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Ovarian sex cord–stromal tumors—a clinicopathological study of 72 cases from the Kiel Pediatric Tumor Registry

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Abstract

We analyzed 72 patients with ovarian sex cord–stromal tumors (OSCST) registered at the German Pediatric Tumor Registry in Kiel over a 20-year period. Juvenile granulosa cell tumors (JGCT, n=48) were the most frequent histological subtype. In addition, there were 14 Sertoli-Leydig cell tumors, 5 sclerosing stromal tumors, 2 sex cord tumors with annular tubules, 2 thecomas and 1 steroid cell tumor. Stage according to FIGO (International Federation of Gynecologists and Obstetricians) was Ia in 39 patients, Ic in 17 patients, II in 3 patients and III in 1 patient (60 patients with complete data). Compared with adult granulosa cell tumors, JGCT showed pronounced mitotic activity [mean 9.8 mitoses/10 high power field (HPF)], which was significantly higher than in other histological subtypes (2.7/10 HPF, P=0.001). Immunohistochemical analysis revealed frequent coexpression of vimentin (positive in 52/52 examined tumors), cytokeratin (27/33), and inhibin (19/20). Of patients, 12 with Ic or higher stage tumors received adjuvant cisplatinum-based chemotherapy. Event-free survival at 10 years was 0.88±0.05 (38/43 patients with follow-up data). Outcome significantly correlated with stage and mitotic activity (<20 versus ≥20 mitoses/10 HPF: event-free survival 1.0 versus 0.48±0.05, P=0.0001). In conclusion, this analysis confirms that the majority of patients with OSCST present at low tumor stage and that prognosis in these patients is excellent. Refractory tumors are characterized by high proliferative activity. Therefore, histopathological evaluation substantially contributes to risk assessment in patients with OSCST and might be useful for therapy stratification in prospective therapeutic protocols.

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Acknowledgments

The authors thank all pathologists that contributed samples to the Kiel Pediatric Tumor Registry. The authors also thank the Deutsche Krebshilfe e.V. for the support of the German Germ Cell Tumor program. The authors thank Irene Kiekbusch, Frantzel Rahlf, and Susanne Koch for secretarial work and data management. The authors wish to thank Dr. Martin Vogel, Institute of Paidopathology and Placentology, Rudolph-Virchow University Hospital, Berlin, for the photograph of the macroscopic aspect of a juvenile granulosa cell tumor. Last, the authors gratefully appreciate Dr. Aleksander Talerman, Thomas Jefferson University, Philadelphia, for critical review of ovarian neoplasms and inspiring discussions during his visit to the Kiel Pediatric Tumor Registry.

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Authors and Affiliations

  1. Clinic of Pediatric Oncology, Hematology and Immunology, Children's Hospital, University Hospital Düsseldorf, Heinrich-Heine-University, Moorenstrasse 5, 40225 , Düsseldorf, Germany

    Dominik T. Schneider, Gabriele Calaminus & Ulrich Göbel

  2. Institute of Pediatric Pathology, Christian-Albrechts-University, Kiel, Germany

    Ute Jänig & Dieter Harms

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  1. Dominik T. Schneider
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  2. Ute Jänig
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  3. Gabriele Calaminus
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  4. Ulrich Göbel
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  5. Dieter Harms
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Correspondence to Dominik T. Schneider.

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Schneider, D.T., Jänig, U., Calaminus, G. et al. Ovarian sex cord–stromal tumors—a clinicopathological study of 72 cases from the Kiel Pediatric Tumor Registry. Virchows Arch 443, 549–560 (2003). https://doi.org/10.1007/s00428-003-0869-0

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