Multidisciplinary Management of a Giant Ovarian Sertoli-Leydig Cell Tumor Presenting With Isosexual Precocious Puberty: A Pediatric Case Report.
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Sertoli-Leydig cell tumors (SLCTs) are rare ovarian sex cord-stromal tumors, particularly uncommon in the pediatric population. They are typically androgen-secreting, while estrogen-secreting forms are rare and may lead to diagnostic confusion with granulosa cell tumors. We report the case of a 7-year-old girl presenting with progressive abdominal distension and isosexual precocious puberty. Imaging revealed a large abdominopelvic mass of uncertain origin. Histopathological examination of a biopsy suggested a sex cord-stromal tumor. The patient received neoadjuvant chemotherapy with the VIP regimen, resulting in significant tumor reduction, followed by fertility-sparing surgery. Final histopathological and immunohistochemical analysis confirmed a SLCT. The tumor was classified as FIGO stage IC2 due to preoperative capsular breach. Postoperatively, an additional cycle of chemotherapy was administered. Hormonal markers, including estradiol and inhibin B, normalized after treatment. During follow-up, a transient rise in these markers was observed in the absence of radiological abnormalities, followed by spontaneous normalization. The patient remains in complete remission after 27 months of follow-up. This case highlights an atypical estrogen-secreting pediatric SLCT and underscores the importance of histopathological confirmation in unusual clinical presentations. It also illustrates the potential role of neoadjuvant chemotherapy in large tumors and supports the use of inhibin B as a biomarker for disease monitoring, although its interpretation should be cautious and integrated with clinical and imaging findings. Long-term follow-up is essential due to the risk of recurrence.