Hormone-active ovarian steroid cell tumor in a 2-year-old girl

Susann Weihrauch-Blüher; Toralf Bernig; Martin Kaiser; Dominik Schneider; Christian Kunze; Katja Ronstedt; Christine Fathke; Amica Müller-Nedebock; Pablo Villavicencio-Lorini; Jessica I. Hoell

doi:10.1515/jpem-2025-0271

Article

Hormone-active ovarian steroid cell tumor in a 2-year-old girl

Susann Weihrauch-Blüher , Toralf Bernig , Martin Kaiser , Dominik Schneider , Christian Kunze , Katja Ronstedt , Christine Fathke , Amica Müller-Nedebock , Pablo Villavicencio-Lorini and Jessica I. Hoell

Published/Copyright: August 21, 2025

Published by

Become an author with De Gruyter Brill

Submit Manuscript Author Information Explore this Subject

From the journal Journal of Pediatric Endocrinology and Metabolism Volume 38 Issue 10

Abstract

Objectives

Steroid cell tumors are very rare in children. During the past 25 years, only 3 cases have been reported in Germany. Symptoms may vary from virilization to signs of precocious puberty and increased growth velocity, making it diagnostically challenging. Due to rarity and the wide morphologic as well as differential diagnostic spectrum, initial clinical features may be misleading.

Case presentation

We report on a 2-year-old girl, who initially presented with symptoms of virilization and precocious puberty, i.e., pubertal hair growth equivalent to Tanner stage P3. Basal hormone profile yielded 10-fold increased testosterone and androstenedione as well as markedly increased estradiol levels in serum. Diagnostic imaging procedures (ultrasound, MRI of the abdomen) revealed a solid tumor in the left ovary, without any signs of peritoneal dissemination or metastases. After salpingo-oophrectomy of the left ovary (en bloc via laparoscopic surgery, without spillage), the diagnosis of an ovarian steroid cell tumor sized 40 × 25 × 22 mm producing both testosterone and estradiol was confirmed. The increased serum levels of androgens as well as estradiol decreased toward prepubertal values within 1 week after surgery.

Conclusions

Hormone-active steroid cell tumors of the ovary are extremely rare in infancy. In our patient, the tumor was classified as clinical stage Ia. We thus opted for clinical, biochemical, and sonographical controls without chemotherapy. We herein present follow-up data until 18 months after surgery and discuss them within the context of international literature.

Keywords: steroid cell tumor; ovary; infancy; virilization; precocious pubery

Corresponding author: Susann Weihrauch-Blüher, Clinic for Pediatrics I, University Medicine Hospital Halle (Saale), Ernst-Grube-Str. 40, 06120, Halle/Saale, Germany, E-mail: susann.weihrauch-blueher@uk-halle.de

Acknowledgments

We would like to thank Prof. Christian Vokuhl (Department of Pathology, University of Bonn, Germany) and Prof. Friedrich Kommoss (Institute for Pathology, Medical Campus Bodensee) for histological evaluation and permission to mention the diagnostic interpretations. We are also very grateful to Mrs. Paula Michel, University Hospital of Halle/S, Germany, for assistance in patient care and documentation.

Research ethics: Not applicable.
Informed consent: Not applicable.
Author contributions: All authors have accepted responsibility for the entire content of this manuscript and approved its submission.
Use of Large Language Models, AI and Machine Learning Tools: Not applicable.
Conflict of interest: The authors state no conflict of interest.
Research funding: Not applicable.
Data availability: Not applicable.

References

1. Skinner, MA, Schlatter, MG, Heifetz, SA. Ovarian neoplasms in children. Arch Surg 1993;128:849–53. https://doi.org/10.1001/archsurg.1993.01420200023004.Search in Google Scholar PubMed

2. Brookfield, KF, Cheung, MC, Koniaris, LG, Sola, JE, Fischer, AC. A population-based analysis of 1037 malignant ovarian tumors in the pediatric population. J Surg Res 2009;156:45–9. https://doi.org/10.1016/j.jss.2009.03.069.Search in Google Scholar PubMed

3. Umeda, S, Takase, K, Takayama, K, Yamamichi, T, Higuchi, K, Kawai, M, et al.. A report of a case with pediatric ovarian steroid cell tumor, not otherwise specified, found with precocious puberty. J Pediatr Hematol Oncol 2023;45:e522–4. https://doi.org/10.1097/mph.0000000000002496.Search in Google Scholar PubMed

4. Tan, EC, Khong, CC, Bhutia, K. A rare case of steroid cell tumor, not otherwise specified (NOS), of the ovary in a young woman. Case Rep Obstet Gynecol 2019:4375839. https://doi.org/10.1155/2019/4375839.Search in Google Scholar PubMed PubMed Central

5. Schnuckle, EM, Williamson, A, Carpentieri, D, Taylor, S. Ovarian sex cord stromal tumor, steroid cell, NOS in an adolescent: a case report. J Pediatr Adolesc Gynecol 2021;34:94–7. https://doi.org/10.1016/j.jpag.2020.08.001.Search in Google Scholar PubMed

6. WHO Classification of Tumours Editorial Board: Female genital tumours. IARC Library Cataloguing-in-Publication Data. 5th Edition. Lyon: International Agency for Research on Cancer 2020. Series: World Health Organization classification of tumours. ISBN 978928325049.Search in Google Scholar

7. Schneider, DT, Jänig, U, Calaminus, G, Göbel, U, Harms, D. Ovarian sex cord-stromal tumors-a clinicopathological study of 72 cases from the Kiel pediatric tumor registry. Virchows Arch 2003;443:549–60. https://doi.org/10.1007/s00428-003-0869-0.Search in Google Scholar PubMed

8. Xiong, Y, Ying, S, Yichao, J, Xing, C, Xiuli, W, Tonghui, J, et al.. Ovarian steroid cell tumor, not otherwise specified, treated with surgery: a case report and review of literature. Int J Clin Exp Pathol 2019;12:1434–8.Search in Google Scholar

9. Kosaka, N, Hasegawa, K, Kiuchi, K, Ochiai, S, Nagai, T, Machida, H, et al.. Cytological findings of ascitic fluid with a malignant ovarian steroid cell tumor: a case report and literature review. Acta Cytol 2017;61:165–71. https://doi.org/10.1159/000458750.Search in Google Scholar PubMed

10. Yoshimatsu, T, Nagai, K, Miyawaki, R, Moritani, K, Ohkubo, K, Kuwabara, J, et al.. Malignant ovarian steroid cell tumor, not otherwise specified, causes virilization in a 4-year-old girl: a case report and literature review. Case Rep Oncol 2020;13:358–64. https://doi.org/10.1159/000506044.Search in Google Scholar PubMed PubMed Central

11. Yılmaz-Ağladıoğlu, S, Savaş-Erdeve, S, Boduroğlu, E, Önder, A, Karaman, I, Çetinkaya, S, et al.. A girl with steroid cell ovarian tumor misdiagnosed as non-classical congenital adrenal hyperplasia. Turk J Pediatr 2013;55:443–6.Search in Google Scholar

12. Chan, LF, Storr, HL, Scheimberg, I, Perry, LA, Banerjee, K, Miraki-Moud, C, et al.. Pseudo-precocious puberty caused by a juvenile granulosa cell tumour secreting androstenedione, inhibin and insulin-like growth factor-I. J Pediatr Endocrinol Metab 2004;17:679–84. https://doi.org/10.1515/jpem.2004.17.4.679.Search in Google Scholar PubMed

13. Choong, CS, Fuller, PJ, Chu, S, Jeske, Y, Bowling, F, Brown, R, et al.. Sertoli-Leydig cell tumor of the ovary, a rare cause of precocious puberty in a 12-month-old infant. J Clin Endocrinol Metab 2002;87:49–56. https://doi.org/10.1210/jcem.87.1.8162.Search in Google Scholar PubMed

14. Fadare, O, Fard, EV, Bhargava, R, Desouki, MM, Hanley, KZ, Ip, PPC, et al.. The malignant potential of ovarian steroid cell tumors revisited: a multi-institutional clinicopathologic analysis of 115 cases. Am J Surg Pathol 2024;48:570–80. https://doi.org/10.1097/pas.0000000000002201.Search in Google Scholar

15. Hayes, MC, Scully, RE. Ovarian steroid cell tumors (not otherwise specified). a clinicopathological analysis of 63 cases. Am J Surg Pathol 1987;11:835–45. https://doi.org/10.1097/00000478-198711000-00002.Search in Google Scholar PubMed

Received: 2025-05-18

Accepted: 2025-08-10

Published Online: 2025-08-21

Published in Print: 2025-10-27

You are currently not able to access this content.

Articles in the same Issue

https://doi.org/10.1515/jpem-2025-0271

Keywords for this article

steroid cell tumor; ovary; infancy; virilization; precocious pubery