• Artificial intelligence
• Molecular diagnostics
• Treatment approaches
• Uterine sarcomas

• Humans
• Female
• Uterine Neoplasms/therapy
• Uterine Neoplasms/diagnosis
• Uterine Neoplasms/pathology
• Sarcoma/therapy
• Sarcoma/diagnosis
• Sarcoma/pathology
• Lymph Node Excision
• Magnetic Resonance Imaging
• Leiomyosarcoma/therapy
• Leiomyosarcoma/diagnosis

• FGF12
• low-grade endometrial stromal sarcoma
• prognostic biomarker
• scRNA-seq
• tumor microenvironments

• Humans
• Female
• Biomarkers, Tumor/genetics
• Sarcoma, Endometrial Stromal/genetics
• Sarcoma, Endometrial Stromal/pathology
• Sarcoma, Endometrial Stromal/metabolism
• Sarcoma, Endometrial Stromal/diagnosis
• Single-Cell Analysis
• Prognosis
• Endometrial Neoplasms/genetics
• Endometrial Neoplasms/mortality
• Endometrial Neoplasms/pathology
• Endometrial Neoplasms/diagnosis
• RNA-Seq
• Tumor Microenvironment/genetics
• Fibroblast Growth Factors/genetics
• Fibroblast Growth Factors/metabolism
• Neoplasm Grading
• Transcriptome
• Gene Expression Regulation, Neoplastic
• Gene Expression Profiling
• Single-Cell Gene Expression Analysis

• National Natural Science Foundation of China

• Sarcoma

• Humans
• Female
• Uterine Neoplasms/therapy
• Sarcoma/therapy

• Humans
• Female
• Adolescent
• Sarcoma, Endometrial Stromal/pathology
• Sarcoma, Endometrial Stromal/therapy
• Endometrial Neoplasms/pathology
• Endometrial Neoplasms/therapy
• Translocation, Genetic
• Combined Modality Therapy
• Prognosis
• Fertility Preservation

• Fertility-sparing surgery
• Gynaecologic oncology
• Gynaecologic surgery
• Mesenchymal tumours
• Uterine sarcoma

• Bilateral ovarian metastasis
• Endometrial stromal sarcoma
• Incidental
• Low grade endometrial stromal sarcoma (LG-ESS)

• Double Barrel Wet Colostomy
• Low-Grade Endometrial Stromal Sarcoma
• Thrombectomy
• Total Pelvic Exenteration
• Tumor Board

• Female
• Humans
• Middle Aged
• Endometrial Neoplasms/surgery
• Endometrial Neoplasms/diagnosis
• Sarcoma, Endometrial Stromal/surgery
• Prognosis
• Thrombosis/surgery
• Pelvic Exenteration

• Nomogram
• Overall survival
• Radiotherapy
• SEER
• Uterine sarcoma

• Humans
• Radiotherapy, Adjuvant
• Nomograms
• Pelvic Neoplasms
• Databases, Factual
• Sarcoma/radiotherapy
• SEER Program

• adenosarcoma
• endometrial stromal sarcoma
• guideline
• leiomyosarcoma
• morcellation
• uterine sarcoma

• Female
• Humans
• Pathology, Clinical
• Pathologists
• Research Report
• Carcinoma/pathology
• Sarcoma

Low-grade uterine endometrial stromal sarcoma (LG-ESS) is a rare tumor characterized by an overall good survival but showing a indolent behavior and a variable risk of recurrence. There is no clear consensus on the optimal management of these tumors and no prognostic or predictive factors have been established. With this study, we evaluated the prognostic relevance of several clinical, surgical, and pathological features in patients affected by LG-ESS to identify risk factors associated with recurrence.

We retrospectively analyzed 52 LG-ESS cases, treated from January 1st, 1994, to May 31st, 2020, in two referral centers. The relationship between recurrence and clinicopathological characteristics as well as surgical treatment was investigated. Risk of recurrence and disease-free survival (DFS) were estimated by Cox regression and the Kaplan-Meier analysis, respectively.

Of 52 patients with LG-ESS, 8 experienced recurrence (15%). The median follow-up was 100 months (SD ± 96, range: 15–336). By univariate analysis, fragmentation/morcellation, tumor size, FIGO stage, higher mitotic count, presence of necrosis, and lymphovascular space invasion (LSVI) resulted associated with a poorer outcome. Conversely, the surgical modality (laparotomic vs laparoscopic and hysterectomy with bilateral salpingo-oophorectomy vs local excision) and pelvic lymphadenectomy were not. Even the different modalities of adjuvant therapy (hormonal therapy, radiotherapy, and chemotherapy) showed no prognostic significance. Tumor fragmentation/morcellation and higher mitotic count resulted independent prognostic variables at multivariate analysis.

This data supports the avoidance of any type of morcellation if LG-ESS is suspected preoperatively. Higher mitotic count and, possibly, tumor size, advanced FIGO stage, necrosis, and LVSI could be exploited to tailor the adjuvant therapy, but these results need to be confirmed in larger prospective studies.

•

AIs may offer prolonged disease stability even in metastatic ESS, but response rates are modest.

Endometrial stromal sarcomas (ESS) are rare, accounting for < 1% of all uterine malignancies. Treatment has been guided by small case series and retrospective studies. Endocrine therapy is used in both adjuvant and metastatic settings. Aromatase inhibitors (AIs) are widely used in clinical practice. We sought to evaluate clinical outcomes of AI use in the largest cohort of patients with LGESS to date.

We performed a retrospective study of patients with LGESS treated with an AI at our institution from 1/1998–12/2020. Response was evaluated using RECIST 1.1. The Kaplan-Meier method was used to estimate median progression-free (PFS) and overall (OS) survival.

Forty patients were identified. Treatment was well tolerated, with 57.5% experiencing adverse effects. The most common were arthralgias (12 patients, 30%) and hot flashes (9, 22.5%). Two of 11 patients with RECIST-evaluable imaging experienced a partial response to treatment. Median PFS for the entire cohort was 79.2 months (95% CI 39.7 months to NE); the 5-year PFS rate was 59.6% (95% CI 41.8% to 73.6, p = 0.065). Median follow-up for the 29 survivors was 97.9 months (range: 12.6–226.7). The 5-year OS rate was 81.5% (95% CI 64.9–90.7%). One patient who discontinued AI after 10 years of treatment recurred 1 year later.

AIs were well tolerated and offered periods of prolonged disease stability, even in the metastatic setting. Our study suggests, however, that response rates may be lower than previously reported. Data on optimal duration of treatment is needed, but the rarity of LGESS is an obstacle to conducting large clinical trials.

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.

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.

(1) Patient demographics are summarized in manuscript. 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 and achieved an R0 resection. After the initial surgery, 7 patients who had multiple metastasis were treated with adjuvant chemotherapy, 2 patients with vaginal ESS were treated with chemotherapy and radiation therapy, and 6 patients with ER/PR positive received hormone therapy with or without chemotherapy. (2) Most EESS patients had multiple tumors. The omentum was the most commonly affected site, followed by the ovaries. (3) The median follow-up was 94 (range: 27–228) months, and recurrence was observed in 3 patients (n = 10, 30%) who underwent non-optimal surgery and no hormone therapy. The 5-year and 10-year DFS rates were both 70%, as shown in Fig. 2. OS was both 100% at 5 and 10 years.

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 may significantly reduce the risk of recurrence.

• Clinicopathological features
• Complete resection
• Extra-uterine endometrial stromal sarcoma
• Oral hormonal therapy
• Recurrence rate

Primary ovarian high-grade endometrial stromal sarcoma is a very rare disease. Even though it has poor prognosis, the gold standard treatment has not been established owing to its rarity. This report aimed to present therapeutic options for primary ovarian high-grade endometrial stromal sarcoma.

A 49-year-old Asian woman presented with disseminated intravascular coagulation due to ruptured primary high-grade ovarian endometrial stromal sarcoma with multiple intraperitoneal metastases. After the initial surgery, the patient underwent adjuvant chemotherapy with three courses of Adriamycin (75 mg/m²). We performed the secondary debulking operation including total hysterectomy, metastasectomy, omentectomy, peritonectomy, appendectomy, and hyperthermic intraperitoneal chemotherapy (paclitaxel 175 mg/m²). Currently she has been alive for 28 months under a new chemotherapy regimen.

We suggest cytoreductive surgery with hyperthermic intraperitoneal chemotherapy could be a therapeutic option for primary high-grade ovarian endometrial stromal sarcoma with peritoneal dissemination.

• HIPEC
• Primary ovarian high-grade endometrial stromal sarcoma

Low-grade endometrial stromal sarcoma is a rare neoplastic growth in the uterine cavity, representing less than 1% of uterine tumors. Such tumors usually affect premenopausal and perimenopausal women, with a mean age of 46 years. Treatment generally starts with surgical resection of the tumor, followed by chemotherapy, radiotherapy, or hormonal therapy.

In the current report, we again present a case of low-grade endometrial stromal sarcoma in a 51-year-old Mediterranean woman presenting with abdominopelvic pain. Computed tomography scan revealed a primary uterine tumor measuring 17 × 9 × 9 cm metastasizing to the lungs, bladder, and ureteral orifice, along with lymphovascular involvement. The patient underwent total abdominal hysterectomy, omentectomy, and lymph node dissection. Estrogen deprivation was accomplished by bilateral salpingo-oophorectomy. Lifelong hormonal therapy consisting of letrozole 2.5 mg per day was prescribed, which demonstrated remarkable efficacy, resulting in a partial remission of lung metastasis within 8 months after surgery. Full remission was observed after 18 months of hormonal therapy, with no recurrence. Another scan was performed after 2.5 years, revealing complete remission with no recurrence.

We again report a case of complete remission of low-grade endometrial stromal sarcoma after surgical removal of the tumor along with first-line hormonal therapy without the use of chemotherapy or radiotherapy, emphasizing the role of hormonal therapy in the treatment of such tumors.

• Case report
• Complete remission
• Endometrial stromal sarcoma
• Hormonal therapy
• Letrozole

• Lymph nodes
• Sarcoma
• Uterus

The prognosis of recurrent low-grade endometrial stromal sarcoma (LGESS) is little known. This study was to investigate the survival outcomes of a cohort of patients with recurrent LGESS.

Patients with primary LGESS diagnosed and treated for first recurrence confirmed by histology in the study center from February 2012 to June 2019 were retrospectively included. The progression-free interval (PFI) after the last treatment for first recurrence and overall survival (OS) since the diagnosis of first recurrence, which were followed up to June 1, 2020, were compared between groups of various therapy modalities.

Fifty-six patients were included, and 43 patients (76.8%) had definite follow-up outcomes. The 5-year PFI and OS rates were 30.0% (95% confidence interval [95% CI] 29.2–30.8) and 75.0% (68.0–82.0), respectively. In univariate analysis, only fertility-sparing treatment, ovarian preservation and surgical treatment had a significant impact on the PFI (hazard ratio [HR] 4.5, 3.1, and 0.2; 95% CI 1.5–13.1, 1.3–7.3, and 0.1–0.7; and p = 0.006, 0.009 and 0.006, respectively), but no factor was found to be associated with increased mortality risk. After adjusted with hormone treatment or chemotherapy, surgical treatment had significant effectiveness on OS (HR 0.3 and 0.3, 95% CI 0.1–1.0 and 0.1–1.0, p = 0.045 and 0.049, respectively). None of the patients with fertility-sparing treatment had successful conception, and all experienced repeated relapse.

For patients with recurrent LGESS, fertility-sparing treatment or ovarian preservation should not be provided. Surgery is the treatment of choice, and hormone treatment and/or chemotherapy was effective for the survival benefits of surgical treatment.

The online version contains supplementary material available at 10.1186/s13023-021-01802-8.

• Fertility-sparing treatment
• Low-grade endometrial stromal sarcoma
• Ovarian preservation
• Recurrence
• Surgery
• Survival outcomes

• Low-grade endometrial stromal sarcoma
• oophorectomy
• ovarian preservation
• recurrence

• uterine cancer

• Aged
• Endometrial Neoplasms/pathology
• Female
• Humans
• Kaplan-Meier Estimate
• Middle Aged
• Nomograms
• Proportional Hazards Models
• SEER Program
• Sarcoma, Endometrial Stromal/pathology

• Diagnosis
• Medical treatment
• Radiotherapy
• Surgery
• Uterine sarcomas

• BCOR
• JAZF1-SUZ12
• NTRK
• SMARCA4
• SMARCB1
• YWHAE-NUTM2A/B
• endometrial stromal sarcoma
• gynecological sarcomas
• histology-driven therapy
• leiomyosarcoma
• molecular characterization
• smooth muscle neoplasms
• systemic therapy
• uterine sarcomas

Low‐grade endometrial stromal sarcoma (LG‐ESS) is a rare tumor that lacks a prognostic prediction model. Our study aimed to develop a nomogram to predict overall survival of LG‐ESS patients.

A total of 1172 patients confirmed to have LG‐ESS between 1988 and 2015 were selected from the Surveillance, Epidemiology and End Results (SEER) database. They were further divided into a training cohort and a validation cohort. The Akaike information criterion was used to select variables for the nomogram. The discrimination and calibration of the nomogram were evaluated using concordance index (C‐index), area under time‐dependent receiver operating characteristic curve (time‐dependent AUC), and calibration plots. The net benefits of the nomogram at different threshold probabilities were quantified and compared with those of the International Federation of Gynecology and Obstetrics (FIGO) criteria‐based tumor staging using decision curve analysis (DCA). Net reclassification index (NRI) and integrated discrimination improvement (IDI) were also used to compare the nomogram's clinical utility with that of the FIGO criteria‐based tumor staging. The risk stratifications of the nomogram and the FIGO criteria‐based tumor staging were compared.

Seven variables were selected to establish the nomogram for LG‐ESS. The C‐index (0.814 for the training cohort and 0.837 for the validation cohort) and the time‐dependent AUC (> 0.7) indicated satisfactory discriminative ability of the nomogram. The calibration plots showed favorable consistency between the prediction of the nomogram and actual observations in both the training and validation cohorts. The NRI values (training cohort: 0.271 for 5‐year and 0.433 for 10‐year OS prediction; validation cohort: 0.310 for 5‐year and 0.383 for 10‐year OS prediction) and IDI (training cohort: 0.146 for 5‐year and 0.185 for 10‐year OS prediction; validation cohort: 0.177 for 5‐year and 0.191 for 10‐year OS prediction) indicated that the established nomogram performed significantly better than the FIGO criteria‐based tumor staging alone (P < 0.05). Furthermore, DCA showed that the nomogram was clinically useful and had better discriminative ability to recognize patients at high risk than the FIGO criteria‐based tumor staging.

A prognostic nomogram was developed and validated to assist clinicians in evaluating prognosis of LG‐ESS patients.

• FIGO criteria-based tumor staging
• Low-grade endometrial stromal sarcoma (LG-ESS)
• nomogram
• overall survival
• prognostic model
• risk stratification

• long-term follow-up
• low-grade endometrial stromal sarcoma
• lymphadenectomy

• Adjuvant hormonal treatment
• Low-grade endometrial stromal sarcoma
• Meta-analysis
• Overall survival
• Recurrence

• adenosarcoma
• endometrial stromal sarcoma
• leiomyosarcoma
• morcellation
• uterine sarcoma

Uterine sarcomas (US) are rare mesenchymal tumours accounting approximately for 3%–7% of all uterine cancers. Histologically, US are classified into mesenchymal tumours or mixed epithelial and mesenchymal tumours. The group of mesenchymal tumours includes uterine leiomyosarcoma (uLMS, 65% of cases), endometrial stromal sarcoma (ESS, 21%) – traditionally divided into low grade (LG-ESS) and high grade–undifferentiated uterine sarcoma (5%) and other rare subtypes such as alveolar or embryonal rhabdomyosarcoma. Despite the fact that several drugs demonstrated clinical activity in advanced or metastatic settings, the role of postoperative therapy in US remains controversial. In this review, we have summarised the current state of the art, including the chief trials on adjuvant treatment modalities in US, especially focusing on uLMS, LG-ESS and other rare histotypes.

• Uterine sarcoma
• Uterine leiomyosarcoma
• Endometrial stromal sarcoma
• Adenosarcoma
• Adjuvant therapy
• Chemotherapy
• Radiotherapy

Gynaecological sarcomas account for 3-4% of all gynaecological malignancies and have a poorer prognosis compared to gynaecological carcinomas. Pivotal treatment for early-stage uterine sarcoma is represented by total hysterectomy. Whereas oophorectomy provides survival advantage in endometrial stromal sarcoma is still controversial. When the disease is confined to the uterus, systematic pelvic and para-aortic lymphadenectomy is not recommended. Removal of enlarged lymph-nodes is indicated in case of disseminated or recurrent disease, where debulking surgery is considered the standard of care. Fertility sparing surgery for uterine leiomyosarcoma is not supported by strong evidence, whilst available data on fertility sparing treatment for endometrial stromal sarcoma are more promising. For ovarian sarcomas, in the absence of specific data, it is reasonable to adapt recommendations existing for uterine sarcomas, also regarding the role of lymphadenectomy in both early and advanced stage disease. Specific recommendations on cervical sarcomas' surgery are lacking. Existing data on surgical approach vary from radical hysterectomy to fertility-preserving surgery in the form of trachelectomy or wide local excision, however no definite conclusions can be drafted on the recommended surgical approach. For vulval sarcomas, complete surgical excision with at least 2 cm of free margin is considered to be the primary treatment which is associated with good prognosis. The aim of this review is to provide highest quality evidence to guide gynaecologic oncologists throughout surgical management of gynaecological sarcomas.

• cervical
• ovarian
• sarcoma
• uterine
• vulval

• Adenosarcoma
• Carcinosarcoma
• Endometrial stromal sarcoma
• Leiomyosarcoma
• Uterine sarcoma

• Endometrial stromal sarcoma
• Undifferentiated endometrial sarcoma
• Uterine sarcoma

• Antineoplastic Agents, Hormonal/therapeutic use
• Chemotherapy, Adjuvant/methods
• Endometrial Neoplasms/diagnosis
• Endometrial Neoplasms/pathology
• Endometrial Neoplasms/therapy
• Endometrium/pathology
• Endometrium/surgery
• Female
• Humans
• Hysterectomy/methods
• Lymph Node Excision/methods
• Neoplasm Grading
• Neoplasm Recurrence, Local/diagnosis
• Neoplasm Recurrence, Local/pathology
• Neoplasm Recurrence, Local/therapy
• Radiotherapy, Adjuvant/methods
• Randomized Controlled Trials as Topic
• Reoperation/methods
• Salpingo-oophorectomy/methods
• Sarcoma, Endometrial Stromal/diagnosis
• Sarcoma, Endometrial Stromal/pathology
• Sarcoma, Endometrial Stromal/therapy

• Adult
• Biopsy
• Chemotherapy, Adjuvant/methods
• Endometrial Neoplasms/diagnostic imaging
• Endometrial Neoplasms/pathology
• Endometrial Neoplasms/therapy
• Endometrial Stromal Tumors/diagnostic imaging
• Endometrial Stromal Tumors/pathology
• Endometrial Stromal Tumors/therapy
• Fallopian Tubes/diagnostic imaging
• Fallopian Tubes/surgery
• Female
• Fertility Preservation/methods
• Humans
• Hysterectomy
• Laparoscopy/methods
• Margins of Excision
• Neoplasm Staging
• Organ Sparing Treatments/methods
• Ovary/diagnostic imaging
• Robotic Surgical Procedures/methods
• Salpingectomy
• Uterine Myomectomy
• Uterus/diagnostic imaging
• Uterus/pathology
• Uterus/surgery

• endometrial stromal sarcoma
• leiomyoma
• leiomyosarcoma
• sarcoma
• undifferentiated uterine sarcoma

• Endometrial stromal sarcoma
• Prognosis
• Sarcoma
• Survival
• Treatment
• Uterine malignancy

• distant metastasis
• hormonal treatment
• immunohistochemistry marker
• low-grade endometrial stromal sarcoma
• recurrence
• treatment strategies

• Adult
• Aged
• Antineoplastic Agents, Hormonal/therapeutic use
• Antineoplastic Protocols
• Biomarkers, Tumor/analysis
• Combined Modality Therapy
• Disease-Free Survival
• Endometrial Neoplasms/pathology
• Endometrial Neoplasms/therapy
• Endometrial Stromal Tumors/pathology
• Endometrial Stromal Tumors/therapy
• Female
• Humans
• Hysterectomy
• Immunohistochemistry
• Middle Aged
• Neprilysin/analysis
• Ovariectomy/methods
• Retrospective Studies
• Treatment Outcome

Clinical practice guidelines for gynecologic cancers have been developed by many organizations. Although these guidelines have much in common in terms of the practice of standard of care for uterine corpus cancer, practice guidelines that reflect the characteristics of patients and healthcare and insurance systems are needed for each country. The Korean Society of Gynecologic Oncology (KSGO) published the first edition of practice guidelines for gynecologic cancer treatment in late 2006; the second edition was released in July 2010 as an evidence-based recommendation. The Guidelines Revision Committee was established in 2015 and decided to produce the third edition of the guidelines as an advanced form based on evidence-based medicine, considering up-to-date clinical trials and abundant qualified Korean data. These guidelines cover screening, surgery, adjuvant treatment, and advanced and recurrent disease with respect to endometrial carcinoma and uterine sarcoma. The committee members and many gynecologic oncologists derived key questions from the discussion, and a number of relevant scientific literatures were reviewed in advance. Recommendations for each specific question were developed by the consensus conference, and they are summarized here, together with other details. The objective of these practice guidelines is to establish standard policies on issues in clinical areas related to the management of uterine corpus cancer based on the findings in published papers to date and the consensus of experts as a KSGO Consensus Statement.

• Chemotherapy
• Consensus
• Irradiation
• Practice Guideline
• Surgery
• Uterine Corpus Neoplasms

Endometrial stromal sarcoma (ESS) is a rare malignancy. Development of extrauterine ESS form endometriosis is particularly rare. The majority of extrauterine ESS occurs in areas with preexisting endometriosis. The most common site is the ovary. We experienced a case of ESS of the ovary that arose from endometriosis with multiple disseminated lesions. This disease was managed by total abdominal hysterectomy, bilateral salpingo-oophorectomy, both pelvic lymph nodes dissection, omentectomy, and appendectomy followed by postoperative high-dose progesterone therapy. Here, we report this case with literature review.

• Endometrial stromal sarcoma
• Endometriosis
• Ovary

• Endometrial stromal sarcoma
• Leiomyosarcoma
• Malignant mixed Mullerian tumor
• Outcome
• Undifferentiated endometrial sarcoma
• Uterine sarcoma

• carcinosarcoma
• endometrial stromal sarcoma
• guideline
• leiomyosarcoma
• uterine sarcoma

• Low-grade endometrial stromal sarcoma
• Lymphadenectomy
• Ovarian preservation
• Prognosis
• Treatment