Recurrence of Endometrial Stromal Sarcoma, Two Decades Post-Treatment

Abstract

Endometrial stromal cell sarcomas (ESS) are a unique subtype of uterine malignancy. Recurrent low grade endometrial stromal sarcomas (LESS) is identified in half of the patients. Here, we discuss a case of a 76-year-old Asian female with a past medical history of adenomyosis and hypertension who presented to the outpatient clinic with a chief complaint of painless hematuria for one day. Computed tomography scan of abdomen and pelvis with contrast showed a new right-sided mixed cystic and solid pelvic mass measuring up to 6 cm, obstructing and invading the distal right ureter, which was concerning for malignancy. Positron emission tomography (PET scan) demonstrated a right pelvic mass with increased radiotracer activity consistent with malignancy. She underwent laparotomy with excision of the right-sided pelvic mass with an abdominal washout and at the same time, also underwent cystoscopy with right ureteral stent placement. Tissue pathology was consistent with spindle cell neoplasm with staining and histologic features consistent with a recurrent stromal cell sarcoma. Uterine sarcomas tend to have an aggressive nature but there are key features about ESS that distinguish it from other uterine sarcomas. ESS has a more indolent clinical course and can reoccur years after initial diagnosis. They usually relapse locally, although relapses in extrauterine sites have also been reported. Treatment of ESS depends on the grade and stage at the time of diagnosis. The main line of treatment for ESS consists of a total abdominal hysterectomy (TAH) and salpingo-oophorectomy (BSO). The significance of this case demonstrates that, although remission can be obtained after the initial diagnosis, recurrence can happen. Even when patients seem to be disease-free, clinicians should follow them closely; early diagnosis is important as treatment for this type of entity has a high survival rate.

Complete remission of advanced low-grade endometrial stromal sarcoma after aromatase inhibitor therapy: a case report

“…Hormonal therapy has also been used to shrink the tumor before surgical resection, as reported in the case of a 47-year-old woman with low-grade ESS [ 31 ]. Despite complete remission of ESS, such tumors carry a high risk of recurrence even after two decades of treatment, as reported by Gangireddy et al ., which highlights the importance of regular follow-ups [ 32 ].…”

Epigenetic Features in Uterine Leiomyosarcoma and Endometrial Stromal Sarcomas: An Overview of the Literature

“…Their five-year survival rates are higher than 80%. For disease stages I and II the five-year survival is approximately 90% whereas for stages III and IV (i.e., advanced disease) the survival rate for the same interval of time is significantly reduced, according to the FIGO stage system [ 75 , 82 ].…”

“…Total hysterectomy with bilateral salpingo-oophorectomy remains the standard treatment for ESS, and lymphadenectomy does not appear to improve overall survival rates [ 83 ]. Adjuvant radiotherapy and hormone therapy are not well-established therapeutic options yet, even though some studies have shown that hormonal agents can be an alternative to the management of LG-ESS [ 82 , 84 ]. In contrast, HG-ESS is generally detected in advanced stages with no effective adjuvant therapy available.…”