Recurrent endometrial stromal sarcoma in the abdominal wall following a total hysterectomy: A rare case report

INTRODUCTION: High-grade endometrial stromal sarcoma (HG-ESS) is a rare pathological type of uterine sarcoma. Over 80 % of affected patients would experience recurrences within a few years of initial presentation. Such case is rare and therefore, we need to report the case including the management. Information on performing good surgical techniques is important. CASE PRESENTATION: A 55-year-old female patient was referred after having a total hysterectomy and bilateral salphingoophorectomy at a private hospital. Results of pathologic examination showed that the patient had HG-ESS. The patient subsequently received adjuvant chemotherapy. The regimens used were Carboplatin (AUC-6) and Paclitaxel (175 mg/m(2)). Within five months following the chemotherapy, she complained rapid abdominal enlargement, which was a clinically mobile palpable solid mass at the level of the navel. Imaging findings suggested recurrent endometrial stromal sarcoma in the abdominal wall. Wide excision and frozen section, which were continued with mesh insertion and abdominal wall reconstruction, were then performed. DISCUSSION: Recurrence develops in one-half to two-third of patients with HG-ESS. It has been reported that the sites of recurrence usually include multiple lung metastases, peritoneal metastases, and/or local recurrences. There is currently no standard therapy for patients with recurrence of the disease as HG-ESS is a rare clinical entity. Treatment has been defined based on experiences gained from retrospective case reports. CONCLUSION: Successful management of patients with recurrent abdominal wall HG-ESS requires interdisciplinary and interprofessional teamwork. Multi-centre prospective trials are required to develop a guideline of optimal treatment for the disease entity, particularly the recurrence.