scholarly journals Primary low-grade extrauterine endometrial stromal sarcoma: analysis of 10 cases with a review of the literature

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.

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.


2015 ◽  
Vol 2015 ◽  
pp. 1-4
Author(s):  
Qiao Wang ◽  
Xia Zhao ◽  
Ping Han

Extrauterine endometrial stromal sarcoma (ESS) arising in endometriosis is extremely rare, particularly in the colorectum. It should always be included in the differential diagnosis of primary tumors originating from gastrointestinal tract in females, given that preoperative endoscopical biopsy may reveal no specific changes. We reported a case of ESS arising in colorectal endometriosis and reviewed the previous 7 cases reported in the English literature. Our patient, who was unavailable for tumor resection and refused further adjuvant therapy, played a role in representing the natural history of low-grade extragenital ESS. This case was the only death from ESS arising in colorectal endometriosis.


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.


2021 ◽  
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.


2016 ◽  
Vol 2016 ◽  
pp. 1-6
Author(s):  
Wataru Kudaka ◽  
Hitoshi Inafuku ◽  
Yuko Iraha ◽  
Tomoko Nakamoto ◽  
Yusuke Taira ◽  
...  

Background. A rare case of low-grade endometrial stromal sarcoma (LG-ESS) extending to inferior vena cava (IVC) and cardiac chambers.Case Report. A 40-year-old woman had IVC tumor, which was incidentally detected by abdominal ultrasonography during a routine medical checkup. CT scan revealed a tumor in IVC, right iliac and ovarian veins, which was derived from the uterus and extended into the right atrium and ventricle. The operation was performed, the heart and IVC were exposed, and cardiopulmonary bypass was initiated. A right atriotomy was performed, and the intracardiac mass was removed. Then the tumor in IVC and the right internal iliac vein were removed after longitudinal venotomies in the suprarenal and infrarenal vena cava, the right common iliac vein. Next the pelvis was explored. Tumors were found originating from the posterior wall of the uterus and continuing into both the right uterine and ovarian vein. The patient underwent total hysterectomy with bilateral salpingooophorectomy. Complete tumor resection was achieved. Histopathological analysis confirmed a diagnosis of LG-ESS. She showed no evidence of disease for 2 years and 3 months.Conclusions. Our case highlights the importance of a multidisciplinary approach in treating this rare cardiovascular pathological condition through preoperative assessment to final operation.


Rare Tumors ◽  
2020 ◽  
Vol 12 ◽  
pp. 203636132097286 ◽  
Author(s):  
Angela Duvalyan ◽  
Kirk Tran ◽  
Christopher Lee ◽  
Shefali Chopra ◽  
James Hu

Endometrial stromal sarcomas (ESS) account for 10-15% of uterine malignancies and are classified into four categories: endometrial stromal nodule (ESN), low-grade endometrial stromal sarcoma (LG-ESS), high-grade endometrial stromal sarcoma (HG-ESS) and undifferentiated uterine sarcoma (USS). Depending on stage, ESS is treated with surgery, hormone therapy, chemotherapy or targeted therapy. A rare complication of ESS with metastatic pulmonary disease is recurrent, bilateral pneumothoraces. The current literature has reported on patients with ESS who either presented with pneumothoraces at their initial diagnosis, and thus were untreated, or after treatment with surgical resection and hormone therapy. There have been no case reports of patients with ESS who presented with pneumothoraces while receiving chemotherapy. Furthermore, there have been no reported cases of patients with HG-ESS presenting with this rare complication. We would like to expand the literature by reporting on two patients with HG-ESS who presented with pneumothoraces while concurrently receiving chemotherapy.


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.


2019 ◽  
Vol 82 (5) ◽  
pp. 385-389 ◽  
Author(s):  
Gunsu Kimyon Comert ◽  
Osman Turkmen ◽  
Irem Kar ◽  
Ozge Yucel ◽  
Cigdem Kilic ◽  
...  

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.


2019 ◽  
Vol 82 (6) ◽  
pp. 445-446 ◽  
Author(s):  
Wen-Ling Lee ◽  
Fa-Kung Lee ◽  
Peng-Hui Wang

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