Abstract
Background
This study aimed to analyze the clinical and pathological features of extrauterine endometrial stromal sarcoma (EESS) and explore an effective therapeutic regimen to reduce the recurrence rate in low-grade EESS patients.
Methods
Ten LG-EESS patients who were treated at the Chinese Academy of Medical Sciences Cancer Institute and Hospital from June 1999 to June 2019 were collected and analyzed.
Results
(1) Patient demographics is summarized in Table 1. Preoperative CA125 examination showed that 8 patients had a median level of 49.5 U/L (15.4–168.0 U/L). (2)All ten patients underwent tumor cytoreductive surgery. Five patients underwent optimal tumor resection, achieved a R0 resection. After the initial surgery ,two patients were treated with adjuvant chemotherapy only, two patients were treated with chemotherapy and radiation therapy, and three patients were treated with chemotherapy plus hormonal treatment.(2) Most EESS patients had multiple tumors. the omentum was the most commonly affected site, followed by ovaries.(3) The median follow-up was 94 (range: 27–228) months, and recurrence was observed in 3 patients (n = 10, 30%). The 5-year and 10-year DFS rates were both 70%, as shown in Fig. 1. OS was both 100% at 5 and 10 years.
Conclusion
As a conclusion, EESS is a rare disease and LG-EESS has a good prognosis. Surgery remains the available treatment for patients. LG-EESS has a risk of late recurrence which requires a long-term follow-up. With a limited sample size, our study shows optimal tumor reductive surgery and adjuvant hormone therapy can significantly reduce the risk of recurrence.
Factors affecting the development of recurrence in stage I low grade - endometrial stromal sarcoma; multicentre retrospective study
