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.

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.

OBJECTIVE: We report a low-grade endometrial stromal sarcoma (ESS) with a novel CDKN1A-JAZF1 fusion gene arising from abdominal wall endometrioma.
METHODS: A 40-year-old woman presented with a 5.5-cm abdominal wall mass juxtaposed to the postoperative scar of two cesarean sections. Histologically, the tumor exhibited obvious tongue-like protrusions into the surrounding tissue, showed spindle cells with multinodular growth pattern that occasionally rotate around small arteries. Immunohistochemically, the tumor cells were positive for CD10, estrogen receptor (ER), progesterone receptor (PR), negatively stained for smooth muscle actin (SMA), CD117, CyclinD1. In addition, a previously undescribed gene fusion between CDNK1A 5\' end of exon 1(NM_000389.5) and JAZF1 3\' end of exon 5 (NM_175,061,3) was reported in this case.
CONCLUSIONS: This report of ESS suggesting that rapidly growing abdominal wall masses without menstruation-related should be promptly evaluated and treated aggressively. In addition, we have expanded the molecular landscape of low-grade ESS.

The clinical value of lymph node dissection remains controversial. This study aimed to evaluate the impact of lymphadenectomy on the prognosis of patients with uterine leiomyosarcomas (uLMS) or endometrial stromal sarcomas (ESS). PubMed, EMBASE and the Cochrane Library were searched for studies describing the prognostic significance of lymphadenectomy in uLMS or ESS. Quality assessments were performed using the Newcastle-Ottawa Scale, relative hazard ratios and a random-effects model. Thirty-two retrospective cohort studies that included 26,693 patients in total were enrolled. Patients with uLMS or low-grade ESS (LG-ESS) had no survival benefits from lymphadenectomy. However, patients with high-grade ESS (HG-ESS), did show survival benefits of lymphadenectomy, with no heterogeneity. No significant evidence of publication bias was found. Lymphadenectomy had little prognostic effect on patients with early-stage uLMS or LG-ESS. The best treatment for HG-ESS is early, comprehensive hysterectomy with lymph node dissection.

Background and Objectives: Uterine sarcomas represents only 3% of all the female genital tract ones. The tumoral stage is the most significant prognostic factor. The role of the bilateral salpingo-oophorectomy (BSO) in the surgical management of FIGO stage IA and IB appears still controversial. This review aims to investigate the impact of bilateral adnexectomy in the treatment of uterine sarcoma. Methods: Following the recommendations in the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement, we systematically searched the PubMed, Scopus, Cochrane, Medline, and Medscape databases in February 2022. We applied no language or geographical restrictions, but we considered only English studies. We included the studies containing data about Recurrence Rate (RR), Disease-free Survival (DFS), and Overall Survival (OS). We used comparative studies for meta-analysis. Results: Seventeen studies fulfilled the inclusion criteria; 2 retrospective observational studies, and 15 retrospective comparative studies, And 14 out of the 15 comparative studies were enrolled in meta-analysis. A total of 3743 patients were analyzed concerning the use of adnexectomy with hysterectomy in patients with uterine sarcoma and compared with those who did not. Meta-analysis highlighted a non-significant worsening of the OS in the BSO group compared to the OP group and showed that adnexectomy does not improve the DFS (BSO OR 1.23 (95% CI 0.81-1.85) p = 0.34; I2 = 24% p = 0.22). Conclusions: Most studies selected for our review showed that adnexectomy does not significantly affect the RR, OS, and PFS in treating FIGO stage I uterine sarcomas. Therefore, even if there is a unanimous consensus about bilateral adnexectomy in menopausal patients, preservation of ovarian tissue may be considered in premenopausal women. Nonetheless, there are not enough cases in the literature to recommend this procedure.

Endometrial stromal sarcoma (ESS) is the second most common uterine mesenchymal neoplasm. ESS can arise from extrauterine locations without any uterine involvement and is called extrauterine ESS (EESS). The epidemiological features of EESS are not well-known. Moreover, the factors affecting its outcome have not been systemically studied. The treatment of EESS closely follows that of uterine ESS, comprised of different combinations of surgical management, hormone therapy, chemotherapy, and radiation therapy. However, the effectiveness of different treatment protocols for EESS has not been studied. Here, we have performed a systematic review of all reported cases of EESS in the English literature. We further performed a meta-analysis of the outcome data and investigated how the patients\' age, tumor site, tumor size, and management affect the overall and progression-free survival of the patients. We found that tumor site and mode of treatment significantly affected the overall survival and progression-free survival of the patients. Tumor size significantly affected overall survival but not progression-free survival, while the age at diagnosis did not affect patient outcome. As far as we know, ours is the first systematic study of this rare malignancy with an emphasis on outcome analysis.

Collision tumors are characterized by occurrence of two or more histologically distinct tumor types at the same anatomic site. Collision tumors have been reported in various organs, such as esophagus, stomach, colon, kidney, lung, skin, thyroid, breast, ovary and uterus. Uterine collision tumors of epithelial and mesenchymal origin are rare and often underrecognized. We report a rare concurrent occurrence of endometrial stromal sarcoma and endometrioid adenocarcinoma in a 65-year-old female. It is important to differentiate collision tumors from carcinosarcoma due to impact on clinical management and prognosis. Extensive gross sampling and careful morphological examination aided by immunohistochemical studies is necessary for the diagnosis of this rare entity. This case report aims to increase the awareness of this rare pathological entity with discussion on the management issue based on review of literature. This is the first case in Indian literature to the best of our knowledge.

Endometrial stromal sarcoma (ESS) is a rare mesenchymal tumor of the uterus that accounts for 7-25% of uterine sarcomas and < 1% of uterine tumors. Previously reported sites include the ovary, bowel wall, abdomen, peritoneum, pelvis, and vagina; however, ESS in the extrauterine area is rare. We report a rare case of endometrial stromal sarcoma that developed in the sigmoid colon along the gonadal vasculature, which was difficult to distinguish from colon cancer. A large polyp was found in the sigmoid colon of a 74-year-old woman during a routine medical examination and was diagnosed as tubular adenoma. On colonoscopy 7 months later, the tumor had grown and blocked the lumen, causing stenosis. She was referred to our hospital for surgery. Although detailed examination at our hospital did not yield a definitive diagnosis, bowel obstruction was considered likely and we performed laparoscopic low anterior resection under a preoperative diagnosis of sigmoid colon cancer. The tumor protruded into the sigmoid colon from the stump of the ovarian arteries and veins outside the intestinal tract. As the left ovarian artery and vein were involved in the tumor, we extracted them as a lump. The tumor was diagnosed as low-grade ESS (LG-ESS). She had a history of hysterectomy and left salpingo-oophorectomy for uterine myoma 25 years ago, and radiation therapy was performed after surgery for an unknown reason. The postoperative course was uneventful, and follow-up was continued at the request of the patient. We report a rare case of ESS infiltrating the sigmoid colon, which was probably a lesion derived from endometriosis of the ovarian arteriovenous stump remaining after surgery 25 years ago.

The aim of this article is to summarize the MRI features of each sarcoma subtype and to correlate them with its pathological findings. Literature review through PubMed/Medline database to identify relevant articles on uterine sarcomas, with a special emphasis on their MRI findings and pathological features. While several, more generalistic, MRI findings of a uterine tumour should raise suspicion for malignancy (including irregular contour, intra-tumoral necrosis/hemorrhage and low ADC values), some particular features may suggest their specific histological subtype such as the gross lymphovascular invasion associated with endometrial stromal sarcomas, the \"bag of worms\" appearance of the low-grade endometrial stromal sarcoma and the \"lattice-like\" aspect of adenosarcomas which results from the mixed composition of solid and multiseptated cystic components. Knowledge of the different histological uterine sarcoma subtypes, their specific MRI features and comprehension of their pathological background allows for a more confident diagnosis and may indicate the correct histological subtype.

Endometrial stromal sarcoma (ESS) is an uncommon mesenchymal tumor that accounts for less than 1% of all primary uterine malignancies and extrauterine endometrial stromal sarcoma (EESS) is even rarer. We report the case of a 75-year-old woman with an abdominal tumor and multiple peritoneal implants. Histological analysis of the surgical specimens showed bland cellularity resembling normal endometrial stroma. The diagnosis of a low-grade EESS was confirmed by immunophenotypic findings and demonstration of JAZF1 translocation. After extensive sampling, no evidence of endometriosis was found. Our case showed atypical aggressive behavior and we discuss the possible influence of the high mitotic count (8/10 HPFs) in some areas of the tumor, the multifocality of the abdominal implants and the postmenopausal status of the patient. The unusual clinical presentation and extrauterine location of such a rare tumor were challenging implying a wide range of differential diagnosis. The correlation of morphological, immunohistochemical and molecular findings was necessary to arrive at the correct diagnosis.