A Rare Case of Extrauterine Endometrial Stromal Sarcoma Arising from Deep Pelvic Endometriosis: Role of Multidisciplinary Team Meeting.

INTRODUCTION: Extrauterine endometrial stromal sarcoma (EESS) arising from Deep pelvic endometriosis (DPE) has a poor life quality and is difficult to diagnose pre-operatively. However, the patient's quality of life can be improved when it is diagnosed precisely and managed successfully. CASE REPORT: A 35-year-old woman presented to our hospital with a 3-month history of hematochezia and anal pain. Initially, she was misdiagnosed as a rectal stromal tumor and then precisely diagnosed as having EESS from DPE following several multidisciplinary team (MDT) meetings. The lesion was shrunk by gonadotrophin-releasing hormone agonist (GnRH-α) treatment and then resected with minimal trauma. CONCLUSION: MDT is crucial in the treatment of the patient. It can promote individualized treatment and improve patient's quality of life.

Primary ovarian endometrioid stromal sarcoma presenting with reno-ureteral colic.

BACKGROUND: Endometrioid Stromal Sarcomas are an infrequent group of mesenchymal tumors that we must take into account in the differential diagnosis despite representing only 0.2% of tumors of the female genital tract, as they can go unnoticed until advanced stages. CASE PRESENTATION: Fifty-fourth year-old woman referred from the Urology department due to incidental finding of adnexal mass in MRI during examination after renoureteral colic, in the case of a 50 mm solid cystic mass in LE. MT were within the normal range, and the CT scan observed this mass in contact with the left ureter. The surgery was completed with hysterectomy and contralateral adnexectomy without incident and chemotherapy treatment was not added. The pathological result was ovarian tissue with low-grade endometrial sarcoma. Currently, after two years of follow-up, the patient remains stable without any recurrence of disease. CONCLUSIONS: Endometrioid stromal sarcomas are rare tumors that originate in the endometrial stroma, the ovarian location being rare. Management lies in surgical treatment, and adjuvant therapy is sometimes necessary in advanced stages.

Extrauterine Endometrial Stromal Sarcoma Mimicking Colorectal Cancer With Metastases.

ABSTRACT: Extrauterine endometrial stromal sarcoma (EESS) is extremely rare, especially with the colorectum involvement. We describe 18 F-FDG PET/CT findings of EESS in the sigmoid colon and rectum in a 46-year-old woman who complained diarrhea. 18 F-FDG PET/CT revealed multiple hypermetabolic lesions in the abdomen and pelvis, including the sigmoid colon and rectum. Therefore, colorectal cancer with metastases was initially suspected; however, ultimately low-grade EESS was pathologically confirmed. Widespread EESS with intestine involvement revealed by 18 F-FDG PET/CT should be carefully distinguished from colorectal cancer with metastases.

An extremely rare disconnection of the external iliac artery and novel collateral remodeling in an endometrial stromal sarcoma woman.

BACKGROUND: Injury to the external iliac artery can have serious consequences and can be extremely challenging for surgeons. Here, we report a patient with bizarre disconnection of the external iliac artery during a laparoscopic operation. CASE PRESENTATION: On May 27, 2020, during a laparoscopic pelvic lymphadenectomy operation to treat endometrial stromal sarcoma, we encountered an unusual anatomy: abnormal disconnection of the left external iliac artery in a 26-year-old female patient. The proximal and distal ends of the left external iliac artery demonstrated old narrowing without active bleeding, and the distance between the two disconnected ends was more than 3 cm. The scenario was surprising to all the staff in the operating theater. After a comprehensive assessment of skin temperature, arterial pulsation and arterial blood flow, a multidisciplinary team determined that collateral circulation of the left lower limb had been established and could meet the blood supply of the lower limbs, which was also confirmed three times by computed tomography angiography and Doppler ultrasound of the blood vessels in the abdomen and lower limbs. Sixteen months after the operation, the patient had no obvious abnormality, and the daily activities of the left lower limb were not affected. Follow-up after treatment for the patient is still in progress. CONCLUSIONS: We describe the details of the whole case of disconnection of the external iliac artery. We hope to summarize the experience and lessons learned through this case and a relevant literature review to improve the safety and orderliness of our future clinical work.

Computer-aided diagnosis of low grade endometrial stromal sarcoma (LGESS).

Low grade endometrial stromal sarcoma (LGESS) accounts for about 0.2% of all uterine cancer cases. Approximately 75% of LGESS patients are initially misdiagnosed with leiomyoma, which is a type of benign tumor, also known as fibroids. In this research, uterine tissue biopsy images of potential LGESS patients are preprocessed using segmentation and stain normalization algorithms. We then apply a variety of classic machine learning and advanced deep learning models to classify tissue images as either benign or cancerous. For the classic techniques considered, the highest classification accuracy we attain is about 0.85, while our best deep learning model achieves an accuracy of approximately 0.87. These results clearly indicate that properly trained learning algorithms can aid in the diagnosis of LGESS.

MR Imaging of uterine sarcomas: a comprehensive review with radiologic-pathologic correlation.

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.

Severe Abdominal Recurrence of Low-grade Endometrial Stromal Sarcoma After Hysteroscopic Surgery.

BACKGROUND: Low-grade endometrial stromal sarcoma (LG-ESS) is an indolent tumor harboring gene fusion involving polycomb family genes. While early LG-ESS has a good clinical course, some tumors have pelvic recurrence. The etiology and genetic alterations involved in the process remain unknown. CASE REPORT: A 44-year-old nulliparous woman underwent hysteroscopic surgery for a 2.5 cm submucosal uterine tumor with negative endometrial cytology. Pathological evaluation revealed LG-ESS. On the 31st day, total laparoscopic hysterectomy was indicated. She was diagnosed with stage IA (pT1aNXM0) LG-ESS without lymphovascular invasion. At 4 years, positron-emission tomography showed multiple pelvic masses. Secondary debulking surgery was performed, which revealed severe intra-abdominal recurrence of LG-ESS with JAZF1-SUZ12 fusion. CONCLUSION: Hysteroscopic surgery is a convenient tool for benign uterine submucosal diseases. However, intrauterine morcellation with fluid can lead to unexpected recurrence of occult LG-ESS. It is important when seeking consent for surgery to inform patients about the possible risk of dissemination of uterine mesenchymal tumors.

Low-Grade Endometrial Stromal Sarcoma Extending to The Right Atrium.

Endometrial Stromal Sarcoma (ESS) are very uncommon malignant tumors that make around 0.2% of the entire uterine related malignancies. They represent the endometrial stromal cells in the proliferative stage. The annual incidence of ESS is 1-2 per million women. We present an unusual case of ESS, which originates from the uterine artery through the Inferior Vena Cava (IVC) and extends to the right atrium. A 48 years old woman presented with menorrhagia and right flank pain for seven months. Physical examination was normal except for a palpable suprapubic mass. Computed tomography (CT) showed the right gonadal vein and IVC tumoral thrombus extending into the right atrium. An echocardiogram and Transesophygeal echocardiogram revealed a large non-mobile echogenic density in the right atrium. Total abdominal hysterectomy with bilateral salpingo-oophorectomy was done. Extensive myometrial and vascular invasion was noted along with the extensive lymphovascular invasion of the uterus. Eventually, distal IVC resections were done, and there was no chance to remove the tumor because it was too attached and invaded the IVC filter. Owing to the unresectability of the tumor, the patient was assigned for palliative chemotherapy. Keywords: Endometrial Stromal Sarcoma, Right Atrium, Inferior Vena Cava, Spindle Cell Sarcoma.

Extra-uterine endometrial stromal sarcoma arising from deep infiltrating endometriosis.

We present a compelling case of a 45-year-old female with a history of endometriosis and leiomyomas, who presented to her gynecologist with chronic pelvic pain complaints. Both a transvaginal ultrasound (US) and an MRI (magnetic resonance imaging) were ordered. The US demonstrated multiple uterine lesions, likely fibroids, and an endometrioma within the right ovary. The MRI of the pelvis with and without gadolinium identified a mass within the right ovary with homogenous intermediate T2-signal, restricted diffusion, and delayed enhancement relative to the myometrium. Several irregular-shaped lesions were also noted within the external myometrium, anterior pelvic wall, and the peritoneum, which were intermediate signal on T2-weighted images, restricted diffusion, and an enhancement pattern similar to the myometrium. The patient underwent a right adnexectomy. The histopathology findings were consistent with a low-grade endometrial stromal sarcoma (low grade-ESS) arising from the endometrial stroma of the right ovary. A debulking surgery confirmed the involvement of external myometrium, anterior pelvic wall, and the peritoneum secondary to a low-grade ESS without the endometrial cavity's involvement. The underlying hypothesis is that the endometriosis stroma from extra-uterine structures such as the right ovary, pelvic and anterior peritoneum, and external myometrium may have subsequently resulted in a low-grade ESS. Low-grade extra-uterine ESS without endometrial involvement is a rare entity. Based on our literature search, this is one of the few reports covering the radiological features of low-grade extra-uterine ESS arising outside the uterus with a concomitant deep infiltrating endometriosis, but without the involvement of the endometrial cavity.

[THE USE OF CT-GUIDED MARKING FOR THE LAPAROSCOPIC RESECTION OF A SOLITARY RETROPERITONEAL TUMOR].

When resecting small tumors or tumors with an irregular margin, a marking technique is conducted prior to the surgery. CT-guided marking techniques are common in pulmonary surgery, but it is rarely used in abdominal or urological surgery. We performed a marking technique for a small tumor that was undetectable by ultrasound using CT guidance prior to laparoscopic resection.A 63 year-old woman, two years after total hysterectomy for uterine stromal sarcoma, underwent combined right kidney resection and retroperitoneal tumor resection for a giant recurrence. Two months after the surgery, micro recurrence was observed in the vicinity of the right intestinal psoas muscle which upon follow up, the tumor size increased to 1 cm. Surgical resection of the small recurrent tumor was planned. Since it was difficult to detect by ultrasound, preoperative CT-guided marking was performed. Retroperitoneal laparoscopic resection was performed the following day. The histopathological diagnosis was endometrial stromal sarcoma.

Imaging in gynecological disease (15): clinical and ultrasound characteristics of uterine sarcoma.

OBJECTIVE: To describe the clinical and ultrasound characteristics of uterine sarcomas. METHODS: This was a retrospective multicenter study. From the databases of 13 ultrasound centers, we identified patients with a histological diagnosis of uterine sarcoma with available ultrasound reports and ultrasound images who had undergone preoperative ultrasound examination between 1996 and 2016. As the first step, each author collected information from the original ultrasound reports from his/her own center on predefined ultrasound features of the tumors and by reviewing the ultrasound images to identify information on variables not described in the original report. As the second step, 16 ultrasound examiners reviewed the images electronically in a consensus meeting and described them using predetermined terminology. RESULTS: We identified 116 patients with leiomyosarcoma, 48 with endometrial stromal sarcoma and 31 with undifferentiated endometrial sarcoma. Median age of the patients was 56 years (range, 26-86 years). Most patients were symptomatic at diagnosis (164/183 (89.6%)), the most frequent presenting symptom being abnormal vaginal bleeding (91/183 (49.7%)). Patients with endometrial stromal sarcoma were younger than those with leiomyosarcoma and undifferentiated endometrial sarcoma (median age, 46 years vs 57 and 60 years, respectively). According to the assessment by the original ultrasound examiners, the median diameter of the largest tumor was 91 mm (range, 7-321 mm). Visible normal myometrium was reported in 149/195 (76.4%) cases, and 80.0% (156/195) of lesions were solitary. Most sarcomas (155/195 (79.5%)) were solid masses (> 80% solid tissue), and most manifested inhomogeneous echogenicity of the solid tissue (151/195 (77.4%)); one sarcoma was multilocular without solid components. Cystic areas were described in 87/195 (44.6%) tumors and most cyst cavities had irregular walls (67/87 (77.0%)). Internal shadowing was observed in 42/192 (21.9%) sarcomas and fan-shaped shadowing in 4/192 (2.1%). Moderate or rich vascularization was found on color-Doppler examination in 127/187 (67.9%) cases. In 153/195 (78.5%) sarcomas, the original ultrasound examiner suspected malignancy. Though there were some differences, the results of the first and second steps of the analysis were broadly similar. CONCLUSIONS: Uterine sarcomas typically appear as solid masses with inhomogeneous echogenicity, sometimes with irregular cystic areas but only very occasionally with fan-shaped shadowing. Most are moderately or very well vascularized. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

Current Status of Magnetic Resonance Imaging in Patients with Malignant Uterine Neoplasms: A Review.

In this study, we summarize the clinical role of magnetic resonance imaging (MRI) in the diagnosis of patients with malignant uterine neoplasms, including leiomyosarcoma, endometrial stromal sarcoma, adenosarcoma, uterine carcinosarcoma, and endometrial cancer, with emphasis on the challenges and disadvantages. MRI plays an essential role in patients with uterine malignancy, for the purpose of tumor detection, primary staging, and treatment planning. MRI has advanced in scope beyond the visualization of the many aspects of anatomical structures, including diffusion-weighted imaging, dynamic contrast enhancement-MRI, and magnetic resonance spectroscopy. Emerging technologies coupled with the use of artificial intelligence in MRI are expected to lead to progressive improvement in case management of malignant uterine neoplasms.

Removal of an endometrioid stromal sarcoma from the inferior vena cava and right atrium.

Entometrioid stromal sarcomas are seen in extra-uterine as well as extra-gonadal sites and have a strong association with endometriosis. Although having better prognosis than other sarcomas, yet these tumors may relapse (whether local or distant) in up to 56% of cases, even as late as 20 years after surgery. We report a case of a 30-year-old female patient with a mass in the inferior vena cava and right atrium which was surgically removed using cardiopulmonary bypass and deep hypothermic circulatory arrest and turned to be an entometrioid stromal sarcoma. The patient gave a history of endometriosis followed by the appearance of a low-grade ovarian endometrioid stromal sarcoma 4 years before the development of the mass in the IVC and right atrium.

Endometrial Stromal Sarcoma Arising from Endometrial Polyp: A Case Report.

Endometrial stromal sarcoma (ESS) is a rare malignant tumor of the uterus. We report an uncommon case of ESS composed of both low-grade ESS and high-grade ESS arising from an endometrial polyp. On the findings of magnetic resonance imaging and contrast computed tomography, a patient was suspected of having uterine malignant tumor. She underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy. Macroscopically, the tumor was a polypoid lesion in the uterine cavity. The tumor was an endometrial polyp with ESS components. ESS was composed of low-grade ESS and high-grade ESS. By immunohistochemistry, both an endometrial polyp and low-grade ESS showed a positivity for CD10, estrogen receptor (ER), and progesterone receptor (PR). However, high-grade ESS showed only a focal and weak CD10 positivity with no immunostaining for ER and PR. A focal or diffuse positivity for α-smooth muscle actin and desmin was noted in both low-grade and high-grade ESS. The positive rates of Ki-67 and p53 in high-grade ESS were elevated up to over 95%. She was diagnosed as having ESS in a stage IA. After surgery, she received no further treatment. She has been without recurrence for 4 years since an initial surgery. In conclusion, immunohistochemical analyses are useful for make an accurate diagnosis of ESS showing a transition from low-grade ESS to high-grade ESS in addition to the conventional method.

Rare case of undifferentiated uterine sarcoma with neuroectodermal differentiation and osteoclast-like giant cells.

OBJECTIVE: We describe the first case of a rare undifferentiated uterine sarcoma exhibiting both neuroectodermal differentiation and osteoclast-like giant cells, and elaborate its morphology. CASE REPORT: A 54-year-old woman presented with suprapubic pain, frequent urination, and perimenopausal abnormal vaginal bleeding. Computed tomography revealed a large heterogeneous uterine mass and multiple lung nodules. She received a staging surgery. The tumor pathology examination revealed an undifferentiated uterine sarcoma (UUS) with neuroectodermal differentiation and osteoclast-like giant cells (OGCs). The patient was managed with palliative hospice care; however, she died within 1.5 months of diagnosis. CONCLUSION: UUSs are rare high-grade tumors observed in elderly women. These women typically present with postmenopausal bleeding and extrauterine diseases and have a poor prognosis. Neuroectodermal differentiation in UUSs has a müllerian origin. The presence of OGCs may suggest a poor prognosis; however, further studies are necessary to determine the exact nature of such neoplasms.

What MRI features suspect malignant pure mesenchymal uterine tumors rather than uterine leiomyoma with cystic degeneration?

OBJECTIVE: To retrospectively assess conventional magnetic resonance imaging (MRI) features that differentiate malignant pure mesenchymal uterine tumors (MPMUT); endometrial stromal sarcoma (ESS) and leiomyosarcoma (LMS) from uterine leiomyoma with cystic degeneration (ULCD). METHODS: We retrospectively reviewed magnetic resonance (MR) images of 30 patients with ULCD, 18 with ESS, and 15 with LMS, to assess tumor location, margin, T2 signal intensity (SI), speckled appearance, and peripheral band using univariate and multivariate analyses. RESULTS: ULCD more frequently showed subserosal location (53.3%), well-defined margin (96.7%), and speckled appearance (90.0%) compared with ESS (0%, 33.3%, and 33.3%, respectively) or LMS (20.0%, 33.3%, and 60.0%, respectively). In quantitative T2 SI comparisons, the T2 SI ratio of the main solid tumor portion to gluteus maximus muscle differed significantly among the three groups, with ULCD showing a lower SI ratio (0.62) compared with ESS (2.44) and LMS (1.13). On multivariate analysis, an ill-defined margin (odds ratio [OR]=44.885; p=0.003) and high T2 SI (OR=4.396; p=0.046) were the significant MR differentiators. CONCLUSION: An ill-defined tumor margin and high T2 SI ratio of the main solid tumor-to-gluteus maximus muscle were useful MRI features in the differentiation of MPMUT from ULCD.

Cardiac metastatic endometrial stromal sarcoma 17 years after hysterectomy.

We report a 60-year-old female who underwent resection of an endometrial stromal sarcoma of the right ventricle 17 years following a hysterectomy and radiation therapy for the same tumor.