scholarly journals Low-grade Endometrial Stromal Sarcoma with Intracaval or Intracardiac Extension: A Retrospective Study of 8 Cases

Author(s):  
Junyu Chen ◽  
Jinhui Wang ◽  
Dongyan Cao ◽  
Jiaxin Yang ◽  
Huifang Huang ◽  
...  

Abstract Purpose: This study aimed to improve the knowledge of low-grade endometrial stromal sarcoma (LG-ESS) with intracaval or intracardiac extension and tried to identify the potential risk factors and optimal treatment method influencing prognosis.Methods: We performed a retrospective review of eight LG-ESS patients with intracaval or intracardiac extension who underwent treatment at Peking Union Medical College Hospital between 2012 and 2020.Results: The median age at diagnosis was 44 years, ranging from 28 to 56 years. Abnormal uterine bleeding was the most common intimal symptom (3/8), followed by low back discomfort (2/8), edema of the lower limbs (2/8), abdominal pain (1/8), and dyspnea (1/8). All patients underwent resection of the intravascular and extravascular portions of the tumor. Two patients were in stage IIIC, and 6 were in stage IVB. After surgery, 4 patients received adjuvant radiotherapy, of whom 3 also received letrozole. One patient was treated with letrozole alone, and 1 patient received medroxyprogesterone. The average follow-up time was 34.5 months, ranging from 6 to 98 months. No patients died or relapsed during the follow-up period.Conclusions: LG-ESS with intracaval or intracardiac extension is an uncommon type of tumor which is easily misdiagnosed and can only be diagnosed by histological evaluation after surgery. Complete tumoral excision followed by adjuvant therapy may benefit patient survival time. Long-term follow-up is essential due to the high rate of late recurrence.

2021 ◽  
Vol 2021 ◽  
pp. 1-4
Author(s):  
Lana Nseir ◽  
Georget Mansour ◽  
Junior Makhoul ◽  
Luna Skaf ◽  
Mohammad Ziad Dahhan ◽  
...  

Endometrial stromal sarcoma (ESS) is a rare and challenging type of endometrial tumor, constituting only about 0.2% of all uterine malignancies and occurring in women 42–58 years of age. ESS is usually misdiagnosed as leiomyoma. They both have nonspecific symptoms, which makes the diagnosis of ESS difficult to achieve. As the ESS is infrequently encountered, optimal management is quite debatable. Here, we report a rare case of a 22-year-old Syrian female who presented with abnormal uterine bleeding; the preoperative diagnosis suggested leiomyoma while histopathological and immunohistochemical studies confirmed the diagnosis of LG-ESS stage IIA. Therefore, the treatment plan was shifted from a conservative myomectomy to a total hysterectomy with both adnexa. The aim of this case report is to draw attention to this uncommon tumor at young age of patients as well as to have awareness of the necessity to suspect this diagnosis especially with the presentation of rapid enlargement of uterine leiomyoma.


1999 ◽  
Vol 195 (7) ◽  
pp. 501-508 ◽  
Author(s):  
Yoshiki Mikami ◽  
Rita I. Demopoulos ◽  
Fouad Boctor ◽  
Elena F. Febre ◽  
Margaret Harris ◽  
...  

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Qianwen Dai ◽  
Baolin Xu ◽  
Huanwen Wu ◽  
Yan You ◽  
Ming Wu ◽  
...  

Abstract Background The prognosis of recurrent low-grade endometrial stromal sarcoma (LGESS) is little known. This study was to investigate the survival outcomes of a cohort of patients with recurrent LGESS. Methods Patients with primary LGESS diagnosed and treated for first recurrence confirmed by histology in the study center from February 2012 to June 2019 were retrospectively included. The progression-free interval (PFI) after the last treatment for first recurrence and overall survival (OS) since the diagnosis of first recurrence, which were followed up to June 1, 2020, were compared between groups of various therapy modalities. Results Fifty-six patients were included, and 43 patients (76.8%) had definite follow-up outcomes. The 5-year PFI and OS rates were 30.0% (95% confidence interval [95% CI] 29.2–30.8) and 75.0% (68.0–82.0), respectively. In univariate analysis, only fertility-sparing treatment, ovarian preservation and surgical treatment had a significant impact on the PFI (hazard ratio [HR] 4.5, 3.1, and 0.2; 95% CI 1.5–13.1, 1.3–7.3, and 0.1–0.7; and p = 0.006, 0.009 and 0.006, respectively), but no factor was found to be associated with increased mortality risk. After adjusted with hormone treatment or chemotherapy, surgical treatment had significant effectiveness on OS (HR 0.3 and 0.3, 95% CI 0.1–1.0 and 0.1–1.0, p = 0.045 and 0.049, respectively). None of the patients with fertility-sparing treatment had successful conception, and all experienced repeated relapse. Conclusion For patients with recurrent LGESS, fertility-sparing treatment or ovarian preservation should not be provided. Surgery is the treatment of choice, and hormone treatment and/or chemotherapy was effective for the survival benefits of surgical treatment.


Author(s):  
Dolly Mehra ◽  
Anjum Saiyyed

Uterine sarcomas are a rare for of uterine cancers. They account for 0.2% of uterine cancers. The median age group is 40 to 60 years. They run an indolent course. About 60% women recur after a long period. Metastasis may occur even after 20 years. They can be classified into low grade, high grade and undifferentiated types. Low grade ESS has good prognosis. Surgery with adjuvant hormonal therapy is the mainstay of treatment. Adjuvant radiotherapy and chemotherapy have no role in management. The role of lymphadenectomy is not clear. The first line treatment for recurrence is a repeat surgery. Patients require a long term follow up to detect recurrence. Here we present a case of perimenopausal women presenting as a case of AUB. MRI initially diagnosed it as a case of fibroid. Patient underwent TAH with BSO. Subsequent hispathology and immunohistochemistry revealed it to be Low grade ESS. Although rare, endometrial stromal sarcoma should be considered as a differential diagnosis in perimenopausal and postmenopausal women presenting as AUB.


2018 ◽  
Vol 2018 ◽  
pp. 1-4
Author(s):  
Nisar A. Chowdri ◽  
Asif Mehraj ◽  
Fazl Q. Parray ◽  
Mudassir A. Khan ◽  
Masood A. Laharwal ◽  
...  

Endometrial stromal sarcomas of uterus are quite rare. Most of the recurrences in these tumors are seen in the pelvis. However, extrapelvic recurrences and metastases to other parts are quite unusual. Here, we are reporting a rare case of caecal recurrence of endometrial stromal sarcoma. Case Report. A 52-year-old female presented to us with pain and lump in the right lower abdomen. The patient was earlier subjected to total abdominal hysterectomy with bilateral salpingo-oophorectomy (TAHBSO) for low-grade endometrial stromal sarcoma. Postoperatively patient received radiotherapy but no hormone therapy. After 10 years of follow up patient presented with a polypoidal lesion in the caecum. Patient was evaluated fully and subjected to resection of this polypoidal lesion, which proved out to be high-grade endometrial stromal sarcoma. Conclusion. Recurrence of endometrial stromal sarcoma in the caecum is very rare. However, this entity needs to be kept in mind for differential diagnosis of a caecal mass. Recurrence in such cases may present quite late.


2022 ◽  
Vol 20 (1) ◽  
Author(s):  
You Wu ◽  
Nan Li ◽  
Rong Zhang ◽  
Ping Bai

Abstract Background This study aimed to analyze the clinical and pathological features of extrauterine endometrial stromal sarcoma (EESS) and explore an effective therapeutic regimen to reduce the recurrence rate in low-grade EESS patients. Methods Ten LG-EESS patients who were treated at the Chinese Academy of Medical Sciences Cancer Institute and Hospital from June 1999 to June 2019 were collected and analyzed. Results (1) Patient demographics are summarized in manuscript. Preoperative CA125 examination showed that 8 patients had a median level of 49.5 U/L (15.4–168.0 U/L). (2) All ten patients underwent tumor cytoreductive surgery. Five patients underwent optimal tumor resection and achieved an R0 resection. After the initial surgery, 7 patients who had multiple metastasis were treated with adjuvant chemotherapy, 2 patients with vaginal ESS were treated with chemotherapy and radiation therapy, and 6 patients with ER/PR positive received hormone therapy with or without chemotherapy. (2) Most EESS patients had multiple tumors. The omentum was the most commonly affected site, followed by the ovaries. (3) The median follow-up was 94 (range: 27–228) months, and recurrence was observed in 3 patients (n = 10, 30%) who underwent non-optimal surgery and no hormone therapy. The 5-year and 10-year DFS rates were both 70%, as shown in Fig. 2. OS was both 100% at 5 and 10 years. Conclusion As a conclusion, EESS is a rare disease and LG-EESS has a good prognosis. Surgery remains the available treatment for patients. LG-EESS has a risk of late recurrence which requires a long-term follow-up. With a limited sample size, our study shows optimal tumor reductive surgery and adjuvant hormone therapy may significantly reduce the risk of recurrence.


2017 ◽  
Vol 1 (2) ◽  
Author(s):  
Victor E Valdespino ◽  
Hilda Mendoza Ramón ◽  
German Maytorena Cordova ◽  
Victor Valdespino Gomez ◽  
Cynthia Lizarraga Mendez

2021 ◽  
Author(s):  
You Wu ◽  
Nan Li ◽  
Rong Zhang ◽  
Ping Bai

Abstract Background This study aimed to analyze the clinical and pathological features of extrauterine endometrial stromal sarcoma (EESS) and explore an effective therapeutic regimen to reduce the recurrence rate in low-grade EESS patients. Methods Ten LG-EESS patients who were treated at the Chinese Academy of Medical Sciences Cancer Institute and Hospital from June 1999 to June 2019 were collected and analyzed. Results (1) Patient demographics is summarized in Table 1. Preoperative CA125 examination showed that 8 patients had a median level of 49.5 U/L (15.4–168.0 U/L). (2)All ten patients underwent tumor cytoreductive surgery. Five patients underwent optimal tumor resection, achieved a R0 resection. After the initial surgery ,two patients were treated with adjuvant chemotherapy only, two patients were treated with chemotherapy and radiation therapy, and three patients were treated with chemotherapy plus hormonal treatment.(2) Most EESS patients had multiple tumors. the omentum was the most commonly affected site, followed by ovaries.(3) The median follow-up was 94 (range: 27–228) months, and recurrence was observed in 3 patients (n = 10, 30%). The 5-year and 10-year DFS rates were both 70%, as shown in Fig. 1. OS was both 100% at 5 and 10 years. Conclusion As a conclusion, EESS is a rare disease and LG-EESS has a good prognosis. Surgery remains the available treatment for patients. LG-EESS has a risk of late recurrence which requires a long-term follow-up. With a limited sample size, our study shows optimal tumor reductive surgery and adjuvant hormone therapy can significantly reduce the risk of recurrence.


Author(s):  
Salma Bhat ◽  
Ambreen Beigh ◽  
Summyia Farooq

Background: Endometrial stromal sarcomas (ESSs) are rare malignant uterine tumours comparatively affecting younger women and the mean age is 42 to 58 years. The World Health Organization (WHO) classification categorises endometrial stromal neoplasms and related tumors as: endometrial stromal nodule (ESN), low-grade endometrial stromal sarcoma (LG-ESS), high-grade endometrial stromal sarcoma (HG-ESS), and undifferentiated uterine sarcoma (UUS).Methods: Present study is a retrospective one and includes 6 patients with histologically proven endometrial stromal sarcoma for a period of 3 years. Authors examined every slide available from each case and new HE-stained slides generated from formaline-fixed, paraffin-embedded tissue were reviewed to confirm the diagnoses. Demographic information, pathologic, and treatment information were collected from the clinic and hospital charts. All had primary surgical management in the form of total abdominal hysterectomy and salpingo-oophorectomy.Results: The mean patient age was 41 years. All of the patients had presented with abnormal uterine bleeding. Diffuse growth of small cells closely resembling those of the normal proliferative endometrial stroma was the characteristic feature of these tumors. All of these patients had a low grade ESS on histopathology. They had regular follow-up visits until the end of study.Conclusions: Endometrial stromal sarcomas are rare malignant tumors of the uterus and a proper preoperative diagnosis is difficult. Their differential diagnosis from typical submucosal uterine myomas or benign endometrial polyps can be difficult. The histological examination of the specimen is necessary to exclude malignancy and establish the final diagnosis.


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