Survival outcomes and prognostic factors of endometrial stromal sarcoma and undifferentiated uterine sarcoma

Cabrera, Sílvia; Bebia, Vicente; Acosta, Úrsula; Franco-Camps, Sílvia; Mañalich, L.; García-Jiménez, Ángel

doi:10.1007/s12094-020-02512-6

Cited by 17 publications

(21 citation statements)

References 29 publications

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“…LG-ESS typically develops in premenopausal and perimenopausal women, with a mean age of 46 (range 18-83) years (11). Older age was thought to be an independent risk factor for OS in some studies (4,12). In our study, the median age was 44 years, ranging from 28 to 56 years.…”

Section: Discussionmentioning

confidence: 59%

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

Chen

Wang

Cao

et al. 2021

Preprint

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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.

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Section: Discussionmentioning

confidence: 59%

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

Chen

Wang

Cao

et al. 2021

Preprint

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“…UUS is an extremely rare and aggressive tumor with no specific line of differentiation that constitutes a diagnosis of exclusion [ 58 , 68 , 69 , 70 ]. The diagnosis should be performed after exclusion of other more common high-grade neoplasms (ULMS, carcinosarcoma, rhabdomyosarcoma, HGESS, LGESS, undifferentiated endometrial carcinoma and diffuse large B-cell lymphoma) ( Figure 8 ).…”

Section: Uterine Sarcomasmentioning

confidence: 99%

“…Approximately 60% of patients presented at advanced stage of the disease have a dismal prognosis (<2-year survival) [ 58 ]. UUS may arise in the endometrium or myometrium and is composed of pleomorphic or uniform cells with a high mitotic index that show no evidence of stromal or smooth muscle derivation [ 68 , 69 , 70 ]. Tumor necrosis, lymphovascular space involvement and destructive myometrial invasion are also presented [ 68 , 69 , 70 ].…”

Section: Uterine Sarcomasmentioning

confidence: 99%

“…UUS may arise in the endometrium or myometrium and is composed of pleomorphic or uniform cells with a high mitotic index that show no evidence of stromal or smooth muscle derivation [ 68 , 69 , 70 ]. Tumor necrosis, lymphovascular space involvement and destructive myometrial invasion are also presented [ 68 , 69 , 70 ]. Studies reported that 70% of UUS were in fact misdiagnosed HGESS [ 70 ].…”

Section: Uterine Sarcomasmentioning

confidence: 99%

“…Studies reported that 70% of UUS were in fact misdiagnosed HGESS [ 70 ]. Mitotic index appears to have prognostic significance in UUS, as a mitotic index higher than 25 mitoses per 10 high-power fields (HPFs) has a negative prognostic impact on survival [ 58 , 68 , 69 , 70 ]. Loss of SMARCA4 expression (mutation is often associated with remodeling ATPase in cancer) has recently been detected in a subset of UUS [ 70 ].…”

Section: Uterine Sarcomasmentioning

confidence: 99%

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New Aspects of Sarcomas of Uterine Corpus—A Brief Narrative Review

Kostov

Kornovski

Ivanova

et al. 2021

Clinics and Practice

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Sarcomas of the uterine corpus are rare malignant neoplasms, which are further classified into mesenchymal tumors, and mixed (epithelial plus mesenchymal) tumors. The main issues concerning these neoplasms are the small number of clinical trials, insufficient data from evidence-based medicine, insignificant interest from the pharmaceutical industry, all of which close a vicious circle. The low frequency of these malignancies implies insufficient experience in the diagnosis, hence incomplete surgical and complex treatment. Additionally, the rarity of these sarcomas makes it very difficult to develop clinical practice guidelines. Preoperative diagnosis, neoadjuvant and adjuvant chemoradiation, target and hormone therapies still raise many controversies. Disagreements about the role and type of surgical treatment are also often observed in medical literature. There are still insufficient data about the role of pelvic lymph node dissection and fertility-sparing surgery. Pathologists’ experience is of paramount importance for an accurate diagnosis. Additionally, genetics examinations become part of diagnosis in some sarcomas of the uterine corpus. Some gene mutations observed in uterine sarcomas are associated with different outcomes. Therefore, a development of molecular classification of uterine sarcomas should be considered in the future. In this review, we focus on the epidemiology, pathogenesis, pathology, diagnosis and treatment of the following sarcomas of the uterine corpus: leiomyosarcoma, low- and high-grade endometrial stromal sarcomas, undifferentiated sarcoma and adenosarcoma. Uterine carcinosarcomas are excluded as they represent an epithelial tumor rather than a true sarcoma.

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Low-grade endometrial stromal sarcoma with intracaval or intracardiac extension: a retrospective study of eight cases

Chen

Wang

Cao

et al. 2022

Arch Gynecol Obstet

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Survival outcomes and prognostic factors of endometrial stromal sarcoma and undifferentiated uterine sarcoma

Cited by 17 publications

References 29 publications

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

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

New Aspects of Sarcomas of Uterine Corpus—A Brief Narrative Review

Low-grade endometrial stromal sarcoma with intracaval or intracardiac extension: a retrospective study of eight cases

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