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We report on a 50-year-old postmenopausal woman who presented with abnormal uterine bleeding and pelvic pain due to a uterine solid mass grew from the uterine fundus to the cervix and with so far undescribed obviously gelatinous grossly change, which was suspected of myxoid leiomyosarcoma in intraoperative diagnosis. Morphologically, the tumor cells displayed haphazard fascicles of uniform mild-to-moderate heteromorphic spindle cell component with significant and abundant myxoid stroma, forming signet ring cells and microcysts. Immunohistochemically, the tumour cells were diffusely positivefor CD10 and cyclin D1 and negative for Desmin and SMA, but the expression of BCOR staining was not present. The FISH study showed a positive BCOR gene break probe, and the RNA sequencing revealed an identified reciprocal fusion gene ZC3H7B-BCOR. The case was finally diagnosed as ZC3H7B-BCOR high-grade endometrial stromal sarcoma. Tumor recurrence occurred rapidly on the pelvic peritoneal and vaginal 2 months after resection. In conclusion, these findings further support ZC3H7B-BCOR HGESS has a poor prognosis and molecular testing of uterine mesenchymal tumors with myxoid matrix and unusual grossly presentation is recommended to avoid misdiagnosis.

UNASSIGNED: Endometrial stromal sarcoma is a relatively rare malignant tumor that derives from the malignant transformation of primitive uterine mesenchymal cells. It can lead to distant metastases. High-grade endometrial stromal sarcoma is extremely rare. The adrenal glands are an unreported site of metastasis.
UNASSIGNED: A 71-year-old woman with a diagnosis of endometrial stromal sarcoma 30 months ago. After receiving treatment with radiotherapy and chemotherapy, the patient was kept asymptomatic during the follow-up until 2 years later, when she complained of dyspnea. Pulmonary and right adrenal gland metastases were detected by 18F-FDG PET/CT. The right upper lobe mass was diagnosed as a high-grade endometrial stromal sarcoma metastasis after postoperative pathology. Due to the patient\'s high risk of surgery, as she had many underlying diseases, we performed adequate preoperative preparation. The physical examination revealed that a hard mass was palpable in the right renal area. The right adrenal mass was resected in our hospital. Immunohistochemistry showed ER (-), PR (-), CD10 (+), P16 (+), Ki-67 (50%). The final diagnosis on pathological examination was a high-grade ESS metastatic to the right adrenal gland. The patient continued treatment in other hospitals after surgical resection. After four months of postoperative follow-up, metastasis was detected again during a PET/CT examination at an outside hospital.
UNASSIGNED: Endometrial stromal sarcoma is rare, and the adrenal glands are an unreported site of metastasis. It has no specific clinical symptoms and mainly found for other reasons. The diagnosis still depends on pathology and immunohistochemistry. If there is no relevant past history, it is difficult to exclude a primary adrenal tumor.

OBJECTIVE: We report a low-grade endometrial stromal sarcoma (ESS) with a novel CDKN1A-JAZF1 fusion gene arising from abdominal wall endometrioma.
METHODS: A 40-year-old woman presented with a 5.5-cm abdominal wall mass juxtaposed to the postoperative scar of two cesarean sections. Histologically, the tumor exhibited obvious tongue-like protrusions into the surrounding tissue, showed spindle cells with multinodular growth pattern that occasionally rotate around small arteries. Immunohistochemically, the tumor cells were positive for CD10, estrogen receptor (ER), progesterone receptor (PR), negatively stained for smooth muscle actin (SMA), CD117, CyclinD1. In addition, a previously undescribed gene fusion between CDNK1A 5\' end of exon 1(NM_000389.5) and JAZF1 3\' end of exon 5 (NM_175,061,3) was reported in this case.
CONCLUSIONS: This report of ESS suggesting that rapidly growing abdominal wall masses without menstruation-related should be promptly evaluated and treated aggressively. In addition, we have expanded the molecular landscape of low-grade ESS.

The clinical value of lymph node dissection remains controversial. This study aimed to evaluate the impact of lymphadenectomy on the prognosis of patients with uterine leiomyosarcomas (uLMS) or endometrial stromal sarcomas (ESS). PubMed, EMBASE and the Cochrane Library were searched for studies describing the prognostic significance of lymphadenectomy in uLMS or ESS. Quality assessments were performed using the Newcastle-Ottawa Scale, relative hazard ratios and a random-effects model. Thirty-two retrospective cohort studies that included 26,693 patients in total were enrolled. Patients with uLMS or low-grade ESS (LG-ESS) had no survival benefits from lymphadenectomy. However, patients with high-grade ESS (HG-ESS), did show survival benefits of lymphadenectomy, with no heterogeneity. No significant evidence of publication bias was found. Lymphadenectomy had little prognostic effect on patients with early-stage uLMS or LG-ESS. The best treatment for HG-ESS is early, comprehensive hysterectomy with lymph node dissection.

BACKGROUND: The coexistence of a uterine leiomyosarcoma and a high-grade endometrial stromal sarcoma (HGESS) is extremely rare, especially when one of the components causes metastasis.
METHODS: A 46-year-old female with aggravated abdominal pain for more than 4 months was diagnosed with uterine malignant mesenchymal tumor composed of predominantly a leiomyosarcoma (99%) and a minor component of HGESS with BCL6 corepressor (BCOR) gene alterations (1%), with ovarian and pelvic metastases.
RESULTS: The volume of HGESS with BCOR gene alterations accounted for less than 1% of the tumor mass but caused ovarian and pelvic metastases.
CONCLUSIONS: HGESS with BCOR gene alterations is extremely aggressive. We suggest that when both components of HGESS with BCOR gene alterations and uterine leiomyosarcoma are present in one patient, the HGESS with BCOR gene alterations needs to be highlighted in the pathological report, even if it accounts for less than 1% of the tumor volume.

OBJECTIVE: The Ki-67 index is used to evaluate cell proliferation activity, which is related to tumor progression, metastasis, and prognosis. We aimed to explore the prognostic value of Ki-67 index in endometrial stromal sarcoma and to explore the optimal cut-off value of Ki-67 index for predicting recurrent endometrial stromal sarcoma.
METHODS: A total of 82 patients with endometrial stromal sarcoma who were treated in our hospital were collected. Clinicopathological data of these patients were retrospectively analyzed. Ki-67 index was detected by the immunohistochemical method. Receiver operating characteristic curve and the Youden index were performed to determine the optimal cut-off value of Ki-67 index for predicting recurrent endometrial stromal sarcoma. The Cox regression was performed to analyze risk factors affecting prognosis of endometrial stromal sarcoma. The Kaplan-Meier method and Log-rank test were performed to analyze the survival of patients.
RESULTS: The optimal cut-off value of Ki-67 index for predicting recurrent endometrial stromal sarcoma was 35%. The results of univariate analysis showed that high Ki-67 index (≥35%) was statistically significantly bound up with shorter progress free survival and overall survival. The results of multivariate analysis showed that Ki-67 index (P = 0.001) and ovarian preservation (P = 0.040) were independent prognostic factors of progress free survival.
CONCLUSIONS: A Ki-67 index cut-off of 35% was optimal for predicting recurrent endometrial stromal sarcoma. Ki-67 index may be a useful prognostic marker in endometrial stromal sarcoma.

Objective: The present study aimed to explore the application value of magnetic resonance imaging (MRI) histograms with multiple sequences in the preoperative differential diagnosis of endometrial stromal sarcoma (ESS) and degenerative hysteromyoma (DH). Methods: The clinical and preoperative MRI data of 20 patients with pathologically confirmed ESS and 24 patients with pathologically confirmed DH were retrospectively analyzed, forming the two study groups. Mazda software was used to select the MRI layer with the largest tumor diameter in T2WI, the apparent diffusion coefficient (ADC), and enhanced T1WI (T1CE) images. The region of interest (ROI) was outlined for gray-scale histogram analysis. Nine parameters-the mean, variance, kurtosis, skewness, 1st percentile, 10th percentile, 50th percentile, 90th percentile, and 99th percentile-were obtained for intergroup analysis, and the receiver operating curves (ROCs) were plotted to analyze the differential diagnostic efficacy for each parameter. Results: In the T2WI histogram, the differences between the two groups in seven of the parameters (mean, skewness, 1st percentile, 10th percentile, 50th percentile, 90th percentile, and 99th percentile) were statistically significant (P < 0.05). In the ADC histogram, the differences between the two groups in three of the parameters (skewness, 10th percentile, and 50th percentile) were statistically significant (P < 0.05). In the T1CE histogram, no significant differences were found between the two groups in any of the parameters (all P > 0.05). Of the nine parameters, the 50th percentile was found to have the best diagnostic efficacy. In the T2WI histogram, ROC curve analysis of the 50th percentile yielded the best area under the ROC curve (AUC; 0.742), sensitivity of 70%, and specificity of 83.3%. In the ADC histogram, ROC curve analysis of the 50th percentile yielded the best area under the ROC curve (AUC; 0.783), sensitivity of 81%, and specificity of 76.9%. Conclusion: The parameters of the mean, 10th percentile and 50th percentile in the T2WI histogram have good diagnostic efficacy, providing new methods and ideas for clinical diagnosis.

To investigate the MRI features and clinical outcomes of unexpected uterine sarcomas in patients after high-intensity focused ultrasound (HIFU) ablation for presumed uterine fibroids.
15,759 consecutive patients who came for HIFU treatment, from November 2008 to September 2019, for presumed uterine fibroids were retrospectively reviewed. All the patients had completed a pre-HIFU MRI. All MRI images were independently analyzed and interpreted by two radiologists in every center.
According to the T2WI MRI features of hyperintensity, accompanied by irregular margins, necrosis or cystic degeneration, multi-lobulated lesion with internal septation, 46 patients were suspected to be uterine sarcomas before HIFU. Eleven patients were histologically diagnosed as uterine sarcomas after laparotomy. Among the 15713 patients who received HIFU treatment for presumed uterine fibroids, 8 patients were found to have occult recurrence during the follow-up period, and 6 were confirmed histologically as uterine sarcomas after laparotomy. The incidence rate of uterine sarcomas was 0.108% (17/15759). Among them, 12 cases were low-grade endometrial stromal sarcoma (LG-ESS) and 5 cases were uterine leiomyosarcoma (LMS). No histological dissemination of the sarcoma was detected in patients with unexpected uterine sarcomas.
Although some MRI features of uterine sarcomas and uterine fibroids overlapped, MRI is valuable in distinguishing between uterine fibroids and uterine sarcomas. HIFU does not seem to cause histological dissemination of the sarcoma, but follow-up visits should be strictly adhered to in order to detect unexpected uterine sarcomas at an early stage and to treat them in a timely manner.