Introduction

Ovarian sex cord-stromal tumors are a rare group of ovarian neoplasms that arise from the specialized cells responsible for supporting ovarian development and hormone production.

Among these tumors, Sertoli-Leydig cell tumors account for less than 0.5% of primary ovarian neoplasms and most commonly occur in adolescents and young adults, although cases in older women have been described [1-5]. Histologically, these tumors are classified as well differentiated, moderately differentiated, poorly differentiated, or retiform, with poorly differentiated forms being associated with greater diagnostic complexity and more aggressive clinical behavior [3,5,6].

Clinical presentation is variable and may include abdominal distension, pelvic pain, a palpable mass, menstrual irregularities, or endocrine manifestations related to androgen or estrogen production [1,6-8]. However, hormonal symptoms may be absent or subtle, particularly in postmenopausal women and in patients with advanced disease [6-8]. Poorly differentiated tumors may also show overlapping morphological and immunohistochemical features with epithelial, mesenchymal, or undifferentiated neoplasms, allowing them to mimic more common ovarian or uterine malignancies and potentially delaying accurate diagnosis, making diagnosis particularly challenging [3,5].

Because advanced Sertoli-Leydig cell tumors are exceptionally uncommon, evidence to guide treatment is limited. Surgery remains the cornerstone of management, whereas adjuvant chemotherapy is generally considered for advanced-stage, recurrent, or poorly differentiated disease [9,10]. Treatment decisions become particularly difficult in elderly or comorbid patients, in whom the potential benefits of systemic therapy must be balanced against toxicity, functional decline, and quality of life [9-12].

We report a rare case of an advanced and poorly differentiated ovarian Sertoli-Leydig cell tumor in an elderly woman, highlighting the diagnostic pitfalls, the value of hormonal evaluation and expert pathology review, and the therapeutic limitations encountered in aggressive disease.