Uterine sarcomas—Recent progress and future challenges

Uterine sarcomas are a rare heterogeneous group of tumors of mesenchymal origin, accounting for approximately 8% of uterine malignancies. They comprise leiomyosarcoma, endometrial stromal sarcoma, undifferentiated endometrial sarcoma, and adenosarcoma. Compared with the more common endometrial carcinomas, uterine sarcomas behave more aggressively and are associated with a poorer prognosis. Due to their distinct clinical and biological behavior, the International Federation of Gynecology and Obstetrics introduced a new staging system for uterine sarcomas in 2009, categorizing uterine carcinosarcoma as a variant of endometrial carcinoma, rather than a pure sarcoma. Magnetic resonance imaging (MRI) has a developing role in the assessment of these malignancies. Features such as tumor localization, irregular or nodular margins, necrosis, rapid growth, intense contrast enhancement, and restriction at diffusion-weighted imaging can suggest the diagnosis and help differentiate from more common leiomyomas and endometrial carcinoma. MRI is therefore extremely useful in preoperative detection and staging and, consequently, in determination of appropriate management. This pictorial review aims to discuss the clinical features of uterine sarcomas, as well as their most common appearances and distinct characteristics in MRI.

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“…Classically, they present as a rapidly growing pelvic mass, which may be accompanied by vaginal bleeding and abdominal or pelvic pain (1,5).…”

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confidence: 99%

Uterine sarcomas: clinical presentation and MRI features

Santos

Cunha²

2015

Diagn Interv Radiol

155

138

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“…9 More radical surgical approaches for ESS have produced acceptable results from available case reports and reviews of the literature and should be considered if substantial residual tumor removal can be achieved. 5,9 Some evidence suggests that adjuvant chemotherapy may reduce local recurrence with the use of anthracyclines and ifosfamide; however, tumor responsiveness is variable with no proven survival benefit. 2,9 Adjuvant hormonal therapy seems advantageous with most ESS tumors being steroid receptor-positive.…”

Section: Discussionmentioning

confidence: 94%

“…2,4,5,8,9 For locally advanced disease and advanced spread of ESS, cytoreductive surgery in the form of radical visceral resections and even isolated lung metastasectomy has been performed with acceptable survival from published reports. 2,5,9 Pul- monary metastectomy has become standard treatment for isolated lung metastasis and done so with curative intent in those with soft-tissue sarcomas. 9 More radical surgical approaches for ESS have produced acceptable results from available case reports and reviews of the literature and should be considered if substantial residual tumor removal can be achieved.…”

Section: Discussionmentioning

confidence: 98%

“…2,5,9 Pul- monary metastectomy has become standard treatment for isolated lung metastasis and done so with curative intent in those with soft-tissue sarcomas. 9 More radical surgical approaches for ESS have produced acceptable results from available case reports and reviews of the literature and should be considered if substantial residual tumor removal can be achieved. 5,9 Some evidence suggests that adjuvant chemotherapy may reduce local recurrence with the use of anthracyclines and ifosfamide; however, tumor responsiveness is variable with no proven survival benefit.…”

Section: Discussionmentioning

confidence: 99%

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Endometrial stromal sarcoma invading the abdominal aorta treated with aortic replacement

Busuito

West

Rasool

et al. 2012

Journal of Vascular Surgery

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Endometrial stromal sarcomas are extremely rare tumors with few reports describing invasion of major blood vessels. Tumors that primarily involve the abdominal aorta or invade the vessel secondarily are uncommon. Outcomes from radical tumor resection and aortic reconstruction are thus poorly understood. We present a rare case of a symptomatic recurrent endometrial stromal sarcoma with encasement of the infrarenal abdominal aorta requiring en bloc tumor resection and in situ aortic replacement.

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“…3,6,10,11 Although little is known about the factors responsible for the initial neoplastic myometrial transformation, it is hypothesized that each leiomyoma arises from a single cell in the myometrium. 1,14 Several observations suggest that estrogens and progesterone play an important role in the growth of uterine fibroids: they occur in women of reproductive age, often enlarge during pregnancy or during oral contraceptive use, and regress after menopause. 3,13,15 As fibroids enlarge, they may outgrow their blood supply, recently evaluated as possible marker of leiomyoma transformations.…”

Section: 11mentioning

confidence: 99%

Uterine leiomyosarcomas and leiomyomas: Two similar uterine solid tumors, totally different for prognosis

Tinelli

2011

JST

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The uterine leiomyosarcoma (LMS) is a rare sarcoma arising from the smooth muscle cells found within the myometrium, it is clinically aggressive smooth muscle malignancy, accounting for 2% to 6% of uterine malignancies and an annual incidence of 1.7 per 100,000 women. Although uterine malignancies such as endometrial cancer are common, uterine LMS accounts for only 1% of uterine cancers and is a part of uterine sarcomas' group, who comprises: carcinosarcoma (formerly known as MMMT, malignant mixed Mullerian tumor) (40% to 50%), leiomyosarcoma (30% to 40%) and endometrial stromal sarcoma (10% to 15%). Every group has its own risk factors, presenting symptoms, treatment response and prognosis.

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Uterine sarcomas—Recent progress and future challenges

Cited by 39 publications

References 150 publications

Uterine sarcomas: clinical presentation and MRI features

Uterine sarcomas: clinical presentation and MRI features

Endometrial stromal sarcoma invading the abdominal aorta treated with aortic replacement

Uterine leiomyosarcomas and leiomyomas: Two similar uterine solid tumors, totally different for prognosis

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