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Introduction: Extrauterine recurrent metastasis of Low-grade endometrial stromal sarcoma (LG-ESS) to major blood vessels is largely rare with few reported cases. Case: Herein, we present a case of a 51-year-old female with recurrent LG-ESS that has metastasized after 12 years to the inferior vena cava (IVC) and extended into the right atrium and common iliac veins. Computed tomography showed an intracardiac larger thrombus within the right atrium extending into the inferior vena cava and common iliac veins. The patient underwent cytoreduction of the intravenous tumor. Final histopathology confirmed metastatic LG-ESS. Follow-up CT scan after six months showed no residual or recurrence. Conclusion: Treatment for extrauterine metastasis from LG-ESS prioritizes reducing tumor burden through surgical resection and may require collaboration across disciplines. Follow-up hormonal therapy with progestins or aromatase inhibitors significantly impacts patient prognosis.

BackgroundLow-grade endometrial stromal sarcoma (LG-ESS) is a rare uterine malignancy characterized by its complex tumor microenvironment (TME) and high recurrence rates, posing challenges to accurate prognosis and effective treatment. Identifying prognostic biomarkers is essential for improving patient stratification and guiding therapeutic strategies.MethodsUsing single-cell transcriptome analysis combined with H&E and multiplex immunofluorescence staining, we identified a subpopulation of tumor cells in LG-ESS and further validated the association of this subpopulation and its characteristic genes with LG-ESS prognosis by molecular characterization and bulk transcriptome data.ResultsOur analysis reveals multiple cellular subpopulations within the tumor tissue, particularly a tumor cell subpopulation among them which is associated with poor prognosis. Originating from normal stromal fibroblasts, this subpopulation appears to play a crucial role in TME remodeling, smooth muscle cell behavior, and potentially in tumorigenesis and metastasis. Of particular interest in this subpopulation is the highly expressed FGF12 gene, which is significantly associated with a shortened survival in ESS, highlighting its potential as a prognostic biomarker.ConclusionOur study reveals the complexity of TME within the LG-ESS and highlights the role that tumor cell subpopulations play in disease progression and patient prognosis. The identification of FGF12 as a prognostic biomarker suggests a new approach for the personalized treatment and prognosis monitoring of patients.

Objective: 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. Methods: 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. Results: 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. Conclusion: 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. [ABSTRACT FROM AUTHOR]

Low-grade endometrial stromal sarcoma (LG-ESS) is a rare uterine neoplasm. Computed tomography (CT) revealed the presence of multiple small bilateral pulmonary nodules in a 58-year-old woman 1 year after surgery for LG-ESS; the clinical diagnosis was pulmonary metastasis. Hormone therapy with progesterone was initiated, after which most of the solid nodules disappeared and some transformed into cystic lesions. Seven years after hormone therapy, the patient experienced repeated pneumothorax. The cause of the pneumothorax was perforation of a metastatic focus within the wall of a small subpleural cyst that was not evident on CT images. [ABSTRACT FROM AUTHOR]

Low-grade endometrial stromal sarcoma (LG-ESS) is a rare uterine malignancy, accounting for less than 1% of all uterine malignancies. And there is no literature reporting about acute obstruction of inferior vena cava (IVC) caused by LG-ESS. In November 2021, A patient diagnosed with LG-ESS was admitted to our hospital, presenting with pitting edema of the right lower limb, menstrual disturbance, nausea, oliguria andincreased serum creatinine. Imaging suggested complete obstruction of IVC cava. This difficult case was successfully treated with staging surgery combined with endocrine therapy by a cooperative multidisciplinary team (MDT). The important role of MDT in the treatment process of such a difficult and serious case was demonstrated.

Purpose: Uterine sarcoma (US) as a histologically heterogeneous group of tumors is rare and associated with poor prognosis. Prognostic factors based on systematic data collection need to be identified to optimize patients' treatment. Methods: This unicenter, retrospective cohort study includes 57 patients treated at the University Hospital Freiburg, Germany between 1999 and 2017. Progression-free survival (PFS) and overall survival (OS) were calculated and visualized in Kaplan–Meier curves. Prognostic factors were identified using log-rank test and Cox regression. Results: 44 Leiomyosarcoma (LMS), 7 low-grade endometrial stromal sarcoma (LG-ESS), 4 high-grade ESS and 2 undifferentiated US patients were identified. The median age at time of diagnosis was 51.0 years (range 18–83). The median follow-up time was 35 months. PFS for the total cohort was 14.0 (95%-Confidence-Interval (CI) 9.7–18.3) and OS 36.0 months (95%-CI 22.1–49.9). Tumor pathology was prognostically significant for OS with LG-ESS being the most favorable (mean OS 150.3 months). In the multivariate analysis, patients over 52 years showed a four times higher risk for tumor recurrence (hazard ratio (HR) 4.4; 95%-CI 1.5–12.9). Progesterone receptor negativity was associated with a two times higher risk for death (HR 2.8; 95%-CI 1.0–7.5). For LMS patients age ≥ 52 years (p = 0.04), clear surgical margins (p = 0.01), FIGO stage (p = 0.01) and no application of chemotherapy (p = 0.02) were statistically significant factors for OS. Conclusion: Tumor histology, age at time of diagnosis and progesterone receptor status were prognostic factors for US. Unfavorable OS in LMS patients was associated with advanced FIGO stage, suboptimal cytoreduction and application of chemotherapy. [ABSTRACT FROM AUTHOR]

This article reports a case of low-grade endometrial stromal sarcoma (LG-ESS), which was accidentally found after 2 times of transumbilical laparoendoscople single-site surgery (TU-LESS), retrospectively. The patient was diagnosed with uterine fibroid before the first operation. The uterine fibroid was removed by TU -LESS bag cold knife rotary cutting. The pathological diagnosis was LG-ESS after the operation. After 30 days, a small amount of peritoneal planting was seen in the TULESS comprehensive staging operation again. Progesterone treatment was given after the operation. The tumor markers were normal after 20 months of follow-up. The umbilical incision and vaginal stump healed well. TU-LESS has the characteristics of light pain, beautiful wound, and rapid postoperative recovery. The most important thing is that the myoma specimens can be safely and effectively removed to avoid the widespread of sarcoma in the whole abdomen caused by the use of electric rotary cutter. However, during the operation, it is still necessary to be vigilant for peritoneal implantation caused by tumor splash during myomectomy, and each operation should strictly follow the principle of tumor-free. [ABSTRACT FROM AUTHOR]

Purpose: 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. Methods: 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. Results: The median age at diagnosis was 44 years, ranging from 28 to 56 years. 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 34.5 months, ranging from 6 to 98 months. No patients died or relapsed during the follow-up period. Conclusions: 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. [ABSTRACT FROM AUTHOR]

Background: Low-grade endometrial stromal sarcoma (LG-ESS) is a rare uterine malignancy characterized by its complex tumor microenvironment (TME) and high recurrence rates, posing challenges to accurate prognosis and effective treatment. Identifying prognostic biomarkers is essential for improving patient stratification and guiding therapeutic strategies., Methods: Using single-cell transcriptome analysis combined with H&E and multiplex immunofluorescence staining, we identified a subpopulation of tumor cells in LG-ESS and further validated the association of this subpopulation and its characteristic genes with LG-ESS prognosis by molecular characterization and bulk transcriptome data., Results: Our analysis reveals multiple cellular subpopulations within the tumor tissue, particularly a tumor cell subpopulation among them which is associated with poor prognosis. Originating from normal stromal fibroblasts, this subpopulation appears to play a crucial role in TME remodeling, smooth muscle cell behavior, and potentially in tumorigenesis and metastasis. Of particular interest in this subpopulation is the highly expressed FGF12 gene, which is significantly associated with a shortened survival in ESS, highlighting its potential as a prognostic biomarker., Conclusion: Our study reveals the complexity of TME within the LG-ESS and highlights the role that tumor cell subpopulations play in disease progression and patient prognosis. The identification of FGF12 as a prognostic biomarker suggests a new approach for the personalized treatment and prognosis monitoring of patients., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision., (Copyright © 2024 Miao, Dong, Zhou, Thiel, Li, Cai, Yuan, Wang, Jin, Yang, Wang, Frey, Gaipl, Ma and Zhou.)

We present the first case of low-grade endometrial stromal sarcoma (ESS) invading a leiomyoma, which was difficult to diagnose preoperatively. A 49-year-old multiparous woman was referred to our institution due to the enlargement of an old leiomyoma after menopause. Transvaginal ultrasonography revealed a 40-mm lesion in the myometrium of the uterine body with calcification and edema. Color Doppler imaging showed blood flow along the margins of the mass. Pelvic contrast-enhanced magnetic resonance imaging (MRI) revealed a 39-mm mass in the uterine body, predominantly having a low intensity on T2-weighted images, suggesting leiomyoma containing a cystic lesion with a small solid component. This solid component appeared to have a high intensity on T2-weighted images, high intensity on diffusion-weighted images, low value on apparent diffusion coefficient (ADC) map images, and contrast effect. 18 F-fluoro-deoxyglucose positron emission tomography (FDG-PET)-computed tomography (CT) showed a non-significant FDG uptake in the cyst's solid component. Based on a preoperative diagnosis of cystic or myxoid degeneration of leiomyoma, laparoscopic total hysterectomy and bilateral salpingo-oophorectomy were performed. The uterus was retrieved vaginally without morcellation. Macroscopic examination of the hysterectomy specimens revealed a 55-mm white solid tumor with scattered yellow nodules. Histopathological analysis identified spindle-shaped smooth muscle cells with non-atypical nuclei, confirming a leiomyoma. However, the tumor's nodules contained slightly atypical cells with round nuclei, resembling endometrial stromal cells interspersed with small blood vessels. Immunohistochemical staining showed the nodules were negative for alpha-smooth muscle actin and positive for CD-10, estrogen receptor, and progesterone receptor. These nodules invaded the leiomyoma along vascular vessels. The final diagnosis was leiomyoma coexisting with low-grade ESS, classified as International Federation of Gynecology and Obstetrics (FIGO) stage IB. The patient received no further treatment and remains disease-free after 45 months., Competing Interests: Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Inoue et al.)

Sarcomas of the uterine corpus are rare malignant neoplasms, which are further classified into mesenchymal tumors, and mixed (epithelial plus mesenchymal) tumors. The main issues concerning these neoplasms are the small number of clinical trials, insufficient data from evidence-based medicine, insignificant interest from the pharmaceutical industry, all of which close a vicious circle. The low frequency of these malignancies implies insufficient experience in the diagnosis, hence incomplete surgical and complex treatment. Additionally, the rarity of these sarcomas makes it very difficult to develop clinical practice guidelines. Preoperative diagnosis, neoadjuvant and adjuvant chemoradiation, target and hormone therapies still raise many controversies. Disagreements about the role and type of surgical treatment are also often observed in medical literature. There are still insufficient data about the role of pelvic lymph node dissection and fertility-sparing surgery. Pathologists’ experience is of paramount importance for an accurate diagnosis. Additionally, genetics examinations become part of diagnosis in some sarcomas of the uterine corpus. Some gene mutations observed in uterine sarcomas are associated with different outcomes. Therefore, a development of molecular classification of uterine sarcomas should be considered in the future. In this review, we focus on the epidemiology, pathogenesis, pathology, diagnosis and treatment of the following sarcomas of the uterine corpus: leiomyosarcoma, low- and high-grade endometrial stromal sarcomas, undifferentiated sarcoma and adenosarcoma. Uterine carcinosarcomas are excluded as they represent an epithelial tumor rather than a true sarcoma.

Endometrial stromal sarcoma (ESS) rarely causes infertility in young women. We report a nulligravida in her 30s who presented with primary infertility of 15 years. Hysteroscopy revealed a submucosal necrotic fibroid polyp. Ultrasonography detected multiple intramural fibroids. Open myomectomy with polypectomy was performed. Histopathology revealed low-grade ESS (LGESS) within the fibroid polyp. Subsequently, the patient underwent completion surgery. Her final diagnosis was estrogen-receptor positive LGESS stage IIIB, and she was suggested anastrozole adjuvant therapy and long-term surveillance. ESS with abnormal perimenopausal bleeding, though the most common presentation, may not always observed. Hence, a high index of suspicion of ESS should always be kept as a differential diagnosis in uterine fibroid polyp, though rare. Considering the scarcity of more extensive studies on ESS, reporting of cases will aid in formulating management protocols. [ABSTRACT FROM AUTHOR]

Abstract Background 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. Methods 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. Results 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. Conclusion 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.

Low-grade endometrial stromal sarcoma (LG-ESS) is a rare and indolent malignancy. Hormone therapy has been reported as an adjuvant treatment for LG-ESS, although its effectiveness is controversial. Here we aimed to investigate the effects of postoperative hormone therapy on recurrence in patients with uterine LG-ESS. Between January 2010 and December 2019, a total of 152 patients (23 with and 129 without fertility-sparing) with a diagnosis of primary uterine LG-ESS confirmed by pathologists were enrolled in this study. In the cohort without fertility-sparing, 22 (17.7%) patients had recurrence, and the median disease-free survival (DFS) was 47 (2-130) months; only one of these patients died of LG-ESS. No significant difference was found in recurrence between the groups with and without hormone therapy (p=0.802). However, subgroup analysis showed that hormone therapy decreased the recurrence rate in stage II-IV (p=0.001, HR 0.144, 95% CI: 0.038-0.548), but not in stage I disease (p=0.256). High-dose progestins notably reduced recurrence (p=0.012, HR 0.154, 95% CI: 0.036-0.660), whereas non-progestin therapy marginally influenced recurrence (p=0.054) compared with no hormone therapy in stage II-IV disease. Moreover, hormone therapy within 12 months was effective in reducing recurrence (p=0.038, HR 0.241, 95% CI: 0.063-0.922). Ovarian preservation (p=0.004, HR 6.250, 95% CI: 1.786-21.874) and negative expression of ER/PR (p=0.000, HR 23.249, 95% CI: 4.912-110.026) were high-risk factors for recurrence in patients without fertility-sparing. In the fertility-sparing cohort, 15 (65.2%) patients experienced recurrence, and the median DFS was 24 (3-107) months. Six patients successfully delivered healthy fetuses, and five received hormone therapy. Twelve patients finally accepted hysterectomy after repeated recurrence, and only two of them had given birth before surgery. Patients who received hormone therapy showed longer DFS, although this difference was not statistically significant (p=0.466). In conclusion, postoperative hormone therapy reduces recurrence in patients with stage II–IV uterine LG-ESS without fertility-sparing, and high-dose treatment with progestins within 12 months is recommended. Bilateral oophorectomy can also reduce the risk of recurrence. Patients with fertility-sparing have a high risk of recurrence and poor pregnancy outcomes, and hormone therapy may be a reasonable choice in postoperative management. [ABSTRACT FROM AUTHOR]

Low-grade endometrial stromal sarcoma (LG-ESS) is a rare and indolent malignancy. Hormone therapy has been reported as an adjuvant treatment for LG-ESS, although its effectiveness is controversial. Here we aimed to investigate the effects of postoperative hormone therapy on recurrence in patients with uterine LG-ESS. Between January 2010 and December 2019, a total of 152 patients (23 with and 129 without fertility-sparing) with a diagnosis of primary uterine LG-ESS confirmed by pathologists were enrolled in this study. In the cohort without fertility-sparing, 22 (17.7%) patients had recurrence, and the median disease-free survival (DFS) was 47 (2-130) months; only one of these patients died of LG-ESS. No significant difference was found in recurrence between the groups with and without hormone therapy (p=0.802). However, subgroup analysis showed that hormone therapy decreased the recurrence rate in stage II-IV (p=0.001, HR 0.144, 95% CI: 0.038-0.548), but not in stage I disease (p=0.256). High-dose progestins notably reduced recurrence (p=0.012, HR 0.154, 95% CI: 0.036-0.660), whereas non-progestin therapy marginally influenced recurrence (p=0.054) compared with no hormone therapy in stage II-IV disease. Moreover, hormone therapy within 12 months was effective in reducing recurrence (p=0.038, HR 0.241, 95% CI: 0.063-0.922). Ovarian preservation (p=0.004, HR 6.250, 95% CI: 1.786-21.874) and negative expression of ER/PR (p=0.000, HR 23.249, 95% CI: 4.912-110.026) were high-risk factors for recurrence in patients without fertility-sparing. In the fertility-sparing cohort, 15 (65.2%) patients experienced recurrence, and the median DFS was 24 (3-107) months. Six patients successfully delivered healthy fetuses, and five received hormone therapy. Twelve patients finally accepted hysterectomy after repeated recurrence, and only two of them had given birth before surgery. Patients who received hormone therapy showed longer DFS, although this difference was not statistically significant (p=0.466). In conclusion, postoperative hormone therapy reduces recurrence in patients with stage II–IV uterine LG-ESS without fertility-sparing, and high-dose treatment with progestins within 12 months is recommended. Bilateral oophorectomy can also reduce the risk of recurrence. Patients with fertility-sparing have a high risk of recurrence and poor pregnancy outcomes, and hormone therapy may be a reasonable choice in postoperative management.

Abstract Background Long-term follow-up reports of low-grade endometrial stromal sarcoma (LGESS) including its clinical course and pathological data are rare. We previously reported the case of a Japanese woman diagnosed with LGESS, who was treated with multidisciplinary therapy. She had been suffering from uterine cervical tumor diagnosed as cervical polyps, or fibroid in statu nascendi, since 24 years old. The patient had survived for 25 years with the disease. This report presents her progress and pathological change since the previous report. Case presentation At age 45, the patient experienced a relapse of the remnant LGESS tumor between the right diaphragm and liver. Although chemotherapy was not effective, the tumor was eliminated by proton therapy. At age 46 years, the remnant tumors outside the irradiated field were resected. The disease was originally diagnosed as “neuroendocrine carcinoma (NEC)” using the surgical specimen. Therefore, cisplatin and irinotecan combination chemotherapy were administered to treat the remnant dissemination. After 4 cycles of chemotherapy, the liver metastases had enlarged and were resected surgically. Consequently, no remnant tumor was visible in the abdominal cavity at the end of the surgery. To determine the origin of NEC, we examined the previously resected specimens obtained from her ileum at age 40 years. A boundary between the LGESS and neuroendocrine tumor grade 2 (NET G2)-like lesion was found in the tumor, indicating that the origin of these tumors was LGESS. After less than 2 years of chemotherapy and undergoing surgery, a relapse of the tumor in the liver induced biliary duct obstruction with jaundice, which was treated with endoscopic retrograde biliary drainage. Although pazopanib prolonged her life for 10 months, she died from sepsis at age 49 years, which was caused by the infection that spread to the liver metastatic tumor via the stented biliary ducts. Autopsy revealed adenocarcinoma-like differentiation of the tumor. Conclusion This LGESS patient has survived for a long time owing to multidisciplinary treatment including proton therapy. The LGESS tumor differentiated to NET G2-like tissue and then further to adenocarcinoma-like tissue during the long-term follow-up.

Endometrial stromal sarcoma (ESS) is a rare malignant tumor that constitutes about 0.2% of all uterine malignancies and 10% of uterine sarcomas. ESS is generally misdiagnosed as leiomyoma or endometrial polyp and typically discovered on histopathological examination postoperatively because of its rarity. Endometrial stromal tumors are composed of cells resembling normal endometrial stroma in its proliferative phase. The histologic diagnosis of the high grade is made if there is a high-grade sarcoma with a high mitotic index and nuclear anaplasia. The mean age of presentation of high-grade endometrial sarcoma is about 61 years with the most common presenting complaint is menorrhagia. The median overall survival for high-grade endometrial sarcoma is 53 months with optimal cytoreduction. A 49-year-old woman P2 L2 presented with nonspecific complaint of discharge and spotting per vaginum. In the present case, the provisional diagnosis by clinical findings as well as imaging was in favor of the inversion of submucous fibroid. Preoperative histopathological examination and immunohistochemistry confirmed the diagnosis of high-grade undifferentiated ESS. Haultain's operation followed by total abdominal hysterectomy and bilateral salpingo-oophorectomy was performed. The patient was referred to another center for radiotherapy. From there, she was lost to follow-up. Rarity of endometrial stromal tumor limits the clinician view to diagnose it preoperatively. We were fortunate to have preoperative histopathological diagnosis of ESS. Furthermore, as ESS is rare and undifferentiated stromal sarcoma is even rarer, literature is lacking on its optimal management. Hence, it is important for all clinicians to keep the high degree of suspicion for ESS while working up any case of abnormal uterine bleeding.

Sarcomas of the uterine corpus are rare malignant neoplasms, which are further classified into mesenchymal tumors, and mixed (epithelial plus mesenchymal) tumors. The main issues concerning these neoplasms are the small number of clinical trials, insufficient data from evidencebased medicine, insignificant interest from the pharmaceutical industry, all of which close a vicious circle. The low frequency of these malignancies implies insufficient experience in the diagnosis, hence incomplete surgical and complex treatment. Additionally, the rarity of these sarcomas makes it very difficult to develop clinical practice guidelines. Preoperative diagnosis, neoadjuvant and adjuvant chemoradiation, target and hormone therapies still raise many controversies. Disagreements about the role and type of surgical treatment are also often observed in medical literature. There are still insufficient data about the role of pelvic lymph node dissection and fertility-sparing surgery. Pathologists' experience is of paramount importance for an accurate diagnosis. Additionally, genetics examinations become part of diagnosis in some sarcomas of the uterine corpus. Some gene mutations observed in uterine sarcomas are associated with different outcomes. Therefore, a development of molecular classification of uterine sarcomas should be considered in the future. In this review, we focus on the epidemiology, pathogenesis, pathology, diagnosis and treatment of the following sarcomas of the uterine corpus: leiomyosarcoma, low- and high-grade endometrial stromal sarcomas, undifferentiated sarcoma and adenosarcoma. Uterine carcinosarcomas are excluded as they represent an epithelial tumor rather than a true sarcoma. [ABSTRACT FROM AUTHOR]

Synopsis: The present study reports the first case of high‐grade endometrial stromal sarcoma which occurred as tumor thrombus in the inferior vena cava. It was detected by chance during lymphadenectomy and radically resected. [ABSTRACT FROM AUTHOR]

Surgical treatment of low-grade endometrial stromal sarcoma consists of hysterectomy. The role of oophorectomy is yet to be established. We aimed to examine the effect of preserving the ovaries on the pattern of recurrences in patients with stage I disease. Thirty-four patients with stage I low-grade endometrial stromal sarcoma were retrospectively analysed. Based on ovarian preservation the whole cohort was divided into two groups. Recurrence (liver, lung, groin and bone) was detected in 4 (11.8%) cases. No significant differences in overall survival or disease-free survival (DFS) were observed between the ovarian preservation and bilateral salpingo-oophorectomy (BSO) groups. Subset analysis revealed no significant difference in DFS between the ovarian preservation and BSO groups in the premenopausal arm. And also, the performance of pelvic (n = 2) or para-aortic lymphadenectomy (n = 6) or adjuvant hormonal therapy did not alter DFS significantly. The 5-year DFS rate for the group which received adjuvant radiotherapy was 62.5 and 94.4% for those which did not (p =.014). Preserving the ovaries had no adverse effect on the recurrence of stage I disease. What is already known on this subject? Due to the rarity of the disease and the common postoperative diagnosis, only retrospective studies have been reported on low-grade endometrial stromal sarcoma. This disease is commonly diagnosed in premenopausal patients during the early stage. There is no consensus on preserving the ovaries, particularly in young patients, due to the tumour's hormonal characteristics and the risk of late recurrences. What do the results of this study add? Ovarian preservation had no effect on the recurrence of stage I low-grade endometrial stromal sarcoma. Lymphadenectomy and adjuvant hormonal treatment had no effect on DFS, and adjuvant radiotherapy decreased DFS in the current study. What are the implications of these findings for clinical practice and/or further research? Ovarian preservation should be considered, to prevent the negative effects of surgical menopause, particularly in young patients. [ABSTRACT FROM AUTHOR]

In the context of female genital tract malignancy, uterine sarcoma is considered the rarest form of the disease. Despite the inert nature of low-grade endometrial sarcoma, they must be meticulously diagnosed on time, with an exact grading of the severity and staging of the disease, which further guides the treatment modality and prognosis. A married Asian female without any significant past medical and surgical history complained of abdominal distension and discomfort, which was progressive in nature, for which a radiological assessment was made that showed features suggestive of endometrial sarcoma. Total abdominal hysterectomy with sapingoopherectomy was done without any perioperative complications. Histology further confirmed the diagnosis. Post-operatively, the patient had an unremarkable hospital stay and was discharged home. Endometrial stromal sarcoma is one of the rare malignant entities presenting usually in late adult females, but sometimes it can present at an earlier age as well. Abdominal masses in females, although usually overlooked as benign, can sometimes be associated with a malignant picture. Low-grade endometrial sarcomas have been seen to masquerade other minor benign cases, such as leiomyoma. Despite the rarity of such malignant conditions, diagnosis and management are rather straightforward, and post-operative patient prognosis has been found to be rewarding. Among the uterine sarcoma cases, endometrial sarcoma comes under the malignant disease of the least occurrence. Compared to other malignant conditions, these patients present with minor symptoms like discomfort, which may go unchecked. The major factor that should be noted is the on-time diagnosis and appropriate choice of treatment modality. Overall, despite a minute prevalence and difficult diagnosis, the prognosis of the patient is rather good. • Endometrial stromal sarcoma (ESS) is a rare and challenging condition that usually affects women in their late 40s and 50s. • Low-grade Endometrial stromal sarcoma is an underrated differential diagnosis in the presentation of abdominal mass. • Ultrasound lacks specificity for ESS and can suggest an incorrect diagnosis of more prevalent conditions such as adenomyosis or, in our case — an ovarian cyst. • Total abdominal hysterectomy with bilateral salpingo-oophorectomy is recommended for LG-ESS treatment. • Timely diagnosis and prompt surgical intervention can halt the disease progression, thereby achieving the best prognosis. [ABSTRACT FROM AUTHOR]

Background: 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.Methods: 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.Results: 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.Conclusion: 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. [ABSTRACT FROM AUTHOR]

Background: Long-term follow-up reports of low-grade endometrial stromal sarcoma (LGESS) including its clinical course and pathological data are rare. We previously reported the case of a Japanese woman diagnosed with LGESS, who was treated with multidisciplinary therapy. She had been suffering from uterine cervical tumor diagnosed as cervical polyps, or fibroid in statu nascendi, since 24 years old. The patient had survived for 25 years with the disease. This report presents her progress and pathological change since the previous report.Case Presentation: At age 45, the patient experienced a relapse of the remnant LGESS tumor between the right diaphragm and liver. Although chemotherapy was not effective, the tumor was eliminated by proton therapy. At age 46 years, the remnant tumors outside the irradiated field were resected. The disease was originally diagnosed as "neuroendocrine carcinoma (NEC)" using the surgical specimen. Therefore, cisplatin and irinotecan combination chemotherapy were administered to treat the remnant dissemination. After 4 cycles of chemotherapy, the liver metastases had enlarged and were resected surgically. Consequently, no remnant tumor was visible in the abdominal cavity at the end of the surgery. To determine the origin of NEC, we examined the previously resected specimens obtained from her ileum at age 40 years. A boundary between the LGESS and neuroendocrine tumor grade 2 (NET G2)-like lesion was found in the tumor, indicating that the origin of these tumors was LGESS. After less than 2 years of chemotherapy and undergoing surgery, a relapse of the tumor in the liver induced biliary duct obstruction with jaundice, which was treated with endoscopic retrograde biliary drainage. Although pazopanib prolonged her life for 10 months, she died from sepsis at age 49 years, which was caused by the infection that spread to the liver metastatic tumor via the stented biliary ducts. Autopsy revealed adenocarcinoma-like differentiation of the tumor.Conclusion: This LGESS patient has survived for a long time owing to multidisciplinary treatment including proton therapy. The LGESS tumor differentiated to NET G2-like tissue and then further to adenocarcinoma-like tissue during the long-term follow-up. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Endometrial stromal sarcoma (ESS) is a rare malignant tumor that constitutes about 0.2% of all uterine malignancies and 10% of uterine sarcomas. ESS is generally misdiagnosed as leiomyoma or endometrial polyp and typically discovered on histopathological examination postoperatively because of its rarity. Endometrial stromal tumors are composed of cells resembling normal endometrial stroma in its proliferative phase. The histologic diagnosis of the high grade is made if there is a high-grade sarcoma with a high mitotic index and nuclear anaplasia. The mean age of presentation of high-grade endometrial sarcoma is about 61 years with the most common presenting complaint is menorrhagia. The median overall survival for high-grade endometrial sarcoma is 53 months with optimal cytoreduction. A 49-year-old woman P2 L2 presented with nonspecific complaint of discharge and spotting per vaginum. In the present case, the provisional diagnosis by clinical findings as well as imaging was in favor of the inversion of submucous fibroid. Preoperative histopathological examination and immunohistochemistry confirmed the diagnosis of high-grade undifferentiated ESS. Haultain's operation followed by total abdominal hysterectomy and bilateral salpingo-oophorectomy was performed. The patient was referred to another center for radiotherapy. From there, she was lost to follow-up. Rarity of endometrial stromal tumor limits the clinician view to diagnose it preoperatively. We were fortunate to have preoperative histopathological diagnosis of ESS. Furthermore, as ESS is rare and undifferentiated stromal sarcoma is even rarer, literature is lacking on its optimal management. Hence, it is important for all clinicians to keep the high degree of suspicion for ESS while working up any case of abnormal uterine bleeding. [ABSTRACT FROM AUTHOR]

Purpose: 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.Methods: 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.Results: 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.Conclusion: 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. [ABSTRACT FROM AUTHOR]

Pulmonary metastases of endometrial stromal sarcoma (ESS) are uncommon and can be difficult to diagnose. The aims of the present study were to investigate the clinical and pathological features, and enhance the awareness of pulmonary metastases in patients with low-grade ESS. The study reports a case of low-grade ESS that resulted in cystic and nodular pulmonary metastases. Furthermore, the PubMed database was searched using 'pulmonary metastases of low-grade endometrial stromal sarcoma' as the key phrase. The literature on pulmonary metastases of low-grade ESS was reviewed and 35 cases were included in the present study. The clinical manifestations, imaging data, pathological features, treatment and prognosis of the 35 previously reported cases and the current case were retrospectively analyzed. The age range of the 36 patients diagnosed with low-grade ESS was 28–65 years. The time period from confirmation of ESS to lung metastases was 1.5–27 years. In 50% of the patients, the pulmonary metastases were asymptomatic. The most common pulmonary symptom was dyspnea, followed by chest pain, pneumothorax and coughing. The most common chest imaging presentation was multiple pulmonary nodules, followed by a solitary nodule or mass. Histology was used to identify that the pulmonary metastases had the pathological features of low-grade ESS. The immunohistochemical results demonstrated strong diffuse immunoreactivity for cluster of differentiation 10, estrogen receptor and progesterone receptor in almost all the specimens. The review of the literature revealed that pulmonary metastases from low-grade ESS are rare but not negligible. Furthermore, the detailed clinical information, imaging findings and immunohistochemical detection are important for making a diagnosis. [ABSTRACT FROM AUTHOR]

Objective: To compare aromatase inhibitors (AIs) with progestins as adjuvant hormonal therapy(AHT) for low-grade endometrial stromal sarcomas (LGESSs). Methods: We reviewed cases with LGESS at our institution from 1984 to 2017. Disease recurrence and recurrence-free survival (RFS) were assessed among patients who received AI, progestins, or no AHT. Results: Among 39 patients with LGESS, 18 received progestins, 13 received AI, and 8 received no AHT. Thirty patients had stage I disease, and 9 had stage II to IV disease. All underwent hysterectomies. Disease recurred in 70% (7/10) of stage I patients who received no AHT, compared to 14.3% (1/7) receiving AI, and 7.7% (1/13) receiving progestins (P ¼ .003). Among stage I patients taking AI, mean RFS was 153.1 months (95% confidence interval [CI]: 110-195.6) versus 306.2 months (95% CI: 259.7-352.6) for progestin patients and 90.8 months (95% CI: 56.8-124.9) for those who received no AHT. In stage II to IV patients, mean RFS was 148.5 months (95%CI: 148.5-148.5) and 120.8months (95%CI: 55.8-185.9) for the AI and progestin groups, respectively. All stage II to IV patients received AHT. Among stage I patients, median follow-up time for RFS was 159.1 months for progestin patients, 52.6 months for AI, and 53.1 months for those who received no AHT. Of this, 69% of stage I patients taking progestins reduced/stopped treatment prematurely due to side effects. None of the patients taking AI discontinued treatment early. Conclusion: Aromatase inhibitor is associated with longer RFS in patients with advanced LGESS, is better tolerated than progestins, and can be primary AHT for LGESS. [ABSTRACT FROM AUTHOR]

A case of Low-grade endometrial stromal sarcoma (LG-ESS) invading the great vessels is rare. A 34-year-old female who had no past history presented to a previous hospital with abdominal distension. Magnetic resonance imaging revealed a 15 cm pelvic mass beside the uterus, and only the pelvic mass was removed at the surgery. The tumor was judged to be a LG-ESS. The patient chose to be observed to preserve her fertility, and no adjuvant treatment was undertaken. Two years later, she was referred to our hospital due to recurrence of the pelvic mass. Enhanced computed tomography revealed a large tumor in the vena cava which extended from the left internal iliac vein and which originated from the pelvic tumor. An operation was performed by a multidisciplinary team. Complete resection of the tumor was achieved with a radical hysterectomy, bilateral salpingo-oophorectomy, removal of recurrent pelvic masses and the intravascular tumor. We diagnosed a recurrence of LG-ESS. She received a postoperative adjuvant therapy of LG-ESS. Patients with fertility-sparing treatment had higher recurrence rates. In cases of tumor intravenous extension, we should make every effort to extract the tumor to avoid sudden death. This case highlights the importance of a multidisciplinary approach in treating this rare tumor with intravascular extension. In particular, patients with LG-ESS who receive fertility-sparing surgery should undertake postoperative chemotherapy or radiotherapy in order to reduce the risk of recurrence, as was in this case. • Low-grade endometrial stromal sarcoma invading the great vessels is extremely rare. • A multidisciplinary approach in treating with intravascular extension is important. • LG-ESS extended into vessels after fertility-sparing surgery were few reports. [ABSTRACT FROM AUTHOR]

Low-grade endometrial stromal sarcoma (LG-ESS) usually exhibits indolent behavior, often misdiagnosed and is associated with a favorable prognosis [1]. Despite the indolent nature of LG-ESS, recurrence is common, the risk is as high as 1 in 3 to 1 in 2 [2]. The behavior of such tumors can lead to invade blood vessels [3]. An intravascular tumor is associated with direct risk of sudden death from pulmonary embolism. There are very few reported cases with clinically obvious intravascular extension. Chemotherapy, radiotherapy and hormone therapy are adjuvant treatments that have not been able to demonstrate a definitive benefit in survival [4]. We want to take advantage of the opportunity, so far unpublished, to demonstrate with this case the surgical challenge described step by step, as well as to demonstrate the importance of surgery as the only curative treatment even in extended intravascular disease [5]. Here in, we present the surgical challenge of a LG-ESS in a 59-year old woman with iliac and cava vein tumoral thrombus extension. We performed a tumoral thrombectomy and vascular repair, with a pelvic exenteration and double barrel wet colostomy. After performing pelvic exenteration, the thrombus with intravascular involvement was addressed at 2 points: inferior cava vein and right common iliac vein with the extraction of the entire thrombus. These defects are corrected by angioplasty and venorrhaphy, respectively. The surgery got a complete cytoreduction. It lasted 290 minutes, with 430 mL blood loss. In patients with LGESS, cytoreduction is a reliable option even in extended metastatic disease. [ABSTRACT FROM AUTHOR]

Abstract Low-grade endometrial stromal sarcoma (LGESS) is the second most common malignant mesenchymal tumor of the uterus. The most common location is the uterine corpus, but it can also primarily arise in a variety of extrauterine locations such as pelvis, ovary, abdominal cavity, vagina, and vulva. We are reporting a case of a 47-year-old female with no significant medical history who presented with multiple pulmonary nodules. Fine needle aspiration (FNA) specimen revealed spindle cell neoplasm consistent with the diagnosis of LGESS. The differential diagnosis included neuroendocrine tumor, synovial sarcoma, solitary fibrous tumor, smooth muscle tumors, and peripheral nerve sheath tumors. The clinical, cytological, and histopathologic details of this case, as well as a discussion of the potential pitfalls and differential diagnosis of spindle cell lesions of the lung are described. Highlights • A case of low-grade endometrial stromal sarcoma presented as multiple pulmonary nodules is reported with literature review. • Differential diagnosis of spindle cell lesions in the lung are discussed. • Cytological-pathological correlation is addressed. [ABSTRACT FROM AUTHOR]

Low-grade endometrial stromal sarcoma (LGESS) is a rare malignancy. The tumor is reportedly responsive to hormonal therapy, most commonly with medroxyprogesterone acetate (MPA), but the effectiveness of aromatase inhibitors for recurrent LGESS remains unclear. The present study reports a case of stage IC LGESS presenting with abnormal uterine bleeding, and also provides a review of the literature. Following a total abdominal hysterectomy and bilateral salpingo-oophorectomy, MPA therapy was initiated; treatment was successful, but discontinued 19 months later due to disruptive side effects. A further 2 months later, the patient presented with recurrent disease and received chemotherapy. MPA treatment was restarted with a partial response. A second recurrence, 4 years later, presented with lung and para-aortic lymph node metastases. The patient responded to treatment with the aromatase inhibitor letrozole. The patient has since exhibited stable disease and remained free of symptoms for 7 years. This case suggests that aromatase-inhibitor treatment may be effective for recurrent LGESS as a second-line treatment. [ABSTRACT FROM AUTHOR]

Pulmonary tumor thromboembolus ( PTTE) secondary to uterine cancer is a rare cause of sudden unexpected deaths in the elderly population. Additionally, tumor extension to the inferior vena cava ( IVC) makes it rarer. No such cases have been previously reported in the forensic literature. We report this phenomenon in a previously healthy 70-year-old woman with no other known risk factors for the development of deep vein thrombosis. The deceased was found dead while watching television with her relatives. Autopsy examination revealed that her death was attributed to a saddle embolus lodged in the main pulmonary arteries. A uterine tumor was discovered, and microscopic examination revealed low-grade endometrial stromal sarcoma with angiolymphatic invasion. The tumor cells were also found in the embolus and the remnant thrombosis attached to the inner wall of the IVC. This case demonstrates a sudden unexpected death caused by acute PTTE following seeding of uterine cancer into the IVC and raises awareness of such events. [ABSTRACT FROM AUTHOR]

Objective: Endometrial stromal sarcoma is a rare cancer that accounts for 0.2% or less of all female genital tract malignancies. We present a case of extrauterine low-grade endometrial stromal sarcoma arising from endometriosis, which was managed by unilateral salpingo-oophorectomy with postoperative high-dose progesterone adjuvant therapy. Case Report: A 28-year-old nulligravid woman had suffered from progressive abdominal distension accompanied by a palpable firm mass for about 3 months. An abdominal pelvic mass, measuring 13 × 12 × 8 cm, was seen on pelvic sonography and abdominal computed tomography. The CA125 titer was also elevated. Left salpingo-oophorectomy was performed when frozen section examination of the tumor indicated a benign tumor. However, the pathology of the tumor was extrauterine low-grade endometrial stromal sarcoma with extensive endometrioid glandular differentiation arising from endometriosis. The resection margin was also involved. The patient has been receiving high-dose progesterone therapy for 2 months without any adverse effects, except for an increase in body weight of 2 kg. Conclusions: Low-grade endometrial stromal sarcoma typically has an indolent clinical course and favorable prognosis. Surgical resection is the primary therapeutic approach, and adjuvant therapy with radiotherapy, chemotherapy, or progesterone therapy should be considered for the management of residual or recurrent low-grade endometrial stromal sarcomas.

Background: To assess the efficacy of different treatment modalities on the outcome of patients with low-grade endometrial stromal sarcoma (LG-ESS).Methods: Patients with LG-ESS who received hysterectomy from March 1991 to December 2013 were retrospectively analyzed. The associations between clinicopathologic variables and disease free survival (DFS) were evaluated.Results: One hundred and fourteen patients met the eligibility requirements. All patients received hysterectomy as the main treatment, 17.5% (20/114) of patients received ovarian preservation, and 62.3% (71/114) of patients received lymphadenectomy. Fifty-six patients received chemotherapy, 36 patients received radiotherapy, and 11 patients received endocrine therapy. The median follow-up duration was 40 months. The 5-year and 10-year DFS rates were 91.8% and 77.4%, respectively. The 5-year and 10-year overall survival rates were 96.7% and 96.7%, respectively. Univariate analyses showed that there were no risk factors associated with DFS. Lymphadenectomy, lymph node status, ovarian preservation, chemotherapy, radiotherapy, and endocrine therapy had no significant effect on DFS.Conclusions: Hysterectomy has been the mainstay of treatment for LG-ESS. The optimal treatment strategy in LG-ESS remains to be determined. [ABSTRACT FROM AUTHOR]

Introduction: Low-grade endometrial stromal sarcoma is very rare and difficult to diagnose in the early stage. A standard treatment has not been established. In this case report of a patient with long-term survival, we describe an effective treatment for advanced low-grade endometrial stromal sarcoma.Case Presentation: A 24-year-old Japanese woman who presented with prolonged menstruation was diagnosed with leiomyoma on the basis of a specimen resected transvaginally. She underwent ten resections in 10 years without a malignancy diagnosis. During this period, she gave birth. At age 34 years, she visited our hospital, complaining of lower abdominal pain. A 10cm tumor was detected behind her uterus. The disease was diagnosed as an advanced malignant ovarian tumor before surgery. A laparotomy was performed, with many remnants left in the abdominal cavity. The final diagnosis was advanced low-grade endometrial stromal sarcoma. After 12 cycles of gemcitabine and docetaxel combination chemotherapy, the tumor disappeared completely. A retrospective pathological review of the specimens resected transvaginally showed that the tumors included low-grade endometrial stromal sarcoma elements. When the patient was age 42 years, the sarcoma recurred. It was detected around the right diaphragm and liver. Despite administration of gemcitabine and docetaxel, ascites and pleural effusion accumulated. Administration of medroxyprogesterone acetate, leuprorelin acetate, and anastrozole gradually reduced the ascites and pleural effusion. In addition to the three hormone drugs, 18 cycles of paclitaxel and carboplatin were administered. The patient recovered from her critically ill state and is currently alive with reduced tumor at age 45 years.Conclusions: Our patient with low-grade endometrial stromal sarcoma whose disease began in her youth gave birth and experienced long-term survival with surgery, chemotherapy, and hormone therapy. [ABSTRACT FROM AUTHOR]

Despite excellent prognosis, low-grade endometrial stromal sarcoma (ESS) is notorious for late recurrence even in stage I disease. Bone metastases are distinctly rare and only six cases have so far been reported. Two patients presented with back pain due to spinal metastatic low-grade ESS after 15 and 9 years free of disease after resection of the stage 1 primary uterine tumor. Plain radiograph showed an ivory first lumbar vertebra in the first patient and an osteolytic lesion involving the second thoracic vertebra in the second. In both cases, magnetic resonance imaging showed vertebral tumor with intra-spinal extension and spinal cord compression; biopsy confirmed the diagnosis of metastatic low-grade ESS. The first patient received palliative radiotherapy and chemotherapy while the second underwent surgical decompression followed by adjuvant radiotherapy and chemotherapy. The neurologic symptoms in both patients returned 12 months afterwards and progressed relentlessly despite adjuvant chemotherapy and radiotherapy. The first patient developed multiple bone and lung metastasis, culminating in death 44 months after recurrence. While surviving at 24 months from recurrent disease, the second became paraplegic with double incontinence and was wheelchair bound. Delayed osseous metastatic low-grade ESS, although uncommon, deserves recognition given the predilection for spine as the site of involvement. In such a location, where complete surgical removal is notoriously difficult, if not impossible, the prognosis of spinal metastatic low-grade ESS appears grave with considerable morbidity and mortality. [ABSTRACT FROM AUTHOR]

Endometrial stromal tumors (ESTs) with limited infiltration were first proposed by Dionigi et al.1 However, the prognostic significance of these tumors is unclear. We report a case of a 60-year-old woman who presented with a prolapsed uterine corpus and then underwent laparoscopic-assisted vaginal hysterectomy. A very small EST was incidentally found. The tumor manifested focal irregularity and finger-like permeation into the adjacent myometrium not exceeding 3 mm but exceeding 3 in number, features intermediate between a low-grade endometrial stromal sarcoma and an endometrial stromal nodule. By definition, we rendered a descriptive diagnosis of “endometrial stromal tumor with limited infiltration.” A subsequent staging operation confirmed metastasis and, hence, a malignant potential. [ABSTRACT FROM AUTHOR]

Low-grade endometrial stromal sarcomas are relatively rare tumors. We here report a case of a woman presenting with a giant metastatic low-grade endometrial stromal sarcoma with thromboembolic complications requiring urgent surgical management. A 58-year-old obese female was admitted, with a voluminous abdominopelvic mass, due to complications related to its size and extent. The tumor derived from the uterus and invaded the ureters, bladder and rectum. It compressed the right iliac vessels causing both deep vein thrombosis and pulmonary embolism. She developed a painful irreducible umbilical. We proceeded with a debulking surgery (hysterectomy with bilateral salpingo-oophorectomy). Histological findings were consistent with a low-grade endometrial stromal sarcoma. Low-grade endometrial stromal sarcomas are generally low-grade malignant neoplasms with an indolent clinical course. Surgery is the cornerstone of treatment. In low-income countries, malignancies are more often diagnosed at a late stage, which limits therapeutic options. Cytoreduction is recommended in advanced tumors with extrauterine manifestation, depending on symptoms and with palliative intent. Low-grade endometrial stromal sarcomas are indolent uterine malignancies with metastatic potential. Even in advanced cases, cytoreduction must be considered. • Endometrial stromal sarcomas (ESS) are relatively rare tumors. • They are classified into three categories: low-grade, high-grade, and undifferentiated. • Staging is the most important prognostic factor. • Cytoreduction surgery is recommended even in advanced tumors. • Late presentation of cancers is commonplace in most low-income countries. [ABSTRACT FROM AUTHOR]