The symptom of EESS usually involve abnormal uterine bleeding, however, an accurate diagnosis is more challenging [10]. For patients in our study, three showed abdominal distention, two with vaginal bleeding and two with abdominal pain for initial clinical appearance. To assist diagnosis, preoperative curettage and pathology, including immunohistochemistry test, are import methods [11]. LG-EESS characteristically showed positive for CD10, ER and PR [7, 12].
In our study, small endometriosis lesions in both vaginal and vulva ESS were found which contradict with previous research [8, 9, 13]. Although there might be issues for specimen collection, a possibility that vaginal or vulva EESS involved malignant transformation of endometrial stromal cells still existing.
The treatment option for LG-EESS involved a total abdominal hysterectomy, however, ovary removal and comprehensive surgical staging, including pelvic and para-aortic lymphadenectomy remained debatable [14]. In our study, patient received radical surgery, which was uterine and double appendage resection plus tumor resection, achieved optimal tumor reduction with a lower tumor recurrence rate.
Four patients underwent pelvic lymphadenectomy, a postoperative pathology showed negative in lymph nodes. This also supported the findings that ESS usually did not develop lymphatic metastasis [10]. We also did not directly observe clinical benefit of lymphadenectomy which an analysis of EESS patient showed addition of lymphadenectomy to hysterectomy did not improve either cause-specific survival or overall survival compared to hysterectomy alone [15, 16].
For EESS patients of reproductive age, double appendage removal is also recommended [17]. However, a recent study suggested that ovary preservation did not significantly affect the overall survival of patients [15, 18]. In our study, one young patient with LG-EESS underwent fertility preservation surgery (preservation of the normal uterus and appendages), and adjuvant treatment including chemotherapy and hormone therapy. The patient did not relapse in the follow-up with a NED (no evidence of disease) reached 86 months. Previous studies showed two patients with vaginal EESS after local tumor resection with negative margins did not relapse in 36-months and 38-months follow-ups [8, 13]. These studies indicate that fertility preservation surgery can be performed with cautions in EESS patients follow a desire to retain reproductive ability, however, this conclusion need to be confirmed with large-sample-size studies.
Postoperative adjuvant treatment of EESS includes radiotherapy, chemotherapy and hormone therapy [19–22]. Our study showed postoperative radiotherapy and chemotherapy did not reduce the tumor recurrence rate but hormone therapy, especially for patients who underwent optimal tumor reduction surgery, had significantly reduced recurrence rate compared to that of patients who did not undergo hormone therapy. As a hormone receptor positive tumor, study showed that LG-EESS was sensitive for endocrine treatment [14].
Patients with LG-EESS had a good prognosis and long-term survival but a risk of late stage recurrence [23]. In our study, the recurrence rate was 30% and the longest recurrence duration was 60 months.