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Treatment and prognosis of uterine leiomyosarcoma

Nadeem R Abu-Rustum, MD
Martee L Hensley, MD, MSc
Section Editor
Don S Dizon, MD, FACP
Deputy Editors
Sadhna R Vora, MD
Sandy J Falk, MD, FACOG


Uterine leiomyosarcoma (LMS) is a rare uterine malignancy that arises from the smooth muscle of the uterine wall. Compared with other types of uterine cancers, LMS is an aggressive tumor associated with a high risk of recurrence and death, regardless of stage at presentation [1]. Tumor grade and other histologic features can influence clinical behavior of the tumors and may be important determinants of treatment recommendations. Whenever possible, women with uterine sarcomas should be referred to specialty centers with expertise in their diagnosis and management.  

This topic will cover the treatment approach to high-grade uterine LMS. The classification, clinical manifestations, diagnosis, and staging of other uterine sarcomas are covered separately. Treatment of other types of uterine sarcomas is also covered separately.

(See "Uterine sarcoma: Classification, clinical manifestations, and diagnosis".)

(See "Classification and treatment of endometrial stromal sarcoma and uterine adenosarcoma".)

(See "Clinical features, diagnosis, staging, and treatment of uterine carcinosarcoma".)


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Literature review current through: Sep 2016. | This topic last updated: Feb 19, 2016.
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  1. D'Angelo E, Prat J. Uterine sarcomas: a review. Gynecol Oncol 2010; 116:131.
  2. Giuntoli RL 2nd, Metzinger DS, DiMarco CS, et al. Retrospective review of 208 patients with leiomyosarcoma of the uterus: prognostic indicators, surgical management, and adjuvant therapy. Gynecol Oncol 2003; 89:460.
  3. Sagae S, Yamashita K, Ishioka S, et al. Preoperative diagnosis and treatment results in 106 patients with uterine sarcoma in Hokkaido, Japan. Oncology 2004; 67:33.
  4. Nassar OA, Abdul Moaty SB, Khalil el-SA, et al. Outcome and prognostic factors of uterine sarcoma in 59 patients: single institutional results. J Egypt Natl Canc Inst 2010; 22:113.
  5. Park JY, Kim DY, Suh DS, et al. Prognostic factors and treatment outcomes of patients with uterine sarcoma: analysis of 127 patients at a single institution, 1989-2007. J Cancer Res Clin Oncol 2008; 134:1277.
  6. Dinh TA, Oliva EA, Fuller AF Jr, et al. The treatment of uterine leiomyosarcoma. Results from a 10-year experience (1990-1999) at the Massachusetts General Hospital. Gynecol Oncol 2004; 92:648.
  7. Einstein MH, Barakat RR, Chi DS, et al. Management of uterine malignancy found incidentally after supracervical hysterectomy or uterine morcellation for presumed benign disease. Int J Gynecol Cancer 2008; 18:1065.
  8. Oduyebo T, Rauh-Hain AJ, Meserve EE, et al. The value of re-exploration in patients with inadvertently morcellated uterine sarcoma. Gynecol Oncol 2014; 132:360.
  9. Serrano C, Oduyebo T, Manola J, et al. Impact of tumor morcellation on the natural history of uterine leiomyosarcoma. Connective Tissue Oncology Society 2013. Poster 100. http://www.ctos.org/2013%20CTOS%20AM%20FP%20for%20Web.pdf (Accessed on May 21, 2014).
  10. http://www.fda.gov/medicaldevices/safety/alertsandnotices/ucm393576.htm (Accessed on May 21, 2014).
  11. Goff BA, Rice LW, Fleischhacker D, et al. Uterine leiomyosarcoma and endometrial stromal sarcoma: lymph node metastases and sites of recurrence. Gynecol Oncol 1993; 50:105.
  12. Leitao MM, Sonoda Y, Brennan MF, et al. Incidence of lymph node and ovarian metastases in leiomyosarcoma of the uterus. Gynecol Oncol 2003; 91:209.
  13. Major FJ, Blessing JA, Silverberg SG, et al. Prognostic factors in early-stage uterine sarcoma. A Gynecologic Oncology Group study. Cancer 1993; 71:1702.
  14. Kapp DS, Shin JY, Chan JK. Prognostic factors and survival in 1396 patients with uterine leiomyosarcomas: emphasis on impact of lymphadenectomy and oophorectomy. Cancer 2008; 112:820.
  15. Sandruck J, Escobar P, Lurain J, Fishman D. Uterine leiomyosarcoma metastatic to the sphenoid sinus: a case report and review of the literature. Gynecol Oncol 2004; 92:701.
  16. Engle DB, Smiley LM, Baum SL, Wellman GP. Positron Emission Tomography in detection of metastatic leiomyosarcoma in a postoperative patient: a case report. Proc Obstet Gynecol. 2011 July;2(1):Article 14[5 p]. ir.uiowa.edu/cgi/viewcontent.cgi?article=1111&context=pog (Accessed on August 15, 2012).
  17. Sung PL, Chen YJ, Liu RS, et al. Whole-body positron emission tomography with 18F-fluorodeoxyglucose is an effective method to detect extra-pelvic recurrence in uterine sarcomas. Eur J Gynaecol Oncol 2008; 29:246.
  18. Corrigendum to “FIGO staging for uterine sarcomas” [International Journal of Gynecology and Obstetrics (2009) 104:179]. Int J Gynaecol Obstet 2009; 106:277.
  19. Zivanovic O, Leitao MM, Iasonos A, et al. Stage-specific outcomes of patients with uterine leiomyosarcoma: a comparison of the international Federation of gynecology and obstetrics and american joint committee on cancer staging systems. J Clin Oncol 2009; 27:2066.
  20. Omura GA, Blessing JA, Major F, et al. A randomized clinical trial of adjuvant adriamycin in uterine sarcomas: a Gynecologic Oncology Group Study. J Clin Oncol 1985; 3:1240.
  21. Hensley ML, Ishill N, Soslow R, et al. Adjuvant gemcitabine plus docetaxel for completely resected stages I-IV high grade uterine leiomyosarcoma: Results of a prospective study. Gynecol Oncol 2009; 112:563.
  22. Hensley ML, Wathen R, Maki RG, et al. Adjuvant treatment of high-risk primary uterine leiomyosarcoma with gemcitabine/docetaxel (GT), followed by doxorubicin (D): Results of phase II multicenter trial SARC005 (abstract #10021). J Clin Oncol 2010; 28:15s.
  23. Reed NS, Mangioni C, Malmström H, et al. Phase III randomised study to evaluate the role of adjuvant pelvic radiotherapy in the treatment of uterine sarcomas stages I and II: an European Organisation for Research and Treatment of Cancer Gynaecological Cancer Group Study (protocol 55874). Eur J Cancer 2008; 44:808.
  24. Hensley ML, Wathen JK, Maki RG, et al. Adjuvant therapy for high-grade, uterus-limited leiomyosarcoma: results of a phase 2 trial (SARC 005). Cancer 2013; 119:1555.
  25. Pautier P, Floquet A, Berton-Rigaud D, Piperno-Neumann S. A randomized clinical trial of adjuvant chemotherapy with doxorubicin, ifosfamide, and cisplatin in localized uterine sarcomas: Results from 81 randomized patients (abstract 10022). J Clin Oncol 2011; S:10022.
  26. National Comprehensive Cancer Network (NCCN). NCCN Clinical practice guidelines in oncology. http://www.nccn.org/professionals/physician_gls/f_guidelines.asp (Accessed on February 27, 2016).
  27. Zivanovic O, Jacks LM, Iasonos A, et al. A nomogram to predict postresection 5-year overall survival for patients with uterine leiomyosarcoma. Cancer 2012; 118:660.
  28. Iasonos A, Keung EZ, Zivanovic O, et al. External validation of a prognostic nomogram for overall survival in women with uterine leiomyosarcoma. Cancer 2013; 119:1816.
  29. Leitao MM Jr, Hensley ML, Barakat RR, et al. Immunohistochemical expression of estrogen and progesterone receptors and outcomes in patients with newly diagnosed uterine leiomyosarcoma. Gynecol Oncol 2012; 124:558.
  30. Kelley TW, Borden EC, Goldblum JR. Estrogen and progesterone receptor expression in uterine and extrauterine leiomyosarcomas: an immunohistochemical study. Appl Immunohistochem Mol Morphol 2004; 12:338.
  31. Leitao MM, Soslow RA, Nonaka D, et al. Tissue microarray immunohistochemical expression of estrogen, progesterone, and androgen receptors in uterine leiomyomata and leiomyosarcoma. Cancer 2004; 101:1455.
  32. Anraku M, Yokoi K, Nakagawa K, et al. Pulmonary metastases from uterine malignancies: results of surgical resection in 133 patients. J Thorac Cardiovasc Surg 2004; 127:1107.
  33. Burt BM, Ocejo S, Mery CM, et al. Repeated and aggressive pulmonary resections for leiomyosarcoma metastases extends survival. Ann Thorac Surg 2011; 92:1202.
  34. Clavero JM, Deschamps C, Cassivi SD, et al. Gynecologic cancers: factors affecting survival after pulmonary metastasectomy. Ann Thorac Surg 2006; 81:2004.
  35. O'Cearbhaill RE, Maki RG, Zheng J, et al. Evaluation of the role of thermal ablation in the treatment of soft tissue sarcomas. 2009 Connective Tissue Oncology Society Meeting, Miami, FL. #39259.
  36. Moskovic E, MacSweeney E, Law M, Price A. Survival, patterns of spread and prognostic factors in uterine sarcoma: a study of 76 patients. Br J Radiol 1993; 66:1009.
  37. Kanjeekal S, Chambers A, Fung MF, Verma S. Systemic therapy for advanced uterine sarcoma: a systematic review of the literature. Gynecol Oncol 2005; 97:624.
  38. Maki RG, Wathen JK, Patel SR, et al. Randomized phase II study of gemcitabine and docetaxel compared with gemcitabine alone in patients with metastatic soft tissue sarcomas: results of sarcoma alliance for research through collaboration study 002 [corrected]. J Clin Oncol 2007; 25:2755.
  39. Hensley ML, Blessing JA, Mannel R, Rose PG. Fixed-dose rate gemcitabine plus docetaxel as first-line therapy for metastatic uterine leiomyosarcoma: a Gynecologic Oncology Group phase II trial. Gynecol Oncol 2008; 109:329.
  40. Hensley ML, Blessing JA, Degeest K, et al. Fixed-dose rate gemcitabine plus docetaxel as second-line therapy for metastatic uterine leiomyosarcoma: a Gynecologic Oncology Group phase II study. Gynecol Oncol 2008; 109:323.
  41. Hensley ML, Miller A, O'Malley DM, et al. A randomized phase III trial of gemcitabine + docetaxel + bevacizumab or placebo as first-line treatment for metastatic uterine leiomyosarcoma (uLMS): a Gynecologic Oncology Group study. Society of Gynecologic Oncology 45th Annual Meeting on Women's Cancer. March 2014. SP-2.
  42. Seddon B, Whelan J, Strauss SJ, Leahy MG. GeDDiS: A prospective randomised controlled phase III trial of gemcitabine and docetaxel compared with doxorubicin as first-line treatment in previously untreated advanced unresectable or metastatic soft tissue sarcomas (EudraCT 2009-014907-29). J Clin Oncol 2015; 33 (S1):abstract 10500.
  43. Muss HB, Bundy B, DiSaia PJ, et al. Treatment of recurrent or advanced uterine sarcoma. A randomized trial of doxorubicin versus doxorubicin and cyclophosphamide (a phase III trial of the Gynecologic Oncology Group). Cancer 1985; 55:1648.
  44. Sutton G, Blessing J, Hanjani P, et al. Phase II evaluation of liposomal doxorubicin (Doxil) in recurrent or advanced leiomyosarcoma of the uterus: a Gynecologic Oncology Group study. Gynecol Oncol 2005; 96:749.
  45. Look KY, Sandler A, Blessing JA, et al. Phase II trial of gemcitabine as second-line chemotherapy of uterine leiomyosarcoma: a Gynecologic Oncology Group (GOG) Study. Gynecol Oncol 2004; 92:644.
  46. Sutton GP, Blessing JA, Barrett RJ, McGehee R. Phase II trial of ifosfamide and mesna in leiomyosarcoma of the uterus: a Gynecologic Oncology Group study. Am J Obstet Gynecol 1992; 166:556.
  47. Sutton G, Blessing JA, Malfetano JH. Ifosfamide and doxorubicin in the treatment of advanced leiomyosarcomas of the uterus: a Gynecologic Oncology Group study. Gynecol Oncol 1996; 62:226.
  48. Demetri GD, von Mehren M, Jones RL, et al. Efficacy and Safety of Trabectedin or Dacarbazine for Metastatic Liposarcoma or Leiomyosarcoma After Failure of Conventional Chemotherapy: Results of a Phase III Randomized Multicenter Clinical Trial. J Clin Oncol 2016; 34:786.
  49. Monk BJ, Blessing JA, Street DG, et al. A phase II evaluation of trabectedin in the treatment of advanced, persistent, or recurrent uterine leiomyosarcoma: a gynecologic oncology group study. Gynecol Oncol 2012; 124:48.
  50. Sanfilippo R, Grosso F, Jones RL, et al. Trabectedin in advanced uterine leiomyosarcomas: a retrospective case series analysis from two reference centers. Gynecol Oncol 2011; 123:553.
  51. Pautier P, Floquet A, Chevreau C, et al. Trabectedin in combination with doxorubicin for first-line treatment of advanced uterine or soft-tissue leiomyosarcoma (LMS-02): a non-randomised, multicentre, phase 2 trial. Lancet Oncol 2015; 16:457.
  52. García-Del-Muro X, López-Pousa A, Maurel J, et al. Randomized phase II study comparing gemcitabine plus dacarbazine versus dacarbazine alone in patients with previously treated soft tissue sarcoma: a Spanish Group for Research on Sarcomas study. J Clin Oncol 2011; 29:2528.
  53. Anderson S, Aghajanian C. Temozolomide in uterine leiomyosarcomas. Gynecol Oncol 2005; 98:99.
  54. Garcia del Muro X, Lopez-Pousa A, Martin J, et al. A phase II trial of temozolomide as a 6-week, continuous, oral schedule in patients with advanced soft tissue sarcoma: a study by the Spanish Group for Research on Sarcomas. Cancer 2005; 104:1706.
  55. van der Graaf WT, Blay JY, Chawla SP, et al. Pazopanib for metastatic soft-tissue sarcoma (PALETTE): a randomised, double-blind, placebo-controlled phase 3 trial. Lancet 2012; 379:1879.
  56. Schöffski P, Chawla S, Maki RG, et al. Eribulin versus dacarbazine in previously treated patients with advanced liposarcoma or leiomyosarcoma: a randomised, open-label, multicentre, phase 3 trial. Lancet 2016; 387:1629.
  57. Ioffe YJ, Li AJ, Walsh CS, et al. Hormone receptor expression in uterine sarcomas: prognostic and therapeutic roles. Gynecol Oncol 2009; 115:466.
  58. Hardman MP, Roman JJ, Burnett AF, Santin AD. Metastatic uterine leiomyosarcoma regression using an aromatase inhibitor. Obstet Gynecol 2007; 110:518.
  59. O'Cearbhaill R, Zhou Q, Iasonos A, et al. Treatment of advanced uterine leiomyosarcoma with aromatase inhibitors. Gynecol Oncol 2010; 116:424.
  60. George S, Feng Y, Manola J, et al. Phase 2 trial of aromatase inhibition with letrozole in patients with uterine leiomyosarcomas expressing estrogen and/or progesterone receptors. Cancer 2014; 120:738.