Localized high grade endometrial stromal sarcoma and localized undifferentiated uterine sarcoma: a retrospective series of the French Sarcoma Group

Meurer, Marie; Floquet, Anne; Ray‐Coquard, Isabelle; Bertucci, François; Auriche, Morgane; Cordoba, Abel; Piperno‐Neumann, Sophie; Salas, Sébastien; Delannes, M.; Chevalier, Thomas; Italiano, Antoîne; Blay, Jean‐Yves; Mancini, Julien; Pautier, Patricia; Duffaud, Florence

doi:10.1136/ijgc-2018-000064

Cited by 29 publications

(24 citation statements)

References 30 publications

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“…Recently, a retrospective, single-centre study evaluated prognostic factors in 40 patients affected by HG-ESS[56]; the combination of surgery with RT and ChT appeared to improve PFS in early-stage disease. These findings were confirmed by a retrospective analysis of the French Sarcoma Group in which multivariate analysis of adjuvant chemotherapy in completely resected HG-ESS and UUS was correlated with improved DFS[57]. Presently, with respect to the prospective studies which might validate adjuvant ChT in HG-ESS and UUS, current guidelines consider the use of postoperative ChT appropriate taking into account the high risk of recurrence characterising these diseases[6].…”

Section: Other Histotypesmentioning

confidence: 89%

Current status of the adjuvant therapy in uterine sarcoma: A literature review

Rizzo¹,

Pantaleo²,

Saponara³

et al. 2019

WJCC

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Uterine sarcomas (US) are rare mesenchymal tumours accounting approximately for 3%–7% of all uterine cancers. Histologically, US are classified into mesenchymal tumours or mixed epithelial and mesenchymal tumours. The group of mesenchymal tumours includes uterine leiomyosarcoma (uLMS, 65% of cases), endometrial stromal sarcoma (ESS, 21%) – traditionally divided into low grade (LG-ESS) and high grade–undifferentiated uterine sarcoma (5%) and other rare subtypes such as alveolar or embryonal rhabdomyosarcoma. Despite the fact that several drugs demonstrated clinical activity in advanced or metastatic settings, the role of postoperative therapy in US remains controversial. In this review, we have summarised the current state of the art, including the chief trials on adjuvant treatment modalities in US, especially focusing on uLMS, LG-ESS and other rare histotypes.

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Section: Other Histotypesmentioning

confidence: 89%

Current status of the adjuvant therapy in uterine sarcoma: A literature review

Rizzo¹,

Pantaleo²,

Saponara³

et al. 2019

WJCC

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“…The role of lymphadenectomy in the surgical management of ESS patients is not conclusive. Lymph node involvement among these patients was uncommon, and no differences were found either in recurrence-free survival or in overall survival after performing lymphadenectomy (19)(20)(21). In our cohort, all the patients underwent surgery without lymphadenectomy under the direction of gynecologic, vascular and cardiac surgeons.…”

Section: Discussionmentioning

confidence: 83%

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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“…One of the retrospective studies reported that adjuvant radiotherapy and chemotherapy appear to improve overall survival. [ 17 ]…”

Section: Discussionmentioning

confidence: 99%

A high-grade undifferentiated endometrial stromal sarcoma presenting as inversion of the uterus: A rare case

et al. 2020

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Endometrial stromal sarcoma (ESS) is a rare malignant tumor that constitutes about 0.2% of all uterine malignancies and 10% of uterine sarcomas. ESS is generally misdiagnosed as leiomyoma or endometrial polyp and typically discovered on histopathological examination postoperatively because of its rarity. Endometrial stromal tumors are composed of cells resembling normal endometrial stroma in its proliferative phase. The histologic diagnosis of the high grade is made if there is a high-grade sarcoma with a high mitotic index and nuclear anaplasia. The mean age of presentation of high-grade endometrial sarcoma is about 61 years with the most common presenting complaint is menorrhagia. The median overall survival for high-grade endometrial sarcoma is 53 months with optimal cytoreduction. A 49-year-old woman P2 L2 presented with nonspecific complaint of discharge and spotting per vaginum. In the present case, the provisional diagnosis by clinical findings as well as imaging was in favor of the inversion of submucous fibroid. Preoperative histopathological examination and immunohistochemistry confirmed the diagnosis of high-grade undifferentiated ESS. Haultain’s operation followed by total abdominal hysterectomy and bilateral salpingo-oophorectomy was performed. The patient was referred to another center for radiotherapy. From there, she was lost to follow-up. Rarity of endometrial stromal tumor limits the clinician view to diagnose it preoperatively. We were fortunate to have preoperative histopathological diagnosis of ESS. Furthermore, as ESS is rare and undifferentiated stromal sarcoma is even rarer, literature is lacking on its optimal management. Hence, it is important for all clinicians to keep the high degree of suspicion for ESS while working up any case of abnormal uterine bleeding.

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Localized high grade endometrial stromal sarcoma and localized undifferentiated uterine sarcoma: a retrospective series of the French Sarcoma Group

Cited by 29 publications

References 30 publications

Current status of the adjuvant therapy in uterine sarcoma: A literature review

Current status of the adjuvant therapy in uterine sarcoma: A literature review

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

A high-grade undifferentiated endometrial stromal sarcoma presenting as inversion of the uterus: A rare case

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