Low-grade Endometrial Stromal Sarcoma Patients Research Articles

Prognosis of uterine and extrauterine low-grade endometrial stromal sarcoma: an observational cohort study.

Little is known about the survival differences between uterine and extrauterine low-grade endometrial stromal sarcoma (LGESS). Survival outcomes, consisting of disease-free survivals and overall survivals (OS), were compared in these two entities. From February 2012 to June 2019, all primary LGESS cases and LGESS cases with first recurrence in the study center were reviewed. The clinicopathological characteristics and survival outcomes of extrauterine and uterine LGESS patients were compared for both primary and recurrent diseases. During the study period, 143 patients with primary LGESS and 56 patients with recurrent LGESS were included and followed up to 1 June 2020, among whom 8 (5.6%) and 10 (17.8%) patients were identified as having extrauterine LGESS. Patients with primary and recurrent extrauterine LGESS had similar clinicopathological characteristics to those of patients with uterine LGESS. In primary or in recurrent LGESS cases, in univariate analysis, patients with uterine and extrauterine LGESS had similar disease-free intervals after the last treatment, and they also had similar OSs after the diagnosis. Ovarian preservation led to significantly increased recurrence for primary LGESS [hazard ratio (HR) 4.9, 95% CI: 2.3-10.1, P <0.001) and repeated recurrence for recurrent LGESS (HR 3.1, 95% CI: 1.3-7.3, P =0.009). Surgical treatment for recurrent LGESS decreased repeated recurrence after the first recurrence (HR 0.2, 95% CI: 0.1-0.7, P =0.006). No factors were found to be associated with the OS of primary or recurrent LGESS. The clinical characteristics and survival outcomes of extrauterine LGESS are similar to those of uterine LGESS. Surgery is the treatment of choice for recurrent LGESS. Ovarian preservation is detrimental to disease-free survival but not to OS in both uterine and extrauterine LGESS.

Low-grade endometrial stromal sarcoma with intracaval or intracardiac extension: a retrospective study of eight cases.

This study aimed to improve the knowledge of low-grade endometrial stromal sarcoma (LG-ESS) with intracaval or intracardiac extension and tried to identify the potential risk factors and optimal treatment method influencing prognosis. We performed a retrospective review of eight LG-ESS patients with intracaval or intracardiac extension who underwent treatment at Peking Union Medical College Hospital between 2012 and 2020. The median age at diagnosis was 44years, ranging from 28 to 56years. Abnormal uterine bleeding was the most common intimal symptom (3/8), followed by low back discomfort (2/8), edema of the lower limbs (2/8), abdominal pain (1/8), and dyspnea (1/8). All patients underwent resection of the intravascular and extravascular portions of the tumor. Two patients were in stage IIIC, and six were in stage IVB. After surgery, four patients received adjuvant radiotherapy, of whom three also received letrozole. One patient was treated with letrozole alone, and one patient received medroxyprogesterone. The average follow-up time was 34.5months, ranging from 6 to 98months. No patients died or relapsed during the follow-up period. LG-ESS with intracaval or intracardiac extension is an uncommon type of tumor which is easily misdiagnosed and can only be diagnosed by histological evaluation after surgery. Complete tumoral excision followed by adjuvant therapy may benefit patient survival time. Long-term follow-up is essential due to the high rate of late recurrence.

Computer-aided diagnosis of low grade endometrial stromal sarcoma (LGESS)

Low grade endometrial stromal sarcoma (LGESS) accounts for about 0.2% of all uterine cancer cases. Approximately 75% of LGESS patients are initially misdiagnosed with leiomyoma, which is a type of benign tumor, also known as fibroids. In this research, uterine tissue biopsy images of potential LGESS patients are preprocessed using segmentation and stain normalization algorithms. We then apply a variety of classic machine learning and advanced deep learning models to classify tissue images as either benign or cancerous. For the classic techniques considered, the highest classification accuracy we attain is about 0.85, while our best deep learning model achieves an accuracy of approximately 0.87. These results clearly indicate that properly trained learning algorithms can aid in the diagnosis of LGESS.

A nomogram for predicting overall survival in patients with low-grade endometrial stromal sarcoma: A population-based analysis.

BackgroundLow‐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.MethodsA 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.ResultsSeven 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.ConclusionsA prognostic nomogram was developed and validated to assist clinicians in evaluating prognosis of LG‐ESS patients.

The clinical benefits of hormonal treatment for LG-ESS: a meta-analysis.

To evaluate the clinical benefits of hormonal treatment for patients with low-grade endometrial stromal sarcoma (LG-ESS) by reviewing the published literature and performing a meta-analysis. Correlational studies related to hormonal treatment for LG-ESS patients were collected by searching the PubMed, EMBASE, and Cochrane databases up to December 2018. Eligible studies were selected based on inclusion and exclusion criteria. The main inclusion criteria included: original studies with definite diagnoses of LG-ESS that evaluated the clinical benefits of hormonal treatment, studies with at least 10 cases, and studies published in English. Reviews, case reports, letters, comments or conference abstracts, studies without sufficient data and overlapping or republished studies were excluded. The study quality was evaluated, and pooled relative risks and 95% confidence intervals were calculated using Review Manager 5.3. A total of 10 retrospective studies were included. The NOS stars of the 10 studies ranged from 7 to 9 points, which was considered to be of high quality. Recurrence and death information was provided in 9 and 6 studies, respectively. The overall pooled RR for recurrence was 0.66 (95% CI 0.47-0.94), which indicated that hormonal treatment was effective at reducing the recurrence risk (P = 0.02). The overall pooled RR for death was 0.81 (95% CI 0.59-1.12), which showed that hormonal treatment had little effect in prolonging overall survival (P = 0.20). Stratified analysis showed that compared with the group without any adjuvant treatments, hormonal treatment alone significantly decreased the risk of recurrence (P = 0.02), while hormonal treatment had no significant effects on overall survival (P = 0.38). Another subgroup analysis indicated that for stage I-II patients, hormonal treatment could significantly decrease the risk of recurrence (P = 0.02) but could not influence overall survival (P = 0.87). However, for stage III-IV patients, hormonal treatment had little benefit both in reducing the recurrence risk and prolonging overall survival (P = 0.49/0.08). Egger's and Begg's test showed that the publication bias for the literature was satisfactorily controlled. Adjuvant hormonal treatment should be considered as a feasible adjuvant therapy for reducing the recurrence risk of patients with LG-ESS while bearing little benefit on overall survival.

Low-Grade Endometrial Stromal Sarcoma and Uterine Adenosarcoma: A Comparison of Clinical Manifestations and Outcomes.

Objective: Our study aimed to assess factors associated with progression free survival (PFS) and overall survival (OS) in low grade endometrial stromal sarcoma (LG-ESS) and uterine adenosarcoma, and to determine the differences in clinical manifestations and outcomes between the two diseases.Methods: A total of 132 patients were enrolled in this retrospective study at Peking Union Medical College Hospital from 1998 to 2016. The associations of clinical and pathological factors with PFS and OS were evaluated.Results: Of the 132 included patients, 104 had LG-ESS and 28 had uterine adenosarcoma. All patients were followed up for at least 1.5 years. There were significant differences between LG-ESS and uterine adenosarcoma in terms of age distribution (41.05±10.5 vs 46.11±14.96 years, P=0.042), delivery time (nulliparity=0: 18.27% vs 35.71%, P=0.046), history of the uterine leiomyoma (65.38% vs 39.29%, P=0.012), and polypoid tumor growth (14.42% vs 60.71%, P=0.007). According to the pathological findings, the proportion of uterine adenosarcoma patients with uterine leiomyoma (60.71%) was significantly higher than that for the LG-ESS patients (32.69%) (P=0.007). Uterine adenosarcoma seemed to be associated with longer PFS and OS than LG-ESS (PFS: 42.69±29.94 vs 50.50±40.50 months; OS: 58.72±37.29 vs 69.46±47.58 months), but the differences were not statistically significant. Multivariate Cox regression showed that age, menopause, history of uterine leiomyoma, stage, and hormone therapy were independent risk factors with respect to PFS, whereas age and stage were risk factors affecting OS in LG-ESS patient. Peritoneal lavage cytology and radiotherapy were risk factors affecting PFS and peritoneal lavage cytology for OS in patients with uterine adenosarcoma.Conclusion: The patients with advanced LG-ESS had poor prognosis. Age and history of uterine leiomyoma were associated with poor PFS, while menopause and hormone therapy were protective factors associated with improved PFS in patients with LG-ESS. Peritoneal lavage cytology and radiotherapy did not improve prognosis of uterine adenosarcoma.

Ovary and uterus-sparing procedures for low-grade endometrial stromal sarcoma: A retrospective study of 153 cases

ObjectiveTo discuss the optimal treatment options for low grade endometrial stromal sarcoma (LG-ESS). MethodsMedical records of consecutive patients with LG-ESS in our institute were collected. The pertinent data, including clinicopathological characteristics, treatment and prognostic information were evaluated. ResultsA 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. ConclusionHysterectomy 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.

Prolonged long-term survival of low-grade endometrial stromal sarcoma patients with lung metastasis following treatment with medroxyprogesterone acetate

The aim of this study was to investigate the usefulness of medroxyprogesterone acetate (MPA) therapy for patients with metastatic low-grade endometrial stromal sarcoma (LGESS). A retrospective review was performed of five patients with metastatic LGESS lesions in whom MPA therapy prolonged survival. The diagnosis was established by hysterectomy in all five patients. Three patients had stage I disease and two patients had stage IV. The median follow-up period was 77 months (range, 15-283 months). All five patients had recurrent disease in the lung. The median disease-free interval was 50 months (range, 7-120 months). Three of the five patients received several types of chemotherapy, and all of these patients received the same MPA (200-600 mg/day) hormonal therapy. One patient died 149 months after disease recurrence. Interestingly, after the recurrence in the lung, three patients were alive with persistent pulmonary metastases for more than 120 months. The median overall survival from the time of recurrence was 41 months (range, 9-163 months). The patients in this study demonstrate that MPA treatment may extend the survival of patients with LGESS that is metastatic to the lung.

Lymphadenectomy and Ovarian Preservation in Low-Grade Endometrial Stromal Sarcoma

To report the impact on overall survival of lymphadenectomy and ovarian preservation in patients with endometrial stromal sarcoma. Data were extracted from the Surveillance, Epidemiology, and End Results Program from 1988 to 2005. Kaplan-Meier and Cox proportional hazards analyses were used to identify possible predictors for survival. Nine hundred seventy women were reported with endometrial stromal sarcoma. The 384 women with low-grade endometrial stromal sarcoma had a younger age, earlier stage, and longer survival than the 320 women with high-grade lesions. Among the low-grade endometrial stromal sarcoma patients, the incidence of extrauterine disease was 25%, and lymph node metastasis was 7%. Univariable and multivariable analysis demonstrated lymph node metastasis and ovarian preservation were not significant prognostic factors for survival. In low-grade endometrial stromal sarcoma, the risk of extrauterine spread and lymph node metastasis merit consideration for surgical staging. Neither lymph node metastasis nor ovarian preservation seems to affect the excellent overall survival of these patients. II.

Hormonal therapy of endometrial stromal sarcoma

Low-grade endometrial stromal sarcomas are steroid receptor positive tumors with slow tumor progression and high recurrence rates, which lack established treatment protocols. We present an update on hormonal therapy options. In the past, hormonal therapy consisted of progestins for advanced/recurrent/metastatic low-grade endometrial stromal sarcomas. Aromatase inhibitors and gonadotropin-releasing hormone analogues have become new effective alternatives for first and second line treatment. The high recurrence rates after short disease free intervals in low-grade endometrial stromal sarcoma patients were partly due to inadvertent growth stimulation during estrogen-containing hormone replacement therapy and tamoxifen treatment, which - according to current knowledge - are contraindicated. Recently, hormonal therapy has been introduced for the prevention of recurrences. Aromatase inhibitors are becoming the treatment of choice, since progestins are poorly tolerated due to side effects. The effective duration of preventive hormonal therapy is still undetermined. Hormonal therapy with progestins, aromatase inhibitors and gonadotropin-releasing hormone analogues has become an effective treatment alternative to radiation and chemotherapy for low-grade endometrial stromal sarcoma patients. Preventive hormonal therapy is of particular interest in the setting of concomitant endometriosis.