Susann Weihrauch-Blüher

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

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.