Extrauterine endometrial stromal sarcoma arising from malignant transformation of the vagina is an extremely rare condition. The diagnosis is often difficult as the symptomatology and pathological features overlap with that of pelvic endometriosis. A 38 years old female presented with complaints of dyspareunia, dysmenorrhea, and painful defecation along with blood-stained vaginal discharge for a year. Examination revealed the presence of multiple brownish irregular nodules in posterior vaginal fornix and fixed tender nodules which on biopsy revealed florid vaginal endometriosis. She improved symptomatically on medical therapy. After 18 months of diagnosis, she presented again with a necrotic growth in posterior fornix, which on repeat biopsy revealed a low-grade endometrial stromal sarcoma. Laparotomy revealed a 7×5 cm mass in the pouch of Douglas, infiltrating the posterior vaginal wall and rectum. A complete cytoreductive surgery with retrograde hysterectomy, excision of posterior vaginal wall and rectosigmoid resection was done. The patient is disease-free at a follow-up of 65 months.

UNASSIGNED: Endometrial stromal sarcoma (ESS) is a rare tumor that remains a diagnostic and therapeutic challenge to physicians worldwide. The metastatic setting implies a poor prognosis, with a 5-year survival rate below 40%. Patients with advanced-stage high-grade ESS (HG-ESS) have limited therapeutic options, often involving various chemotherapy regimens.
UNASSIGNED: This report depicts the case of a 47-year-old female diagnosed with HG-ESS. She underwent several lines of treatment starting with radiotherapy and brachytherapy, followed by multiple lines of treatment including trabectedin over several months. After retreatment with trabectedin and achieving disease stabilization for 10 months, treatment was optimized by trabectedin combined with radiotherapy, leading to stable disease that is still ongoing and lasts for over 17 months.
UNASSIGNED: Our case underscores the challenging nature of treating patients with HG-ESS and highlights the safety of long-term retrial with trabectedin, coupled with radiotherapy administration. This approach maintained a durable stable disease response in the metastatic setting.

UNASSIGNED: Endometrial stromal sarcoma (ESS) is extremely rare in pregnancy. It shares clinical and imaging features with more common pregnancy findings such as leiomyoma and molar gestations, which makes diagnosis challenging.
UNASSIGNED: A 36-year-old patient presented at 8 weeks and 1 day gestation for vaginal bleeding. An intrauterine pregnancy with an appropriately sized embryo with heart motion and a 9.5 cm complex uterine mass was found on ultrasound. MRI showed an 11.4 cm cystic mass with nodular septations causing mass effect on the endometrial cavity. After extensive counseling, the patient underwent a gravid abdominal hysterectomy and bilateral salpingectomy. Final pathology showed low grade ESS.
UNASSIGNED: This case highlights the importance of evaluating suspicious uterine masses in pregnancy and the necessity for safe abortion access.

The benign tumor uterine leiomyoma (UL) develops from the smooth muscle tissue that constitutes the uterus, whereas malignant tumor uterine sarcoma develops from either the smooth muscle tissue or stroma and is different from UL and endometrial cancer. Uterine sarcoma is broadly classified into three types: uterine leiomyosarcoma, endometrial stromal sarcoma (ESS), and carcinosarcoma. Although uterine leiomyosarcoma and ESS are both classified as uterine sarcoma, they significantly differ in terms of their sites of occurrence, symptoms, and treatment methods. Uterine leiomyosarcoma develops from the muscle tissue constituting the wall of the uterus and accounts for approximately 70% of all uterine sarcoma cases. In contrast, ESS develops from the stromal tissue beneath the endometrium and accounts for approximately 25% of all uterine sarcoma cases. ESS is classified as either low grade (LG) or high grade (HG). This case report aimed to highlight the importance of histopathologic examinations based on surgical specimens. Herein, we reported the case of a 45-year-old woman suspected of having submucosal leiomyoma of the uterus based on imaging results. Transvaginal ultrasonography and endometrial biopsy or partial dilation and curettage were performed. Contrast-enhanced magnetic resonance imaging (MRI) revealed a 32-mm mass projecting from the posterior wall of the uterus into the uterine cavity. T2-weighted imaging revealed a low signal within the mass; thus, submucosal UL was suspected. Histopathologic examination of surgical specimens obtained from a patient suspected of having submucosal UL after contrast-enhanced MRI indicated that the patient had ESS. Despite the remarkable advancements in medical imaging technology, the accuracy of contrast-enhanced MRI for detecting uterine mesenchymal tumors is limited. Therefore, histopathologic diagnosis based on surgical specimens should be performed when medical grounds for diagnosing a benign tumor on contrast-enhanced MRI are lacking.

Endometrial stromal sarcoma (ESS) is an uncommon uterine mesenchymal neoplasm. The primary extra-uterine location of ESS is a very rare occurrence. We present a case of a 39-year-old woman presented with severe abdominal pain, MRI showed bilateral ovarian tumors with heterogeneous intensity on T2-weighted imaging (T2WI) and T1-weighted imaging (T1WI), with restricted diffusion, including hyperintense areas on T1WI, not erased on T1-weighted fat-suppressed imaging, hypointense on T2WI, and not enhanced after contrast. This mass extended to the Douglas and invaded the uterine and the rectum serosa suggesting an underlying endometriosis. No abnormalities were suspected in the endometrium. The exploration revealed a friable mass arising from ovaries associated with nodules in the small intestine and sigmoid. The patient underwent bilateral adnexectomy and the anatomopathological study revealed a low-grade endometrial stromal sarcoma. This is one of the few reports covering the radiological features of low-grade extra-uterine ESS in the ovary which is probably secondary to degeneration of endometriosis with no evidence of primary uterine ESS.

BACKGROUND: Endometrial stromal sarcoma (ESS) is a rare malignancy of uterine in middle aged women. There are numerous subtypes for ESS which share the same clinical picture of uterine bleeding and pelvic pain. Consequently, diagnosis and treatment modalities of LG-ESS with metastasis are challenging. However, both molecular and immunological study of samples can be useful.
METHODS: In this case study, we report a 52-year-old woman presenting with the chief complaint of unusual uterine bleeding. There was no specific finding in her past medical history. The CT study revealed enlarged bilateral ovary with a significantly large left ovarian mass and suspicious mass in uterus. By the diagnosis of ovarian mass, patient went under total abdominal hysterectomy with bilateral salpingo-oophorectomy (BSO), greater omentectomy, and appendectomy followed by post-op hormone therapy. Her follow-up was uneventful. The IHC and pathological study of samples revealed incidental LG-ESS uterus mass with metastasis to ovaries despite her primary diagnosis.
CONCLUSIONS: LG-ESS has low metastasis rate. Surgical modalities and neoadjuvant therapies are recommended base on the stage of ESS. In the following study, we represent a case of incidental LG-ESS with bilateral ovarian invasion who was initially diagnosed as an ovarian mass.
CONCLUSIONS: Our patient was successfully managed by surgical intervention. Despite scarcity of LG-ESS, it is advised to consider LG-ESS as a differential diagnosis in management of patients with a uterus mass with bilateral ovarian involvement.

Uterine sarcomas are very infrequent and heterogeneous entities. Due to its rarity, pathological diagnosis, surgical management, and systemic treatment are challenging. Treatment decision process in these tumors should be taken in a multidisciplinary tumor board. Available evidence is low and, in many cases, based on case series or clinical trials in which these tumors have been included with other soft tissue sarcoma. In these guidelines, we have tried to summarize the most relevant evidence in the diagnosis, staging, pathological disparities, surgical management, systemic treatment, and follow-up of uterine sarcomas.

High-grade endometrial stromal sarcoma (HGESS) is a very rare kind of uterine tumor that accounts for less than 2% of all uterine cancers. The usual sites of metastasis are the abdomen and lung, while bone metastasis is very rare, with only a few cases reported till now. We present a case of HGESS with bone metastasis and review the literature.

BACKGROUND: The ZC3H7B-BCOR fusion gene has recently been described in tumours with kinship to so-called high grade endometrial stromal sarcoma (HG-ESS). This subset of tumour behaves similarly to YWHAE-NUTM2A/B HG-ESS, however, they are both morphologically and immunophenotypically distinct neoplasms. The identified rearrangements in the BCOR gene have been accepted as both the driver and requisite feature in creating a novel sub-entity within the category of HG-ESS. Preliminary investigations into BCOR HG-ESS have shown similar outcomes to YWHAE-NUTM2A/B HG-ESS, with patients typically presenting with high stage disease. Clinical recurrences and metastases to lymph nodes, sacrum/bone, pelvis/peritoneum, lung, bowel and skin have been identified. In this report, we describe a case of BCOR HG-ESS, that is deeply myoinvasive and widely metastatic. Metastatic deposits include a mass in the breast discovered on self-examination; a metastatic site that has yet to be reported in the literature.
METHODS: A 59-year-old female underwent biopsy for post-menopausal bleeding, yielding a diagnosis of \"low-grade spindle cell neoplasm with myxoid stroma and endometrial glands\", favouring endometrial stromal sarcoma (ESS). She was then referred for total hysterectomy and bilateral salpingo-oophorectomy. The resected uterine neoplasm was both intracavitary and deeply myoinvasive with morphology consistent with that of the biopsy specimen. Characteristic immunohistochemistry (IHC) was noted, and fluorescence in situ hybridization confirmed BCOR rearrangement, supporting a diagnosis of BCOR HG-ESS. A few months postoperatively, the patient underwent needle core biopsy of the breast which revealed metastatic HG-ESS.
CONCLUSIONS: This case highlights some of the diagnostic challenges posed by uterine mesenchymal neoplasms, and exemplifies the emerging histomorphologic, immunohistochemical, molecular and clinicopathologic features of the recently described HG-ESS with ZC3H7B-BCOR fusion. It adds to the body of evidence supporting the inclusion of BCOR HG-ESS as a sub-entity of HG-ESS within the endometrial stromal and related tumours subcategory of uterine mesenchymal tumors, as well as the poor prognosis and high metastatic potential of this tumor.

Endometrial stromal sarcoma (ESS) is a rare, malignant tumor of the endometrium. Low-grade endometrial stromal sarcoma (LG-ESS) is a less aggressive subtype of ESS that rarely metastasizes to the heart and large blood vessels. In the present study, we report a case of recurrent LG-ESS after treating the initial mass in the uterus six years ago in a 49-year-old female who presented with a four-month history of dyspnea and easy fatigability. Investigations revealed a right pulmonary embolism, suspicious right psoas muscle mass, and a large inferior vena cava (IVC) thrombus. One month later, she presented with multiple gastrointestinal symptoms and weight loss. Investigations then showed the development of a new right atrial mass, infra-diaphragmatic metastatic lymphadenopathy, progression of the presacral soft tissue component, invasion of the ileal bowel loop, and a tumoral thrombus in the IVC besides new metastatic lymphadenopathy and pulmonary metastasis. Therefore, a multidisciplinary team, which had a crucial role in this complicated case, decided to commence chemotherapy treatment. Such an unusual aggressive metastatic course of LG-ESS is limited in the literature; herein, we recognize a rarely documented disease.