Outcome and Management of Uterine Leiomyosarcoma Treated Following Surgery for Presumed Benign Disease: Review of Literature

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Abstract

Uterine leiomyosarcoma (uLMS) is a rare and aggressive cancer, usually diagnosed incidentally at the time of myomectomy or hysterectomy. There have been concerns for several years about the fact that the inadvertent disruption of occult uLMS may have a negative impact on patient outcome. This study reviews the outcome and management of patients with a diagnosis of uLMS after surgery for presumed benign disease. We conducted a literature search in which 47 published English-language articles were obtained for evaluation. A total of 23 studies with outcomes data were included. It is evidenced that patients who underwent surgery with tumor disruption resulted in poorer outcomes compared with en bloc tumor, especially by power morcellation. The power morcellation was associated with an increased risk of recurrence, shorten time to recurrence, and upstage after re-exploration. Early re-exploration and surgical staging are appreciated for better prognosis and may alter postoperative treatment. We also updated on the incidence and preoperative evaluation to assess the risk of patient and give an effective counseling.

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Most cited references 47

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Uterine sarcomas: a review.

Emanuela D'Angelo, Jaime Prat (2010)

Uterine sarcomas are rare tumors that account for 3% of uterine cancers. Their histopathologic classification was revised by the World Health Organization (WHO) in 2003. A new staging system has been recently designed by the International Federation of Gynecology and Obstetrics (FIGO). Currently, there is no consensus on risk factors for adverse outcome. This review summarizes the available clinicopathological data on uterine sarcomas classified by the WHO diagnostic criteria. Medline was searched between 1976 and 2009 for all publications in English where the studied population included women diagnosed of uterine sarcomas. Since carcinosarcomas (malignant mixed mesodermal tumors or MMMT) are currently classified as metaplastic carcinomas, leiomyosarcomas remain the most common uterine sarcomas. Exclusion of several histologic variants of leiomyoma, as well as "smooth muscle tumors of uncertain malignant potential," frequently misdiagnosed as sarcomas, has made apparent that leiomyosarcomas are associated with poor prognosis even when seemingly confined to the uterus. Endometrial stromal sarcomas are indolent tumors associated with long-term survival. Undifferentiated endometrial sarcomas exhibiting nuclear pleomorphism behave more aggressively than tumors showing nuclear uniformity. Adenosarcomas have a favorable prognosis except for tumors showing myometrial invasion or sarcomatous overgrowth. Adenofibromas may represent well-differentiated adenosarcomas. The prognosis of carcinosarcomas (which are considered here in a post-script fashion) is usually worse than that of grade 3 endometrial carcinomas. Immunohistochemical expression of Ki67, p53, and p16 is significantly higher in leiomyosarcomas and undifferentiated endometrial sarcomas than in endometrial stromal sarcomas. Evaluation of H&E stained sections has been equivocal in the prediction of behavior of uterine sarcomas. Immunohistochemical studies of oncoproteins as well as molecular analysis of non-random translocations will undoubtedly lead to an accurate and prognostically relevant classification of these rare tumors.

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Clinical management of uterine sarcomas.

Frédéric Amant, An Coosemans, Maria Debiec-Rychter … (2009)

Malignant pure mesenchymal uterine tumours encompass endometrial stromal sarcoma (ESS), uterine leiomyosarcoma, and undifferentiated sarcomas. This Review discusses pathology, preoperative diagnosis, and standard treatment of uterine leiomyosarcoma and low-grade ESS (distinct from undifferentiated uterine sarcomas), with an emphasis on targeted treatment. We show that several features on ultrasonography and MRI can raise suspicion of a uterine sarcoma; however, there are no pathognomonic features on any imaging technique. For both ESS and uterine leiomyosarcoma, hysterectomy with bilateral salpingo-oophorectomy, but without lymphadenectomy, is the standard surgical treatment for early stage disease. The clinical benefit of chemotherapy is limited, which underscores the importance of targeted therapy. ESS and uterine leiomyosarcoma are driven by different pathways, resulting in a different clinical behaviour. ESS typically is a hormone-sensitive tumour with indolent growth. Uterine leiomyosarcoma is notorious for its aggressive growth and poor outcome. Individualisation of treatment is mandatory, because randomised trials are almost non-existent. The progesterone and oestrogen receptors are clinically important targets for most primarily advanced or recurrent ESS and a subset of recurrent uterine leiomyosarcomas. Potential future targets and targeted treatments that are under investigation are presented for both entities.

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The impact of tumor morcellation during surgery on the prognosis of patients with apparently early uterine leiomyosarcoma.

Jeong-Yeol Park, Sun-Kyung Park, Dae-Yeon Kim … (2011)

Uterine leiomyosarcoma (LMS) is usually diagnosed after surgery for leiomyoma; thus tumor morcellation frequently occurs. We evaluated the impact of tumor morcellation during surgery on the prognosis of patients with apparently early uterine LMS. Outcomes were retrospectively compared between patients who underwent total abdominal hysterectomy without tumor morcellation and those who underwent surgery that included abdominal, vaginal or laparoscopic tumor morcellation. We assessed 56 consecutive patients with stage I and II uterine LMS between 1989 and 2010, 25 with and 31 without tumor morcellation. There were no significant between group differences in age, parity, menopausal status, body mass index, stage, mitotic count, tumor grade, lymph node dissection, adjuvant therapy, and follow-up duration. However, tumor size was significantly smaller (9.8 cm vs. 7.3 cm, P=0.022) and ovarian tissue was more frequently preserved (38.7% vs. 72%, P=0.013) in patients with tumor morcellation. In univariate analysis, only tumor morcellation was significantly associated with poorer disease-free survival (DFS) (odds ratio [OR], 2.59; 95% confidence interval [CI], 1.03-6.50; P=0.043), and higher stage (I vs. II; (OR, 19.12; 95% CI, 1.19-307.11; P=0.037)) and tumor morcellation (OR, 3.07; 95% CI, 1.05-8.93; P=0.040) were significantly associated with poorer overall survival (OS). In multivariate analysis, higher stage (OR, 20.34; 95% CI, 1.27-325.58; P=0.033) and tumor morcellation (OR, 3.11; 95% CI, 1.07-9.06; P=0.038) were significantly associated with poorer OS. The percentage of patients with abdomino-pelvic dissemination, as shown by peritoneal sarcomatosis or vaginal apex recurrence, was significantly greater in patients with than without tumor morcellation (44% vs. 12.9%, P=0.032). Tumor morcellation during surgery increased the rate of abdomino-pelvic dissemination and adversely affected DFS and OS in patients with apparently early uterine LMS. Copyright © 2011 Elsevier Inc. All rights reserved.

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Author and article information

Journal

Journal ID (nlm-ta): Gynecol Minim Invasive Ther

Journal ID (iso-abbrev): Gynecol Minim Invasive Ther

Journal ID (publisher-id): GMIT

Title: Gynecology and Minimally Invasive Therapy

Publisher: Medknow Publications & Media Pvt Ltd (India )

ISSN (Print): 2213-3070

ISSN (Electronic): 2213-3089

Publication date (Print): Apr-Jun 2018

Publication date (Electronic): 02 May 2018

Volume: 7

Issue: 2

Pages: 47-55

Affiliations

[1 ]Department of Obstetrics and Gynecology, Faculty of Medicine, Srinakharinwirot University, Nakhonnayok, Thailand

[2 ]Department of Obstetrics and Gynecology, Linkou Chang Gung Memorial Hospital, Tao-Yuan, Taiwan

[3 ]Department of Obstetrics and Gynecology, Chang Gung University College of Medicine, Tao-Yuan, Taiwan

[4 ]Department of Obstetrics and Gynecology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand

Author notes

Address for correspondence: Dr. Chih-Feng Yen, No.5, Fuxing St., Guishan Dist., Taoyuan City 33305, Taiwan (R.O.C.). E-mail: yen2158@ 123456cgmh.org.tw

Article

Publisher ID: GMIT-7-47

DOI: 10.4103/GMIT.GMIT_10_18

PMC ID: 6113990

SO-VID: 5a3740e7-8454-4ef0-a1f3-355fbb6114e7

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Outcome and Management of Uterine Leiomyosarcoma Treated Following Surgery for Presumed Benign Disease: Review of Literature

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SICOT-J (Orthopedic surgery and traumatology)

Most cited references 47

Uterine sarcomas: a review.

Clinical management of uterine sarcomas.

The impact of tumor morcellation during surgery on the prognosis of patients with apparently early uterine leiomyosarcoma.

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