Low-grade Endometrial Stromal Sarcoma Research Articles

A Rare Collision of Endometrioid Adenocarcinoma and Second Recurrent Low-Grade Endometrial Stromal Sarcoma 8 Years After Hysterectomy: A Case Report and Literature Review

Low grade endometrial stromal sarcoma (LG-ESS) is a rare malignancy of mesenchymal origin in the female reproductive system, which has the characteristic of late recurrence. Endometrial carcinoma is a group of epithelial tumors, and is one of the most common gynecological malignancies among women worldwide. The coexistence of LG-ESS and endometrial carcinoma is extremely rare, and there is no relevant report about the collision of endometrial carcinoma and recurrent LG-ESS currently. In the present study, we report a tumor of recurrent LG-ESS accompanied with endometrioid adenocarcinoma in a young patient 8 years after hysterectomy. She was first diagnosed with LG-ESS at the age of 19 and received fertility-sparing therapy. During the 13-year follow-up period, she successfully delivered and experienced two recurrences of LG-ESS, and an unexpected endometrioid adenocarcinoma component was found in the second recurrent tumor. Adjuvant chemotherapy and endocrine therapy were administrated to the patient after completely removing the tumor, and no sign of disease recurrence or metastasis were found 7 months after the last surgery. This study emphasizes the importance of long-term management of LG-ESS and brings new insights to clinicians and pathologists about collision tumors involving recurrent tumor.

UTERINE SAROMAS Retrospective Studies on 7 Cases at the Hassan II University Hospital in Fez

Introduction: Uterine sarcomas are rare tumors, representing less than 3% of malignant tumors of the female genital tract and between 3 and 7% of malignant tumors of the uterine body. They are a heterogeneous group of tumors comprising different histological subtypes, but with a generally identical evolutionary profile. Unlike uterine epithelial tumors, and apart from low-grade endometrial stromal sarcomas, uterine sarcomas have a poor prognosis. They have a high rate of recurrence (between 50 and 70%), local and especially metastatic (in approximately 70% of cases). The objective of our work is to specify the epidemiological characteristics of uterine sarcomas, the diagnostic difficulties faced by practitioners at the clinical, radiological and anatomopathological stages, the modalities of management as well as the different determinants of the prognosis of these rare tumors. Patients And Methods: Seven cases of uterine sarcomas treated in the gynecology - obstetrics II department at the Hassan II University Hospital in Fez between 2019 and 2023 were analyzed retrospectively. Results: The average age of our patients varies between 50 and 75 years with an average of 62.2 years. Of the 7 patients, 90% are multiparous, 100% menopausal. 78% of our patients consulted for metrorrhagia. On the paraclinical level, we performed a pelvic ultrasound for all patients, 80% of the results of which were in favor of an enlarged uterus with a heterogeneous echogenic intracavitary image, hysteroscopy in 04 patients for exploration of postmenopausal metrorrhagia and magnetic resonance imaging in 06 patients. The diagnosis was made at the preoperative stage in five patients. 71% of our patients benefited from surgical treatment. The anatomopathological analysis of the surgical specimens showed that 05, 07 patients benefited from adjuvant radiotherapy, while a combined chemotherapy/radiotherapy postoperatively was indicated in 02 patients. One patient presented a recurrence having ...

Clinicopathologic Profile and Survival of Patients with Uterine Low Grade Endometrial Stromal Sarcoma

Clinicopathologic Profile and Survival of Patients with Uterine Low Grade Endometrial Stromal Sarcoma

Hormonal therapy in low-grade endometrial stromal sarcoma: What is the current standard of care?

Hormonal therapy in low-grade endometrial stromal sarcoma: What is the current standard of care?

Low Grade Extrauterine Endometrial Stromal Sarcoma Arising from Peritoneum Endometriosis: A Case Report and Literature Review

Introduction: Endometrial stromal sarcoma (ESS) is an uncommon condition that accounts for approximately 0.2% of all reproductive tract malignancies. Among the various subtypes of ESS, low-grade extrauterine endometrial stromal sarcoma (LG-EESS) is even rarer and has only been reported in a limited number of case studies. Case Presentation: We reported a case of LG-EESS in a 35-year-old woman with a five-year history of stage IV endometriosis. The patient presented with chronic abdominopelvic pain and dysmenorrhea. Transvaginal ultrasound showed bilateral tubo-ovarian complexes with multicystic structures, while MRI revealed solid lesions with T1 iso signal, T2 hypersignal, and diffusion restriction, indicating metastatic implantation extending to the peritoneum and subhepatic region. A core needle biopsy confirmed LG-EESS, and immunohistochemistry showed CD10 positivity, aiding in the diagnosis and distinguishing it from other tumors. Conclusions: Our case highlights the potential utility of preoperative diagnostic imaging, particularly MRI, in the evaluation of LG-EESS. Additionally, it emphasizes the importance of considering the possibility of extrauterine ESS, as this tumor exhibits histopathological features and clinical behavior similar to its uterine counterpart.

Survival Trends for Uterine Sarcomas from a Tertiary Center: The Oxford Experience.

Uterine sarcomas are rare and aggressive gynecological malignancies. We evaluated the epidemiology, treatment outcomes and survival rates in uterine sarcoma patients managed in our institute. The medical records of women with histology proven uterine sarcoma, including leiomyosarcoma (LMS), adenosarcoma (ADS), and endometrial stromal sarcoma (ESS), treated at our institution from February 2010 to February 2022, were analyzed. In total, 41 patients were identified. In detail, LMS, ADS, and high-grade and low-grade ESS were identified, respectively, in 60.9%, 19.5%, 12.1%, and 7.3% of the cases. The majority of women affected by LMS (72%) underwent primary surgery and 40% of them also received adjuvant chemotherapy. A surgical approach was the preferred mode of treatment in 83% of the recurrences. The median OS (overall survival), DFS (disease free survival), and PFS (progression free survival) for the LMS group were 25, 44.5, and 8 months, respectively. The 5-year survival rates for LMS, ADS, and ESS groups were 30.5%, 100% and 37.5%, respectively. The 5-year survival for LMS was found to be significantly worse than for other histology types (p = 0.016). Our study provides valuable data for the evaluation of treatment strategies and survival trends among these rare cancers. The management and follow-up planning of each subtype requires a thorough patient-focused multidisciplinary discussion.

Immunohistochemical Expression of Lymphoid Enhancer-binding Factor 1 in Low-grade Endometrial Stromal Tumors.

Endometrial stromal tumors (ESTs) are uncommon uterine mesenchymal lesions. Nuclear expression of β-catenin, an indication of activated Wnt/β-catenin signaling pathway, was described in 50% to 92% of low-grade ESTs, including endometrial stromal nodule and low-grade endometrial stromal sarcoma. Activation of the Wnt/β-catenin signaling pathway leads to the translocation of β-catenin into the nucleus and interaction with the T-cell factor/lymphoid enhancer-binding factor-1 (LEF1) family of transcription factors to regulate cell proliferation, differentiation, migration, and survival. Immunohistochemical analysis of β-catenin and LEF1 was performed in 2 endometrial stromal nodules and 20 low-grade endometrial stromal sarcomas and demonstrated 90.9% and 81.8% positive rates for β-catenin and LEF1, respectively. The sensitivity, specificity, positive predictive value, and negative predictive value of β-catenin and LEF1 were 90.9% versus 81.8%, 81.0% versus 85.7%, 83.3% versus 85.7%, 89.5% versus 81.8%, respectively, in the diagnosis of low-grade ESTs. There is no statistical significance of the performance of β-catenin and LEF1 in all ESTs ( P = 0.664) or in primary or metastatic/recurrent settings ( P = 0.515 and 0.999, respectively). Only 3 smooth muscle tumors showed focal and weak positivity for LEF1. Our results indicate LEF1 can be a useful marker in aiding a diagnosis of low-grade EST and differentiating from smooth muscle tumors alone or in combination with β-catenin.

Letter to the Editor for the article 'Prognosis of uterine and extrauterine low-grade endometrial stromal sarcoma: an observational cohort study'.

Letter to the Editor for the article 'Prognosis of uterine and extrauterine low-grade endometrial stromal sarcoma: an observational cohort study'.

Cystic endometrial stromal sarcoma mimicking a cystic ovarian tumor : an unusual presentation

Low grade endometrial stromal sarcoma (LGESS) typically presents as a solid intracavitary or intramural uterine mass. On imaging, LGESS is usually seen as an endometrial or myometrial solid mass. Cystic change is unusal and may lead to a differential diagnosis of an ovarian mass as was seen in the present case. Here we present a case of a 38 year old woman who presented with a cystic ovarian mass clinically and radiologically. Per-operatively, it was found to be a multiloculated cystic mass, filled with serosanguinous fluid, in the uterine fundus. Histopathological examination showed a tumor mass composed of mainly spindle cells arranged in fascicular pattern showing marked myxoid degeneration and cystic areas. On immunohistochemistry(IHC), tumor cells showed positivity for CD 10, ER, PR, SMA and desmin while HMB45 was negative. Based on histopathological and IHC, a diagnosis of LGESS was made. Most cases of cystic uterine masses have a benign course but, LGESS exhibits a relatively poorer outcome and a risk of metastasis. Hence, we present this case for its unusual presentation which mimics an ovarian mass but has worse prognosis.

A CARE-compliant article: A case report of retroperitoneal endometrial stromal sarcoma with multiple pulmonary metastases and literature review.

Endometrial stromal sarcoma is an extremely rare mesenchymal neoplasm occurring in the extrauterine. Retroperitoneal endometrial stromal sarcoma with multiple pulmonary metastases, in particular, is extremely rare. Forty-seven-year-old woman (gravida 3, para 2), was referred to our institution with complaints of fever. Ultrasound and computed tomographic imaging of the abdomen identified the presence of masses in the pelvic region. Additionally, computed tomographic scans and X-ray evaluations of the thorax detected dispersed masses in both the lungs and pelvic area. Histopathological analysis of the pelvic region indicated the presence of low-grade endometrial stromal sarcoma. A computed tomography-guided pneumocentesis was conducted to further characterize the pulmonary lesions, confirming the diagnosis of low-grade endometrial stromal sarcoma. The patient underwent tumor resection, subsequent treatment with Medroxyprogesterone acetate for 6 months, received microwave ablation for multiple lung metastases, PARP1 inhibitor for 4 courses, and has been undergoing chemotherapy (epirubicin/ifosfamide) up to the present time. Partial remission was obtained after the above treatment and this patient is now still alive maintaining for 3 years. The identification and management of this disease remain a significant challenge due to its low prevalence. Further research involving a larger number of cases is necessary to ensure consistency in diagnosis and to establish effective treatment guidelines.

Low-grade endometrial stromal sarcoma with extensive sex cord and smooth muscle differentiation: report of a case

Low-grade endometrial stromal sarcoma with extensive sex cord and smooth muscle differentiation: report of a case

Prognostic Factors in Uterine Sarcoma Based on the Tumor Size Stratification: A Retrospective Study.

Uterine sarcoma is a rare malignant gynecological tumor with a poor prognosis. Many studies have identified the clinical stage as an important prognostic factor; however, the heterogeneity of patient distribution in the International Federation of Gynecology and Obstetrics (FIGO) stage has reportedly required further revision. Therefore, this study retrospectively investigated the factors related to the prognosis of uterine sarcoma, with particular attention to tumor size, which can be evaluated preoperatively. Clinical data were extracted from the medical records of patients with uterine sarcoma treated between January 2010 and January 2023. Kaplan-Meier survival curves were plotted according to clinical factors such as histological type, clinical stage, chemotherapy, and tumor size. Factors that were significant in the univariate analysis were subjected to the multivariate analysis using Cox proportional hazards regression. Thirty-four patients with uterine sarcoma, comprising 24 (70.5%), five (14.7%), three (8.8%), and two (5.9%) with leiomyosarcoma, undifferentiated sarcoma, high-grade endometrial stromal sarcoma, and low-grade endometrial stromal sarcoma, respectively, were included. Based on the FIGO stage, 15 (44.1%), six (17.6%), three (8.8%), and 10 patients had stage I, II, III, and IV disease, respectively, at the time of diagnosis. All patients underwent surgery as initial treatment; 15 received postoperative chemotherapy. Among the 32 patients with uterine leiomyosarcoma, undifferentiated sarcoma, or high-grade endometrial stromal sarcoma, overall survival differed significantly in the univariate analysis based on disease stage (I + II vs. III + IV) and tumor size (≤10 vs. >10 cm). However, only tumor size was an independent prognostic factor in the multivariate analysis. Tumor size (≤10 vs. >10 cm) may possibly have a prognostic impact on uterine sarcoma.

Integrated Transcriptomic Landscape and Deep Learning Based Survival Prediction in Uterine Sarcomas.

The genomic characteristics of uterine sarcomas have not been fully elucidated. This study aimed to explore the genomic landscape of the USs. Comprehensive genomic analysis through RNA-sequencing was conducted. Gene fusion, differentially expressed genes (DEGs), signaling pathway enrichment, immune cell infiltration, and prognosis were analyzed. A deep learning model was constructed to predict the survival of US patients. A total of 71 US samples were examined, including 47 endometrial stromal sarcomas (ESS), 18 uterine leiomyosarcomas (uLMS), 3 adenosarcomas, 2 carcinosarcomas, and 1 uterine tumor resembling an ovarian sex-cord tumor (UTROSCT). ESS (including high-grade ESS and low-grade ESS) and uLMS showed distinct gene fusion signatures; a novel gene fusion site, MRPS18A - PDC-AS1 could be a potential diagnostic marker for the pathology differential diagnosis of uLMS and ESS; 797 and 477 uDEGs were identified in the ESS vs. uLMS and HGESS vs. LGESS groups, respectively. The uDEGs were enriched in multiple pathways. Fifteen genes including LAMB4 were confirmed with prognostic value in USs; immune infiltration analysis revealed the prognositic value of myeloid dendritic cells, plasmacytoid dendritic cells, natural killer cells, macrophage M1, monocytes and hematopoietic stem cells in USs; the deep learning model named MMN-MIL showed satisfactory performance in predicting the survival of US patients, with the area under the receiver operating curve curve reached 0.909 and accuracy achieved 0.804. USs harbored distinct gene fusion characteristics and gene expression features between HGESS, LGESS, and uLMS. The MMN-MIL model could effectively predict the survival of US patients.

Diagnostic Value of Combined BCOR, Cyclin D1, and CD10 in Differentiating Endometrial Stromal Sarcoma From Other Uterine Spindle Cell Lesions.

Uterine spindle cell lesions share a dilemmatic overlapped features that needed to be addressed by the pathologist to reach a conclusive accurate diagnosis for its prognostic value and different management decisions. Usage of combined IHC panel can be an aiding guiding tool in this context. The aim of this study is to evaluate the diagnostic value of combined BCOR, Cyclin D1, and CD10 IHC panel in differentiating endometrial stromal sarcoma from other uterine spindle cell lesions. This study included 60 cases categorized into endometrial stromal sarcoma group (ESS) (12 cases high-grade endometrial stromal sarcoma [HGESS] and 18 cases low-grade endometrial stromal sarcoma [LGESS]), malignant uterine spindle cell lesions group (5 cases adenosarcoma [AS], 6 cases leiomyosarcoma [LS], 4 cases carcinosarcoma [CS]), and benign uterine lesions group (5 cases endometrial stromal nodule [ESN], 5 cases leiomyoma, and 5 cases adenomyosis). IHC staining procedure and evaluation for BCOR, Cyclin D1, and CD10 was performed on all studied cases. BCOR IHC staining was positive in all HGESS (12/12) of ESS group cases, with diffuse pattern in 75% of cases. BCOR-diffuse staining pattern was not recorded in any of LGESS (0/18), malignant mesenchymal lesions group (0/15), and also benign lesions group (0/15). Cyclin D1 positivity was observed only in HGESS cases, in parallel with positive-BCOR expression. On the contrary, CD10 was negatively expressed in all HGESS and positive in all LGESS, ESN, and adenomyosis cases. A specificity of 100% and sensitivity of 75% were recorded in differentiating HGESS from malignant mesenchymal lesions (including LMS, AS, and CS) and also HGESS from LGESS when using the combined panel BCOR +ve D /Cyclin D1 +ve / CD10 -ve , considering only the BCOR-diffuse staining pattern. In conclusion, BCOR +ve D /Cyclin D1 +ve /CD10 -ve as a combined panel is 100% specific and with lesser sensitivity in diagnosing HGESS as well as differentiating it from LGESS and other malignant uterine spindle cell lesions.

Exploring the Clinicopathological Diversity in Sarcomatous Transformations in the Uterus.

Introduction Uterine sarcomas are a rare group of mesenchymal tumors.They originate either from the uterine smooth muscle or from the endometrial stroma. The spectrum of malignant uterine sarcomatous transformations includes leiomyosarcoma, low-grade endometrial stromal sarcoma (LG-ESS), high-grade endometrial stromal sarcoma (HG-ESS), undifferentiated uterine sarcoma, and carcinosarcoma. The purpose of this study is to determine the distribution of malignant uterine sarcomatous transformations, in relation to the patient's age, epidemiological aspects, and clinical features, and to analyze the different histological types of uterine sarcomas diagnosed at our institutionand thereby to quantify their frequency and determine the most common histopathological subtype occurring in the population. Materials and methods This was a retrospective descriptive study of 21 cases of malignant uterine sarcomatous transformations, diagnosed in Saveetha Medical College, Chennai, between January 2019 and December 2022. Data was collected from the archives of the department of pathologyand from the department of medical records in the hospital. The spectrum of uterine sarcomas was analyzed in relation to the patient's age, menopausal status, presenting symptoms, preoperative diagnosis, biopsy and frozen section reports wherever available, and histopathological reports with pathological staging. Results The most common age group in which uterine sarcoma was diagnosed was found to be 61-70 years. All 10 patients who were diagnosed with carcinosarcoma were aged 60 years, andall seven patients diagnosed with low-grade endometrial stromal sarcoma were less than 50 years of age. Most of the patients were postmenopausal females, except for one patient who was premenopausal. The most common histological variety found was carcinosarcoma, malignant mixed mullerian tumor (47.6%), followed by LG-ESS (33.3%), leiomyosarcoma (14.28%), and HG-ESS (4.7%). Conclusion Uterine sarcomas are anaggressive group of tumors having a very poor prognosis. Due to its rarity and histopathological diversity, there is a lack of consensus on the risk factors.

Current Treatment Options: Uterine Sarcoma.

The cornerstone of treatment for uterine sarcoma, regardless of histologic type, remains en bloc surgical resection with total hysterectomy. In the case of incidental diagnosis during another procedure, such as myomectomy, where a hysterectomy was not performed initially, completion hysterectomy or cervical remnant removal is recommended. The completion of additional surgical procedures, including bilateral salpingo-oophorectomy and lymphadenectomy, remains nuanced. Bilateral salpingo-oophorectomy remains controversial in the setting of most subtypes of uterine sarcoma, except in the case of hormone-receptor positivity, such as in low grade endometrial stromal sarcoma, where it is indicated as part of definitive surgical treatment. In the absence of apparent nodal involvement, we do not recommend performing universal lymphadenectomy for patients with sarcoma. We recommend systemic therapy for patients with extra-uterine or advanced stage disease, high-grade histology, and recurrence. The most active chemotherapy regimens for advanced, high-grade disease remain doxorubicin or gemcitabine and docetaxol combination therapy. A notable exception is low grade endometrial stromal sarcoma, where we recommend anti-hormonal therapy in the front-line setting. Radiation therapy is reserved for selected cases where it can aid in palliating symptoms.

Mullerian adenosarcoma of the uterus with MASO: a case report of cervical adenosarcoma in a young female that never had sexual behavior

BackgroundCervical mullerian adenosarcoma is a rare uterine sarcoma, especially in young women. Its pathological features are low-grade malignant tumors with bidirectional differentiation, and the degree of malignancy is similar to that of low-grade endometrial stromal sarcoma. This paper reports the case of a young asexual patient who has been closely followed up after tumor resection and has not had any recurrences.Case presentationA 20-year-old, young asexual woman was diagnosed with cervical mullerian adenosarcoma with sarcomatous overgrowth (MASO). Cervical tumor resection was performed after admission, and the resection margin was negative. After the operation, she refused to undergo secondary surgery due to fertility requirements and did not receive adjuvant treatment. The patient was closely followed up after the operation and has not yet relapsed.ConclusionA young woman with cervical MASO did not receive adjuvant treatment after cervical tumor resection. For women with fertility requirements, close follow-ups should be conducted after the operation to guard against tumor recurrence and radical tumor resection should be performed as early as possible after the patient no longer requires their fertility.

The Spectrum of Fusions Occurring in Non-Smooth Muscle Mesenchymal Uterine Tumors: A Review of the Current Knowledge.

Non-smooth muscle uterine sarcomas are mostly represented by low-grade endometrial stromal sarcoma. However, several other rare, distinct types of uterine sarcoma are recognized, including high-grade endometrial stromal sarcoma, tumors with kinase fusions, uterine tumors resembling ovarian sex cord tumors, soft tissue-type sarcoma, and emerging entities such as KAT6A/B-rearranged tumors. The landscape of uterine sarcomas has changed, mostly because of the increasing knowledge concerning their molecular aberrations. To offer a comprehensive review of the literature focusing on fusions occurring in other than smooth muscle mesenchymal uterine tumors with respect to their type, frequency, and overlap between diagnostic categories and entities. The data were mined from the PubMed/MEDLINE database covering the time period from January 1988 to June 2023. In total, 156 studies focusing on the problematics of fusions occurring in non-smooth muscle mesenchymal uterine tumors were selected, and thus became the basis for this review. One hundred ten fusions were identified in 703 tumors. The diagnostic significance of the molecular aberrations occurring in these tumors can be unclear in some cases. This can be related to the rare aberrations with a limited number of reported cases. Additionally, even well-known aberrations considered as specific for a certain distinct entity can occur in more lesions, the biological behavior and clinical significance of which can differ substantially.

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.

Identification of hub genes distinguishing subtypes in endometrial stromal sarcoma through comprehensive bioinformatics analysis.

Diagnosing low-grade and high-grade endometrial stromal sarcoma (LG-ESS and HG-ESS) is a challenge. This study aimed to identify biomarkers. 22 ESS cases were analyzed using Illumina microarrays. Differentially expressed genes (DEGs) were identified via Limma. DEGs were analyzed with String and Cytoscape. Core genes were enriched with GO and KEGG, their pan-cancer implications and immune aspects were studied. 413 DEGs were found by exome sequencing, 2174 by GSE85383 microarray. 36 common genes were identified by Venn analysis, and 10 core genes including RBFOX1, PCDH7, FAT1 were selected. Core gene GO enrichment included cell adhesion, T cell proliferation, and KEGG focused on related pathways. Expression was evaluated across 34 cancers, identifying immune DEGs IGF1 and AVPR1A. Identifying the DEGs not only helps improve our understanding of LG-ESS, HG-ESS but also promises to be potential biomarkers for differential diagnosis between LG-ESS and HG-ESS and new therapeutic targets.