Low-Grade Endometrial Stromal Sarcoma - a Review

Thiel, Falk; Halmen, Sonja

doi:10.1159/000494225

Cited by 62 publications

(68 citation statements)

References 64 publications

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“…This disease was also known to affect females in their 50s to 60s while our data showed that patients with stage I LGESS were initially diagnosed in their 40s . Approximately 30–50% of patients with ESS show atypical symptom of abnormal uterine bleeding . Positive lymph nodes are usually associated with extrauterine metastasis, deep myometrial invasion and lymphvascular space invasion, which also could enlarge nodes .…”

Section: Discussionmentioning

confidence: 64%

“…9 Approximately 30-50% of patients with ESS show atypical symptom of abnormal uterine bleeding. 13 Positive lymph nodes are usually associated with extrauterine metastasis, deep myometrial invasion and lymphvascular space invasion, which also could enlarge nodes. 15,23 Therefore, the benefits of systematic LND remain controversial.…”

Section: Discussionmentioning

confidence: 99%

“…[7][8][9][10] Different treatments are given for LGESS and HGESS. [9][10][11][12] Even though LGESS is a heterogeneous uterine sarcoma that accounts for a large proportion of ESS, 10,13,14 few studies on LGESS have been conducted since it is a rare disease. Currently, the primary treatment for early stage is a total hysterectomy (TH) plus a bilateral salpingo-oophorectomy (BSO).…”

Section: Introductionmentioning

confidence: 99%

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Long‐term impact of lymphadenectomies in patients with low‐grade, early‐stage uterine endometrial stroma sarcoma

Zhang

Wang

et al. 2020

J of Obstet and Gynaecol

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Aim The aim of our study was to investigate the lymph node metastasis (LNM) rate and effect of lymph node dissection (LND) in patients with stage I, low‐grade endometrial stromal sarcoma (LGESS). Methods Patients with stage I LGESS (n = 119) that underwent surgery from July 1969 to July 2017, following up over 48 years at the China National Cancer Center were retrospectively analyzed in this study. Results Surgical records and consulting data for patients with LGESS were analyzed to find that 47 patients received systematic pelvic LND. The number of patients with menopause in the LND(+) group were significantly lower than those in LND(−) group (2.1% vs 22.2%, P = 0.005), meanwhile, patients received bilateral salpingo‐oophorectomy procedure in LND(+) group were significantly higher than LND(−) (97.9% vs 58.3%, P < 0.001). Neither progression‐free survival nor overall survival was significantly improved in the LND(+) group even after propensity score matching although the progression‐free survival has a stronger trend in LND(+) population. Conclusion A systematic LND was not significantly associated with prognosis for patients with early‐stage LGESS. There is no sufficient indication for a systematic LND for patients with early‐stage LGESS. A systematic LND might be necessary if enlarged lymph nodes were detected by image graphology or observation during surgery.

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Section: Discussionmentioning

confidence: 64%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Long‐term impact of lymphadenectomies in patients with low‐grade, early‐stage uterine endometrial stroma sarcoma

Zhang

Wang

et al. 2020

J of Obstet and Gynaecol

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“…Therefore, they have better prognosis than other uterine sarcomas [17]. In most cases, the recurrence period varied from 3 months to 23 years, with a median interval of 3 years [18]. Long-term follow-up is critical due to the high longterm recurrence rate.…”

Section: Discussionmentioning

confidence: 99%

“…According to the literature, the most frequently reported sites of metastases of the LG-ESS are the vagina, pelvis, and peritoneal cavity. Cardiac metastases are rare because ESS commonly spreads through the lymph nodes and venous system [6].To our knowledge, 18 LG-ESS patients with heart involvement have been reported to date, and their clinical characteristics are summarized as shown in Table 1.…”

Section: Discussionmentioning

confidence: 99%

Low-Grade Endometrial Stromal Sarcoma with Intravenous and Intracardiac Extension: A Case Report

Chai

Ding

Zhou

et al. 2020

Preprint

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Background: Low-grade endometrial stromal sarcoma (ESS) is rare mesenchymal neoplasm, which has an indolent history with late recurrences. ESS usually spread through the lymph nodes and venous system but very seldom involve large vessels or the heart.Case presentation: A 38-year-old Chinese woman was admitted to our department due to pelvic mass found on physical examination. The superior and inferior vena cava CT angiography (CTA) showed an enlarged uterine as well as low density image in the left internal iliac vein, the left common iliac vein, the inferior vena cava, the left renal vein adjacent to the heart and the right atrium, with a range of 110*16mm. The filling defect of right atrium was about 30*14mm. The three-dimensional computed tomography reconstruction showed that the mass originated from the uterine and invaded into the reproductive vein, subsequently extended along the inferior vena cava to the right atrium. Needle biopsy of the pelvic mass was performed and the tissue indicates smooth muscle. The preoperative diagnosis was intravascular leiomyomatosis and the patient underwent radical resection: thrombectomy and total hysterectomy with bilateral salpingo-oophorectomy. Postoperative histopathology revealed low grade endometrial stromal sarcoma. Microscopically, the tumors in both original uterine lesions and intravascular and intracardiac metastases shared morphologic features characterized by neoplastic cells similar to proliferative-phase endometrial stromal cells, in which small spiral artery differentiation was recognized and tumor tissue showed invasive growth pattern by inserting into the surrounding smooth muscle. Immunohistochemistry showed tumor cells were reactive to Estrogen Receptor, Progesterone Receptor,CD10. Primary uterine foci showed cyclin D1(5%+) and Ki-67(20%+),whereas metastatic lesions of the intracardiac and the intravascular component identified cyclin D1(negative) and Ki-67(2%+). The patient is alive without evidence of recurrence 3 months after surgery.Conclusions: Distant metastasis of low-grade endometrial stromal sarcoma is rare, especially involving large vessels or the heart. This case demonstrates that malignant tumor metastasis should be considered as a differential diagnosis of intracardiac and intravascular masses. The treatment rely on multidisciplinary cooperation.

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

Zhang

et al. 2020

Cancer Communications

289

189

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

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Low-Grade Endometrial Stromal Sarcoma - a Review

Cited by 62 publications

References 64 publications

Long‐term impact of lymphadenectomies in patients with low‐grade, early‐stage uterine endometrial stroma sarcoma

Long‐term impact of lymphadenectomies in patients with low‐grade, early‐stage uterine endometrial stroma sarcoma

Low-Grade Endometrial Stromal Sarcoma with Intravenous and Intracardiac Extension: A Case Report

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

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