Early-stage sertoliform endometrioid carcinoma of the ovary: diagnostic, molecular, and therapeutic considerations

The sertoliform variant of ovarian endometrioid carcinoma is exceedingly rare and often mimics sex-cord stromal or Brenner tumors, creating diagnostic challenges. <b>Case</b> <b>:</b> A 72-year-old woman presented with progressive abdominal distention and pain. MRI revealed a 22.7 × 13.8 × 16 cm mixed cystic-solid pelvic mass with mild ascites and elevated tumor markers. She underwent total hysterectomy, bilateral salpingo-oophorectomy, omentectomy, and lymphadenectomy. Pathology demonstrated low-grade endometrioid carcinoma, sertoliform variant, confined to the left ovary. Immunostains were positive for ER, AE1/AE3, β-catenin (nuclear/cytoplasmic), CDX2, and EMA. Molecular profiling revealed CTNNB1 (p.S33A) and FBXW7 (p.Q624*) pathogenic variants. She received adjuvant letrozole and remains disease-free at 3 years post-surgery. <b>Conclusion</b> <b>:</b> This case highlights the diagnostic complexity of ovarian tumors with sertoliform morphology and the importance of integrating morphologic, immunohistochemical, and molecular data. When confined to the ovary, prognosis is excellent following surgical resection. Endocrine therapy may be considered on an individualized basis in hormone-responsive tumors, although its role in early-stage disease remains unproven.