Uterine Cancer pp 351-363 | Cite as

Surgical Management of Uterine Sarcoma

  • Amita Maheshwari


Uterine sarcomas are rare malignant tumors of mesenchymal origin and account for 3–8 % of all uterine cancers [1, 2]. They are heterogeneous tumors of diverse histological types. The three most common subtypes are carcinosarcoma (CS) also known as malignant mixed Mullerian tumor (MMMT), leiomyosarcoma (LMS), and endometrial stromal sarcoma (ESS). Although recently carcinosarcoma has been reclassified as a dedifferentiated or metaplastic form of endometrial carcinoma [3], it is still included in most studies on uterine sarcomas. The important pure mesenchymal malignant tumors of the uterus are leiomyosarcoma (LMS) and endometrial stromal sarcoma (ESS). Uterine sarcomas are in general the most malignant group of uterine tumors and differ from endometrial cancers with respect to diagnosis, clinical behavior, pattern of spread, and management.


Common Subtype Uterine Sarcoma Endometrial Stromal Sarcoma Ovarian Metastasis Minimal Access Surgery 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. 1.
    Zaloudek C, Hendrickson M. Mesenchymal tumors of the uterus. In: Kurman RJ, editor. Blaustein’s pathology of the female genital tract. 5th ed. New York: Springer; 2002. p. 561–615.Google Scholar
  2. 2.
    Brooks SE, Zhan M, Cote T, Baquet CR. Surveillance, epidemiology, and end results analysis of 2677 cases of uterine sarcoma 1989–1999. Gynecol Oncol. 2004;93:204–8.CrossRefPubMedGoogle Scholar
  3. 3.
    Amant F, Moerman P, Neven P, Timmerman D, Van Limbergen E, Vergote I. Endometrial cancer. Lancet. 2005;366:491–505.CrossRefPubMedGoogle Scholar
  4. 4.
    El Husseiny G, Al Bareedy N, Mourad WA, et al. Prognostic factors and treatment modalities in uterine sarcoma. Am J Clin Oncol. 2002;25:256–60.CrossRefPubMedGoogle Scholar
  5. 5.
    Kapp D, Shin J, Chan J. Prognostic factors and survival in 1396 patients with uterine leiomyosarcomas. Emphasis on impact of lymphadenectomy and oophorectomy. Cancer. 2008;112:820–30.CrossRefPubMedGoogle Scholar
  6. 6.
    Bokhman JV, Yakovleva IA, Urmanchejeva AF. Treatment of patients with sarcoma of the uterus. Eur J Gynaecol Oncol. 1990;11:225–31.PubMedGoogle Scholar
  7. 7.
    Major FJ, Blessing JA, Silverberg SG, et al. Prognostic factors in early-stage uterine sarcoma: a Gynecologic Oncology Group study. Cancer. 1993;71(4 Suppl):702–9.Google Scholar
  8. 8.
    Gard GB, Mulvany NJ, Quinn MA. Management of uterine leiomyosarcoma in Australia. Aust N Z J Obstet Gynaecol. 1999;39:93–8.PubMedGoogle Scholar
  9. 9.
    Leitao MM, Sonoda Y, Brennan MF, et al. Incidence of lymph node and ovarian metastases in leiomyosarcoma of the uterus. Gynecol Oncol. 2003;91:209–12.CrossRefPubMedGoogle Scholar
  10. 10.
    Larson B, Silfversward C, Nilsson B, et al. Prognostic factors in uterine leiomyosarcoma. A clinical and histopathological study of 143 cases. The Radiumhemmet series 1936–1981. Acta Oncol. 1990;29:185–91.CrossRefPubMedGoogle Scholar
  11. 11.
    Giuntoli 2nd RL, 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;89:460–9.CrossRefPubMedGoogle Scholar
  12. 12.
    Gadducci A, Landoni F, Sartori E, et al. Uterine leiomyosarcoma: analysis of treatment failures and survival. Gynecol Oncol. 1996;62:25–32.CrossRefPubMedGoogle Scholar
  13. 13.
    Wu TI, Chang TC, Hsueh S, et al. Prognostic factors and impact of adjuvant chemotherapy for uterine leiomyosarcoma. Gynecol Oncol. 2006;100:166–72.CrossRefPubMedGoogle Scholar
  14. 14.
    Garg G, Shah JP, Liu JR, Bryant CS, Kumar S, Munkarah A. Morris RT validation of tumor size as staging variable in the revised International Federation of Gynecology and Obstetrics stage I leiomyosarcoma: a population-based study. Int J Gynecol Cancer. 2010;20(7):1201–6.CrossRefPubMedGoogle Scholar
  15. 15.
    Goff BA, Rice LW, Fleischhacker D, Muntz HG, Falkenberry SS, Nikrui N, Fuller Jr AF. Uterine leiomyosarcoma and endometrial stromal sarcoma: lymph node metastases and sites of recurrence. Gynecol Oncol. 1993;50(1):105–9.CrossRefPubMedGoogle Scholar
  16. 16.
    Ayhan A, Tuncer ZS, Tanir M, et al. Uterine sarcoma: the Hacettepe hospital experience of 88 consecutive patients. Eur J Gynaecol Oncol. 1997;18:146–8.PubMedGoogle Scholar
  17. 17.
    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;134:1277–87.CrossRefPubMedGoogle Scholar
  18. 18.
    Koivisto-Korander R, Butzow R, Koivisto AM, et al. Clinical outcome and prognostic factors in 100 cases of uterine sarcoma: experience in Helsinki University Central Hospital 1990–2001. Gynecol Oncol. 2008;111:74–81.CrossRefPubMedGoogle Scholar
  19. 19.
    Hoellen F, Waldmann A, Benthin S, Hanker L, Rody A, Fischer D. The role of lymphadenectomy in uterine sarcoma: a clinical practical approach based on retrospective analysis. Anticancer Res. 2014;34(2):985–93.PubMedGoogle Scholar
  20. 20.
    Leitao Jr MM, Zivanovic O, Chi DS, Hensley ML, O’Cearbhaill R, Soslow RA, Barakat RR. Surgical cytoreduction in patients with metastatic uterine leiomyosarcoma at the time of initial diagnosis. Gynecol Oncol. 2012;125(2):409–13.CrossRefPubMedGoogle Scholar
  21. 21.
    Levenback C, Rubin SC, McCormack PM, et al. Resection of pulmonary metastases from uterine sarcomas. Gynecol Oncol. 1992;45:202–5.CrossRefPubMedGoogle Scholar
  22. 22.
    Leitao MM, Brennan MF, Hensley M, et al. Surgical resection of pulmonary and extrapulmonary recurrences of uterine leiomyosarcoma. Gynecol Oncol. 2002;87:287–94.CrossRefPubMedGoogle Scholar
  23. 23.
    Abeler VM, Royne O, Thoresen S, et al. Uterine sarcomas in Norway. A histopathological and prognostic survey of a total population from 1970 to 2000 including 419 patients. Histopathology. 2009;54:355–64.CrossRefPubMedGoogle Scholar
  24. 24.
    Chan JK, Kawar NM, Shin JY, et al. Endometrial stromal sarcoma: a population-based analysis. Br J Cancer. 2008;99:1210–5.PubMedCentralCrossRefPubMedGoogle Scholar
  25. 25.
    Hendrickson MR, Tavassoli FA, Kempson RL, et al. Mesenchymal tumours and related lesions. In: Tavassoli FA, Deville P, editors. Pathology and genetics of tumours of the breast and female organs. Lyon: IARC Press; 2003. p. 233–44.Google Scholar
  26. 26.
    Xue WC, Cheung AN. Endometrial stromal sarcoma of uterus. Best Pract Res Clin Obstet Gynaecol. 2011;25(6):719–32.CrossRefPubMedGoogle Scholar
  27. 27.
    Sagae S, Yamashita K, Ishioka S, et al. Preoperative diagnosis and treatment results in 106 patients with uterine sarcoma in Hokkaido. Jpn Oncol. 2004;67:33–9.CrossRefGoogle Scholar
  28. 28.
    Dos Santos LA, Garg K, Diaz JP, et al. Incidence of lymph node and adnexal metastasis in endometrial stromal sarcoma. Gynecol Oncol. 2011;121:319–22.CrossRefPubMedGoogle Scholar
  29. 29.
    Li N, Wu LY, Zhang HT, et al. Treatment options in stage I endometrial stromal sarcoma: a retrospective analysis of 53 cases. Gynecol Oncol. 2008;108:306–11.CrossRefPubMedGoogle Scholar
  30. 30.
    Berchuck A, Rubin SC, Hoskins WJ, et al. Treatment of endometrial stromal tumors. Gynecol Oncol. 1990;36:60–5.CrossRefPubMedGoogle Scholar
  31. 31.
    Chu MC, Mor G, Lim C, et al. Low-grade endometrial stromal sarcoma: hormonal aspects. Gynecol Oncol. 2003;90:170–6.CrossRefPubMedGoogle Scholar
  32. 32.
    Li AJ, Giuntoli 2nd RL, Drake R, et al. Ovarian preservation in stage I low-grade endometrial stromal sarcomas. Obstet Gynecol. 2005;106:1304–8.CrossRefPubMedGoogle Scholar
  33. 33.
    Amant F, De Knijf A, Van Calster B, et al. Clinical study investigating the role of lymphadenectomy, surgical castration and adjuvant hormonal treatment in endometrial stromal sarcoma. Br J Cancer. 2007;97:1194–9.PubMedCentralCrossRefPubMedGoogle Scholar
  34. 34.
    Shah JP, Bryant CS, Kumar S, et al. Lymphadenectomy and ovarian preservation in low-grade endometrial stromal sarcoma. Obstet Gynecol. 2008;112:1102–8.CrossRefPubMedGoogle Scholar
  35. 35.
    Kim WY, Lee JW, Choi CH, et al. Low-grade endometrial stromal sarcoma: a single center’s experience with 22 cases. Int J Gynecol Cancer. 2008;18:1084–9.CrossRefPubMedGoogle Scholar
  36. 36.
    Bai H, Yang J, Cao D, Huang H, Xiang Y, Wu M, Cui Q, Chen J, Lang J, Shen K. Ovary and uterus-sparing procedures for low-grade endometrial stromal sarcoma: a retrospective study of 153 cases. Gynecol Oncol. 2014;132(3):654–60.CrossRefPubMedGoogle Scholar
  37. 37.
    Yoon A, Park JY, Park JY, Lee YY, Kim TJ, Choi CH, Bae DS, Kim BG, Lee JW, Nam JH. Prognostic factors and outcomes in endometrial stromal sarcoma with the 2009 FIGO staging system: a multicenter review of 114 cases. Gynecol Oncol. 2014;132(1):70–5.CrossRefPubMedGoogle Scholar
  38. 38.
    Feng W, Hua K, Malpica A, Zhou X, Baak JP. Stages I to II WHO 2003-defined low-grade endometrial stromal sarcoma: how much primary therapy is needed and how little is enough? Int J Gynecol Cancer. 2013;23(3):488–93.CrossRefPubMedGoogle Scholar
  39. 39.
    Gadducci A, Sartori E, Landoni F, Zola P, Maggino T, Urgesi A, Lissoni A, Losa G, Fanucchi A. Endometrial stromal sarcoma: analysis of treatment failures and survival. Gynecol Oncol. 1996;63(2):247–53.CrossRefPubMedGoogle Scholar
  40. 40.
    Riopel J, Plante M, Renaud MC, et al. Lymph node metastases in low-grade endometrial stromal sarcoma. Gynecol Oncol. 2005;96:402–6.CrossRefPubMedGoogle Scholar
  41. 41.
    Reich O, Winter R, Regauer S. Should lymphadenectomy be performed in patients with endometrial stromal sarcoma? Gynecol Oncol. 2005;97:982.CrossRefPubMedGoogle Scholar
  42. 42.
    Leath 3rd CA, Huh WK, Hyde Jr J, et al. A multi-institutional review of outcomes of endometrial stromal sarcoma. Gynecol Oncol. 2007;105:630–4.CrossRefPubMedGoogle Scholar
  43. 43.
    Signorelli M, Fruscio R, Dell’Anna T, et al. Lymphadenectomy in uterine low-grade endometrial stromal sarcoma: an analysis of 19 cases and a literature review. Int J Gynecol Cancer. 2010;20:1363–6.PubMedGoogle Scholar
  44. 44.
    Dong R, Pang Y, Mao H, Yang N, Liu P. Successful pregnancy following conservative management of low-grade endometrial stromal sarcoma: a case report. Oncol Lett. 2014;7(4):1039–42.PubMedCentralPubMedGoogle Scholar
  45. 45.
    Yan L, Tian Y, Fu Y, Zhao X. Successful pregnancy after fertility-preserving surgery for endometrial stromal sarcoma. Fertil Steril. 2010;93:269.e1–3.Google Scholar
  46. 46.
    Delaney AA, Gubbels AL, Remmenga S, Tomich P, Molpus K. Successful pregnancy after fertility-sparing local resection and uterine reconstruction for low-grade endometrial stromal sarcoma. Obstet Gynecol. 2012;120:486–9.CrossRefPubMedGoogle Scholar
  47. 47.
    Koskas M, Morice P, Yazbeck C, Duvillard P, Walker F, Madelenat P. Conservative management of low-grade endometrial stromal sarcoma followed by pregnancy and severe recurrence. Anticancer Res. 2009;29:4147–50.PubMedGoogle Scholar
  48. 48.
    Morimoto A, Tsubamoto H, Inoue K, Ikeda Y, Hirota S. Fatal case of multiple recurrences of endometrial stromal sarcoma after fertility-sparing management. J Obstet Gynaecol Res. 27 Aug 2014. doi: 10.1111/jog.12506. [Epub ahead of print].
  49. 49.
    Amant F, Coosemans A, Debiec-Rychter M, Timmerman D, Vergote I. Clinical management of uterine sarcomas. Lancet Oncol. 2009;10:1188–98.CrossRefPubMedGoogle Scholar
  50. 50.
    Takamizawa S, Minakami H, Usui R, et al. Risk of complications and uterine malignancies in women undergoing hysterectomy for presumed benign leiomyomas. Gynecol Obstet Invest. 1999;48:193–6.CrossRefPubMedGoogle Scholar
  51. 51.
    Leibsohn S, d’Ablaing G, Mishell Jr DR, Schlaerth JB. Leiomyosarcoma in a series of hysterectomies performed for presumed uterine leiomyomas. Am J Obstet Gynecol. 1990;162:968–74.CrossRefPubMedGoogle Scholar
  52. 52.
    FDA safety communication: laparoscopic uterine morcellation in hysterectomy and myomectomy. 2014.
  53. 53.
    Rekha W, Amita M, Sudeep G, Hemant T. Unexpected complication of uterine myoma morcellation. Aust N Z J Obstet Gynaecol. 2005;45(3):248–9.CrossRefPubMedGoogle Scholar
  54. 54.
    Morice P, Rodriguez A, Rey A, et al. Prognostic value of initial surgical procedure for patients with uterine sarcoma: analysis of 123 patients. Eur J Gynaecol Oncol. 2003;24:237–40.PubMedGoogle Scholar
  55. 55.
    Park JY, Park SK, Kim DY, et al. The impact of tumor Morcellation during surgery on the prognosis of patients with apparently early uterine leiomyosarcoma. Gynecol Oncol. 2011;122:255–9.CrossRefPubMedGoogle Scholar
  56. 56.
    Perri T, Korach J, Sadetzki S, Oberman B, Fridman E, Ben-Baruch G. Uterine leiomyosarcoma: does the primary surgical procedure matter? Int J Gynecol Cancer. 2009;19:257–60.CrossRefPubMedGoogle Scholar
  57. 57.
    George S, Barysauskas C, Serrano C, Oduyebo T, Rauh-Hain JA, Del Carmen MG, Demetri GD, Muto MG. Retrospective cohort study evaluating the impact of intraperitoneal morcellation on outcomes of localized uterine leiomyosarcoma. Cancer. 2014. doi: 10.1002/cncr.28844.Google Scholar
  58. 58.
    Seidman MA, Oduyebo T, Muto MG, Crum CP, Nucci MR, Quade BJ. Peritoneal dissemination complicating morcellation of uterine mesenchymal neoplasms [serial online]. PLoS One. 2012;7:e50058.PubMedCentralCrossRefPubMedGoogle Scholar
  59. 59.
    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;18:1065–70.CrossRefPubMedGoogle Scholar
  60. 60.
    Park JY, Kim DY, Kim JH, Kim YM, Kim YT, Nam JH. The impact of tumor morcellation during surgery on the outcomes of patients with apparently early low-grade endometrial stromal sarcoma of the uterus. Ann Surg Oncol. 2011;18(12):3453–61.CrossRefPubMedGoogle Scholar
  61. 61.
    Oduyebo T, Rauh-Hain AJ, Meserve EE, Seidman MA, Hinchcliff E, George S, Quade B, Nucci MR, Del Carmen MG, Muto MG. The value of re-exploration in patients with inadvertently morcellated uterine sarcoma. Gynecol Oncol. 2014;132(2):360–5.CrossRefPubMedGoogle Scholar
  62. 62.
    Galaal K, Kew FM, Tam KF, et al. Evaluation of prognostic factors and treatment outcomes in uterine carcinosarcoma. Eur J Obstet Gynecol Reprod Biol. 2009;143:88–92.CrossRefPubMedGoogle Scholar
  63. 63.
    Inthasorn P, Carter J, Valmadre S. Analysis of clinicopathologic factors in malignant mixed Mullerian tumors of the uterine corpus. Int J Gynecol Cancer. 2002;12:348–53.CrossRefPubMedGoogle Scholar
  64. 64.
    Sartori E, Bazzurini L, Gadducci A, et al. Carcinosarcoma of the uterus: a clinicopathological multicenter CTF study. Gynecol Oncol. 1997;67:70–5.CrossRefPubMedGoogle Scholar
  65. 65.
    Kokawa K, Nishiyama K, Ikeuchi M, et al. Clinical outcomes of uterine sarcomas: results from 14 years worth of experience in the Kinki district in Japan (1990–2003). Int J Gynecol Cancer. 2006;16:1358–63.CrossRefPubMedGoogle Scholar
  66. 66.
    Yamada SD, Burger RA, Brewster WR, et al. Pathologic variables and adjuvant therapy as predictors of recurrence and survival for patients with surgically evaluated carcinosarcoma of the uterus. Cancer. 2000;88:2782–6.CrossRefPubMedGoogle Scholar
  67. 67.
    Callister M, Ramondetta LM, Jhingran A, et al. Malignant mixed Mullerian tumors of the uterus: analysis of patterns of failure, prognostic factors, and treatment outcome. Int J Radiat Oncol Biol Phys. 2004;58:786–96.CrossRefPubMedGoogle Scholar
  68. 68.
    Nemani D, Mitra N, Guo M, et al. Assessing the effects of lymphadenectomy and radiation therapy in patients with uterine carcinosarcoma: a SEER analysis. Gynecol Oncol. 2008;111:82–8.CrossRefPubMedGoogle Scholar
  69. 69.
    Park JY, Kim DY, Kim JH, et al. The role of pelvic and/or para-aortic lymphadenectomy in surgical management of apparently early carcinosarcoma of uterus. Ann Surg Oncol. 2010;17:861–8.CrossRefPubMedGoogle Scholar
  70. 70.
    Garg G, Kruger M, Christensen C, Deppe G, Toy EP. Stage III uterine carcinosarcoma: 2009 International Federation of Gynecology and Obstetrics staging system and prognostic determinants. Int J Gynecol Cancer. 2011;21(9):1606–12.CrossRefPubMedGoogle Scholar
  71. 71.
    Geszler G, Szpak CA, et al. Prognostic value of peritoneal washings in patients with malignant mixed mullerian tumors of the uterus. Am J Obstet Gynecol. 1986;155:83–9.CrossRefPubMedGoogle Scholar
  72. 72.
    Barakat RR, Markman M, Randall M. Principles and practice of gynecologic oncology. 5th ed. Philadelphia: Wolters Kluwer Health/Lippincott, Williams & Wilkins; 2009.Google Scholar
  73. 73.
    Giuntoli 2nd RL, Garrett-Mayer E, Bristow RE, et al. Secondary cytoreduction in the management of recurrent uterine leiomyosarcoma. Gynecol Oncol. 2007;106:82–8.CrossRefPubMedGoogle Scholar
  74. 74.
    Korets SB, Curtin JP. Surgical options for recurrent uterine sarcomas. Am Soc Clin Oncol Educ Book. 2012:362–6.Google Scholar

Copyright information

© Springer India 2015

Authors and Affiliations

  1. 1.Gynecologic OncologyTata Memorial HospitalMumbaiIndia

Personalised recommendations