecology and Obstetrics, Sygehus Sønderjylland i Aabenraa, Aabenraa, Denmark; finn.lauszus{at}


Introduction Sertoli–Leydig cell tumors are rare tumors of the ovary. Moderate and poorly differentiated tumors can metastasize and have a poor outcome. A pathogenic variant in DICER1 is associated with an increased risk of developing these tumors along with other clinical phenotypes. We aimed to describe a national cohort of all Sertoli–Leydig cell tumors with regard to clinicopathological characteristics and frequency of DICER1 pathogenic variants.

Methods In May 2018, all patients registered from January 1997 to December 2017 with the Systematized Nomenclature of Medicine code M86310 (Sertoli–Leydig cell tumor) were obtained from the Danish National Pathology Registry. Validation of the diagnosis depended on comments in the reports that two pathologists validated the initial diagnosis or revision of the pathology at another facility. We performed descriptive statistics to describe baseline characteristics, and cancer related survival was calculated using Kaplan–Meier analysis followed by a log rank test for differences between variables

Results 41 women with Sertoli–Leydig cell tumors were identified. Median age was 41 years (range 6–79). The stages according to the International Federation of Gynecology and Obstetrics (FIGO) were: stage I, 85% (n=35), stage II, 2% (n=1), stage III, 5% (n=2), and stage IV, 7% (n=3). The 5 year cancer related survival was 100% for patients with localized disease (stages I–II) and 0% in advanced tumor stages (stages III–IV). Histological differentiation grade of the tumors was well differentiated in 29% (n=12), moderately differentiated in 56% (n=23), and poorly differentiated in 15% (n=6), and the 5 year cancer related survival was 100%, 96%, and 33%, respectively, according to grade. All patients underwent surgery. Twenty-two patients had fertility sparing surgery and four of these had given birth at the time of follow-up. Analysis of DICER1 was performed in eight women. Four carried a pathogenic variant. Four patients received adjuvant chemotherapy, three because of advanced tumor stage, and one because of a poorly differentiated Sertoli–Leydig cell tumor.

Conclusion The prognosis for women with Sertoli–Leydig cell tumors with localized disease is excellent. Women with advanced stages (III–IV) have a poor prognosis, regardless of adjuvant chemotherapy. Fertility sparing surgery seems to be a viable option for localized Sertoli–Leydig cell tumors. DICER1 screening was rarely performed in previous cohorts and concomitant organ screening programs are topics for discussion.

  • Sertoli-Leydig Cell Tumor
  • Ovarian Cancer
  • Sex Cord-Gonadal Stromal Tumors

Data availability statement

Data are available upon reasonable request.

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Data availability statement

Data are available upon reasonable request.

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  • PB and ARM-S contributed equally.

  • Contributors FFL is guarantor accepts full responsibility for the work and the conduct of the study, had access to the data, and controlled the decision to publish.

    SHK and FFL: conceptualizing the project, validating the data, and writing of the manuscript. PB and ARM-S: conceptualizing, gathering the data, and writing the manuscript. MPS: validating the data and writing the manuscript. THS: writing the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial, or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.