Low-grade endometrial stromal sarcoma (LGESS) is a rare disease that accounts for 0.2%of all malignant tumors of the uterine corpus. Cytotoxic anticancer drugs have little effect on advanced LGESS, and hormone therapy, such as aromatase inhibitors, is recommended.
A 45-year-old woman (2 gravida, 2 para) visited a local gynecologist because of left lower abdominal pain and a mass in the pouch of Douglas was found. Exploratory laparoscopy revealed disseminated lesions in the pouch of Douglas and the abdominal cavity. The pathological diagnosis of the lesions was LGESS and she was referred to our hospital. Lower gastrointestinal endoscopy revealed rectal mucosal invasion and she was diagnosed with LGESS, stage IVB. As complete resection of the disseminated lesions was not possible, we performed tumor reduction surgery. Total abdominal hysterectomy, bilateral salpingo-oophorectomy, ileal resection, and Hartmann' s surgery were performed, and the pathological diagnosis was LGESS (pT4NXM1). Aromatase inhibitors were administered postoperatively for the residual lesion, and no progression or recurrence was observed 1 year and 2 months after the operation. Aromatase inhibitors for LGESS are not covered by insurance in Japan but should be considered as one of the treatment options.
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