Endometrial stromal sarcoma (ESS) is a rare uterine malignancy that requires accurate pathological diagnosis for proper treatment. This study aimed to clarify the discrepancies in the pathological diagnosis of ESS and obtain practical clues to improve diagnostic accuracy. Between 2002 and 2015, 148 patients with low-grade ESS (LGESS), high-grade ESS (HGESS), undifferentiated endometrial sarcoma (UES), or undifferentiated uterine sarcoma (UUS) diagnosed at 31 institutions were included. We performed immunohistochemistry, real-time polymerase chain reaction for JAZF1-SUZ12 and YWHAE-NUTM2A/B, and break-apart fluorescent in situ hybridization for JAZF1, PHF1, and YWHAE. Central pathology review (CPR) was performed by six pathologists. After CPR, LGESS, HGESS, UES/UUS, and other diagnoses were confirmed in 72, 25, 16, and 31 cases, respectively. Diagnostic discrepancies were observed in 19.6% (18/92) of LGESS and 34% (18/53) of HGESS or UUS/UES. Adenosarcomas, endometrial carcinomas, carcinosarcomas, and leiomyosarcomas were common diagnostic pitfalls. JAZF1-SUZ12 transcript, PHF1 split signal, and YWHAE-NUTM2A/B transcript were mutually exclusively detected in 23 LGESS, 3 LGESS, and 1 LGESS plus 3 HGESS, respectively. JAZF1-SUZ12 and YWHAE-NUTM2A/B transcripts were detected only in cases with CPR diagnosis of LGESS or HGESS. The CPR diagnosis of LGESS, HGESS, and UUS was a significant prognosticator, and patients with LGESS depicted a favorable prognosis, while those with UUS showed the worst prognosis. Pathological diagnosis of ESS is often challenging and certain tumors should be carefully considered. The accurate pathological diagnosis with the aid of molecular testing is essential for prognostic prediction and treatment selection.

UNASSIGNED: Low-grade endometrial stromal sarcoma (LG-ESS) is a rare gynecological tumor. Whether adjuvant radiotherapy benefits survival in patients with resected early-stage ESS remains controversial. This study was designed to explore the role of adjuvant radiotherapy in stage I to II LG-ESS.
UNASSIGNED: We retrospectively reviewed patients with stage I to II LG-ESS in our center from Jan. 1998 to Feb. 2018. All patients underwent a total hysterectomy and postoperative radiotherapy was administrated based on clinical and pathological characteristics.
UNASSIGNED: A total of 152 patients with stage I to II resected LG-ESS were included. Forty patients received adjuvant radiotherapy (RT group) while 112 patients did not receive adjuvant radiotherapy (no RT group). The baseline characteristics of the two groups were comparable, except that the proportion of stage II patients in the RT group was higher than that in the no RT group (32.5% vs. 11.6%, in RT vs. no RT groups, respectively; p = 0.003). For both patient groups, median overall survival was not reached. The median disease-free survival (DFS) was 144 months. Radiotherapy was associated with significantly improved DFS (92 months vs. not reached in RT vs. no RT groups, respectively; p = 0.008) and pelvic failure-free survival (PFFS) (92 months vs. not reached in RT vs. no RT groups, respectively; p=0.004). Subgroup analysis revealed that RT benefited survival most among patients with stage IB to IIB disease. Adjuvant radiotherapy significantly reduced the pelvic recurrence rate (10.0%, 4/40 vs. 28.6%, 32/112, p = 0.018). No radiotherapy-induced grade 4 to 5 toxicity was observed.
UNASSIGNED: For patients with stage I to II LG-ESS, adjuvant radiotherapy showed significant improvement in DFS and PFFS with tolerable adverse effects, especially in patients with stage IB to IIB disease.

OBJECTIVE: To evaluate the risk of encountering unexpected uterine smooth muscle tumors of uncertain malignant potential (STUMPs) or sarcomas during surgical treatment of mesenchymal tumors of the uterus using morcellation.
METHODS: Data were collected retrospectively from subjects who were pathologically diagnosed with uterine leiomyoma or its variants, STUMP or other premalignant mesenchymal tumors of uterus, or sarcoma during surgical treatment between July 2014 and June 2017.
RESULTS: A total of 3785 women were investigated; 2824 laparoscopic procedures (74.6%) were performed, and an electronic power morcellator was used in 1636 patients (43.2%). Sixteen women (0.42%) were diagnosed with STUMP and 14 (0.37%) were diagnosed with uterine sarcoma. The incidence rate of unexpected STUMP or uterine sarcoma was 0.61% (23 of 3785 women); unexpected STUMP in 13 (0.34%), and unexpected sarcoma was in 10 (0.26%). Moreover, the unexpected leiomyosarcoma rate was 0.08% (3 in 3785). The rate of unintended morcellation of STUMPs was relatively high at 0.26% (10 in 3785), however, that for uterine sarcomas was 0.05% (2 in 3785).
CONCLUSIONS: The risks of unintended morcellation were very low for sarcomas and STUMPs, although the risk of the latter was approximately 5-fold that of the former. To reduce the unintended dissemination of tumors, patients suspected of having malignancies should be provided adequate information regarding their treatment options as well as their associated risks. Meanwhile, improved preoperative screening methods for STUMP and sarcoma should be established.

UNASSIGNED: To analyse the clinical presentation, histological type, management, and prognosis of cases diagnosed with uterine sarcomas.
UNASSIGNED: This was a hospital-based retrospective study conducted at the Institute of Obstetrics and Gynaecology, Chennai, and Meenakshi Medical College and RI, Kancheepuram, from 2009 to 2015. Thirty patients who were diagnosed with uterine sarcoma were analysed for type of sarcoma, clinical details, treatment, and prognosis.
UNASSIGNED: Leiomyosarcoma was the commonest type seen in ten cases followed by mixed mullerian tumours in nine cases and endometrial stromal sarcomas in six cases. Leiomyosarcomas and mixed mullerian tumours were seen between the ages of 45 and 65 years. Women with endometrial stromal tumours were younger in the age group between 35 and 45 years. Pelvic mass and pain were the predominant symptom in leiomyosarcoma and mixed mullerian tumours. Endodermal stromal tumours presented predominantly with abnormal uterine bleeding. Forty percentage of cases were diagnosed with stage I disease. Twenty-six patients were managed surgically. Post-operative adjuvant therapy was given in 12 patients. Median duration of follow-up was 52 months. During the above period, 11 were alive, eight were lost to follow-up, and 11 were dead. In those who were alive, two cases presented with local recurrences.
UNASSIGNED: The pre-operative diagnosis of sarcoma is difficult and prognosis is poor even in early stage disease because of the aggressive nature of the tumours.

To provide refined prognostic information from large cohorts of women with low-grade or high-grade endometrial stromal sarcoma (ESS).
We performed an observational retrospective cohort analysis of women diagnosed with low-grade or high-grade ESS from the 1998-2013 National Cancer Database. Kaplan-Meier and multivariable accelerated failure time survival analyses were performed to identify prognostic factors after multiple imputation of missing data. Recursive partitioning methods were used to rank prognostic factors in high-grade ESS. Matched cohort analyses were performed to hypothesis-test effects of adjuvant treatments.
We identified 2414 and 1383 women with low-grade or high-grade ESS, respectively. Women with high-grade ESS had markedly decreased survival compared to women with low-grade ESS (five-year survival (95% CI): 32.6 (30.1-35.3%) versus 90.5% (89.3-91.8%), P<0.001). Among women with high-grade ESS, median survival (95% CI) was only 19.9 (17.1-22.1) months. Increased age and tumor size were associated with decreased survival in low-grade ESS. In high-grade ESS, additional negative prognostic factors were distant or nodal metastasis, omission of lymphadenectomy, and pathologically-positive surgical margins (all P<0.001). Use of adjuvant chemotherapy (time ratio (TR) (95% CI): 1.36 (1.17-1.58), P<0.001) and radiotherapy (TR (95% CI): 1.57 (1.32-1.87), P<0.001) were associated with increased survival for high-grade ESS.
The contrasting excellent versus poor prognosis of low-grade versus high-grade ESS, respectively, was confirmed. The best treatment of high-grade ESS is early and complete surgical resection including lymphadenectomy. Adjuvant chemotherapy and radiotherapy may increase survival of women with high-grade ESS.

OBJECTIVE: To discuss the optimal treatment options for low grade endometrial stromal sarcoma (LG-ESS).
METHODS: Medical records of consecutive patients with LG-ESS in our institute were collected. The pertinent data, including clinicopathological characteristics, treatment and prognostic information were evaluated.
RESULTS: A total of 153 cases of LG-ESS were included. The 5-year relapse free survival (RFS), overall survival (OS) and survival after relapse (SAR) rates were 66.1%, 95.8% and 82.9%, respectively. Ovary-sparing procedures, positive resection-margins, and myomectomy were the independent adverse factors for relapse (P<0.0001, =0.0041, and =0.0075, respectively). Post-menopause, cervical involvement, and positive lymphovascular space involvement were significantly associated with survival (P<0.0001, =0.0020, and =0.0163, respectively). Distance recurrence and macroscopically residual tumors negatively affected SAR (P=0.0137 and =0.0004, respectively). No benefit was found for lymphadenectomy in terms of both RFS and OS (P=0.1187 and =0.5138, respectively). Initial ovary-sparing procedures and myomectomy had no impact on OS (P=0.0810 and =0.8845, respectively). Adjuvant treatment had a slightly beneficial effect both on OS and SAR.
CONCLUSIONS: Hysterectomy with bilateral salpingo-oophorectomy and complete resection of the macroscopic lesion should be treated as the initial and salvage mainstay treatments for LG-ESS patients. Ovary-sparing procedures could be considered for young women without cervical involvement; however, long-term follow-up should be mandatory. Myomectomy should only be conserved for young patients with a strong desire for future fertility, with fully informed consent; hysterectomy was recommended after the completion of pregnancy and delivery. However, the roles of lymphadenectomy and adjuvant treatment deserve further investigation.

OBJECTIVE: To assess the difficulties associated with diagnosing endometrial stromal tumors (ESTs) on endometrial biopsy.
METHODS: We examined 25 endometrial biopsy specimens from 19 consecutive women diagnosed with either endometrial stromal nodule (n = 3) or endometrial stromal sarcoma (n = 16).
RESULTS: Rereview of the biopsy specimens revealed a stromal fragment suspicious for an EST in 16, of which eight had received a benign diagnosis on initial review. Most ESTs had an aglandular stromal fragment that was 5 mm or larger. Stromal fragments of this size were not encountered in the control material. Problematic areas included highly cellular leiomyoma and a lack of attention to the stromal compartment.
CONCLUSIONS: Most endometrial stromal tumors present with large aglandular stromal fragments (≥5 mm). These fragments are large enough that difficulties in diagnosis appear to be due to a lack of attention to the stromal compartment.