A case report on endometrial stromal sarcoma: a diagnostic challenge

Abstract

Endometrial stromal sarcoma (ESS) is a rare and malignant tumor of the endometrial stroma, predominantly affecting women in their 40s and 50s. Here, we report a unique case of a 60-year-old post-menopausal woman, para 3 live 3 (P3L3), presenting with post-menopausal bleeding, initially thought to be due to a primary vaginal sarcoma. Despite an extensive diagnostic workup, including pelvic exams, Pap smears, and vaginal ultrasound, no abnormalities were detected. However, repeated episodes of post-menopausal bleeding prompted further investigation, including magnetic resonance imaging (MRI) and fractional curettage, which revealed a polypoidal growth in the right fornix extending through the OS. The clinical impression favored a primary vaginal sarcoma metastasizing to the uterus. The patient underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy. Surprisingly, histopathological analysis determined the tumor originated from the endometrial stromal tissue with metastasis to the vagina. The tumor was classified as low-grade with significant myometrial invasion (greater than 50%). Post-operatively, lymph node resection was performed, followed by adjuvant radiotherapy to mitigate recurrence risk. This case highlights the diagnostic complexities of ESS, particularly when it mimics primary vaginal tumors, and underscores the importance of histopathological evaluation in determining the correct tumor origin. ESS is rare, constituting about 0.2% of all uterine malignancies, and this case emphasizes the importance of multidisciplinary management and vigilant follow-up to optimize patient outcomes.