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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.

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.

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.

We report on a 50-year-old postmenopausal woman who presented with abnormal uterine bleeding and pelvic pain due to a uterine solid mass grew from the uterine fundus to the cervix and with so far undescribed obviously gelatinous grossly change, which was suspected of myxoid leiomyosarcoma in intraoperative diagnosis. Morphologically, the tumor cells displayed haphazard fascicles of uniform mild-to-moderate heteromorphic spindle cell component with significant and abundant myxoid stroma, forming signet ring cells and microcysts. Immunohistochemically, the tumour cells were diffusely positivefor CD10 and cyclin D1 and negative for Desmin and SMA, but the expression of BCOR staining was not present. The FISH study showed a positive BCOR gene break probe, and the RNA sequencing revealed an identified reciprocal fusion gene ZC3H7B-BCOR. The case was finally diagnosed as ZC3H7B-BCOR high-grade endometrial stromal sarcoma. Tumor recurrence occurred rapidly on the pelvic peritoneal and vaginal 2 months after resection. In conclusion, these findings further support ZC3H7B-BCOR HGESS has a poor prognosis and molecular testing of uterine mesenchymal tumors with myxoid matrix and unusual grossly presentation is recommended to avoid misdiagnosis.

UNASSIGNED: Endometrial stromal sarcoma is a relatively rare malignant tumor that derives from the malignant transformation of primitive uterine mesenchymal cells. It can lead to distant metastases. High-grade endometrial stromal sarcoma is extremely rare. The adrenal glands are an unreported site of metastasis.
UNASSIGNED: A 71-year-old woman with a diagnosis of endometrial stromal sarcoma 30 months ago. After receiving treatment with radiotherapy and chemotherapy, the patient was kept asymptomatic during the follow-up until 2 years later, when she complained of dyspnea. Pulmonary and right adrenal gland metastases were detected by 18F-FDG PET/CT. The right upper lobe mass was diagnosed as a high-grade endometrial stromal sarcoma metastasis after postoperative pathology. Due to the patient\'s high risk of surgery, as she had many underlying diseases, we performed adequate preoperative preparation. The physical examination revealed that a hard mass was palpable in the right renal area. The right adrenal mass was resected in our hospital. Immunohistochemistry showed ER (-), PR (-), CD10 (+), P16 (+), Ki-67 (50%). The final diagnosis on pathological examination was a high-grade ESS metastatic to the right adrenal gland. The patient continued treatment in other hospitals after surgical resection. After four months of postoperative follow-up, metastasis was detected again during a PET/CT examination at an outside hospital.
UNASSIGNED: Endometrial stromal sarcoma is rare, and the adrenal glands are an unreported site of metastasis. It has no specific clinical symptoms and mainly found for other reasons. The diagnosis still depends on pathology and immunohistochemistry. If there is no relevant past history, it is difficult to exclude a primary adrenal tumor.

The World Health Organization currently divides endometrial stromal sarcomas into 4 different entities, based on their clinical and pathological features: endometrial stromal nodule, low-grade endometrial stromal sarcomas (LG-ESS), High-grade endometrial stromal sarcomas, and undifferentiated uterine sarcoma. The fibroblastic variant of LG-ESS is rare and is usually made of small tumoral cells of oval to fusiform shape, demonstrating low cytologic atypia and low mitotic activity, which can lead to confusion with a benign myofibroblastic proliferation. We hereby report a rare case of a fibroblastic variant of LG-ESS in a 37-year-old woman presenting abundant metrorrhagia, which was initially misdiagnosed as an inflammatory pseudotumor before proofreading in our laboratory, along with a review of a histological and immunohistochemical findings, aiming to help pathologists avoid this diagnosis pitfall.