Uterine Cancer pp 339-350 | Cite as

Uterine Sarcomas: Risk Factors, Clinical Presentation, Diagnosis, and Staging

  • Monisha Gupta
  • Shalini Rajaram


Mesenchymal tumors of the uterine corpus are rare, accounting for approximately 7–8 % of all uterine cancers [1]. As per The American cancer society’s estimates for cancer of uterine corpus in the USA for 2014, about 52,630 new cases of cancer of the uterine corpus will be diagnosed, but only about 1,600 (3 %) of these cases will be uterine sarcomas [1, 2]. In stark contrast, a registry from a tertiary centre in north India has shown that uterine sarcomas constitutes roughly 25 % of all uterine malignancies [3]. There has also been a rise in the incidence of uterine malignancies in the Indian subcontinent in the past decade [4].


Endometrial Carcinoma Endometrial Adenocarcinoma Uterine Sarcoma Abnormal Uterine Bleeding Endometrial Stromal Sarcoma 
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.
    D’ Angelo E, Prat J. Uterine sarcomas: a review. Gynecol Oncol. 2010;116:131–9.CrossRefGoogle Scholar
  2. 2.
    Doss LL, Llorens AS, Henriquez EM. Carcinosarcoma of the uterus: a 40-year experience from the state of Missouri. Gynecol Oncol. 1984;18:43–53.CrossRefPubMedGoogle Scholar
  3. 3.
    Rajaram S, Malik R, Agarwal S, Suneja A. The role of hospital-based cancer registries in low and middle income countries-Indian statistics. Cancer Epidemiol. 2013;37(1):e7.CrossRefPubMedGoogle Scholar
  4. 4.
    Agarwal S, Malhotra KP, Sinha S, Rajaram S. Profile of gynecologic malignancies reported at a tertiary care center in India over the past decade: comparative evaluation with international data. Indian J Cancer. 2012;49(3):298–302.CrossRefPubMedGoogle Scholar
  5. 5.
    Trop É CG, Abeler MV. Diagnosis and treatment of sarcoma of the uterus. A review. Acta Oncol. 2012;51:694–705.CrossRefGoogle Scholar
  6. 6.
    Shah SH, Jagannathan JP, Krajewski K, O’Regan KN, George S, Ramaiya NH. Uterine sarcomas: then and now. Am J Roentgenol. 2012;199:213–23.CrossRefGoogle Scholar
  7. 7.
    McCluggage WG. Malignant biphasic uterine tumours: carcinosarcoma or metaplastic carcinomas? J Clin Pathol. 2002;55:321–5.PubMedCentralCrossRefPubMedGoogle Scholar
  8. 8.
    Zelmanowicz A, Hildesheim A, Sherman ME, et al. Evidence for a common etiology for endometrial carcinomas and malignant mixed mullerian tumors. Gynecol Oncol. 1998;69(3):253.CrossRefPubMedGoogle Scholar
  9. 9.
    Park J, Kim D, Suh D, 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
  10. 10.
    Abeler VM, R ø yne O, Thoresen S, Danielsen HE, Nesland JM, Kristensen GB. 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
  11. 11.
    Giuntoli 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.
    Toro JR, Travis LB, Wu HJ, Zhu K, Fletcher CD, Devesa SS. Incidence patterns of soft tissue sarcomas, regardless of primary site, in the surveillance, epidemiology and end results program, 1978–2001: an analysis of 26,758 cases. Int J Cancer. 2006;119:2922–30.CrossRefPubMedGoogle Scholar
  13. 13.
    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
  14. 14.
    Helman LJ, Meltzer P. Mechanisms of sarcoma development. Nat Rev Cancer. 2003;3:685–94.CrossRefPubMedGoogle Scholar
  15. 15.
    Hiroshi K, Chiaki U, Juria A, et al. The biology of uterine sarcomas: a review and update. Mol Clin Oncol. 2013;1:599–609.Google Scholar
  16. 16.
    Lee C, Ou W, Marino-Enriquez A, et al.14-3-3 fusion oncogenes in high grade endometrial stromal sarcoma. PNAS. 2012;109:929–34.Google Scholar
  17. 17.
    Hayashi T, Kobayashi Y, Kohsaka S, Sano K. The mutation in the ATP-binding region of JAK1, identified in human uterine leiomyosarcomas, results in defective interferon-gamma inducibility of TAP1 and LMP2. Oncogene. 2006;25:4016–26.CrossRefPubMedGoogle Scholar
  18. 18.
    Saegusa M, Hashimura M, Kuwata T, et al. Requirement of Akt/ b-catenin pathway for uterine carcinosarcoma genesis, modulating E-catherin expression through the transactivation of slug. Am J Pathol. 2009;174:2107–15.PubMedCentralCrossRefPubMedGoogle Scholar
  19. 19.
    Guntupali SR, Ramirez PT, Anderson ML, et al. Uterine smooth muscle tumor of uncertain malignant potential: a retrospective analysis. Gynecol Oncol. 2009;113:324–6.CrossRefGoogle Scholar
  20. 20.
    Christopherson WM, Williamson EO, Gray LA. Leiomyosarcoma of the uterus. Cancer. 1972;29:1512–7.CrossRefPubMedGoogle Scholar
  21. 21.
    Norris HJ, Roth E, Taylor HB. Mesenchymal tumors of the uterus. Obstet Gynecol. 1966;28:57–63.PubMedGoogle Scholar
  22. 22.
    Meredith RJ, Eisert DR, Kaka Z, et al. An excess of uterine sarcomas after pelvic irradiation. Cancer. 1986;58:2003–7.Google Scholar
  23. 23.
    Pothuri B, Ramondetta L, Eifel P, et al. Radiation associated endometrial cancers are prognostically unfavourable tumours: a clinicopathologic comparison with 527 sporadic endometrial cancers. Gynecol Oncol. 2006;103:948–51.CrossRefPubMedGoogle Scholar
  24. 24.
    Jaakkola S, Lyytinen HK, Pukkala E, et al. Use of estradiol-progestin therapy associates with increased risk for uterine sarcomas. Gynecol Oncol. 2011;122:260–3.CrossRefPubMedGoogle Scholar
  25. 25.
    Hoogendoorern WE, Hollema H, Van Boven HH, et al. Prognosis of uterine corpus cancer after tamoxifen treatment for breast cancer. Breast Cancer Res Treat. 2008;112:99–108.CrossRefGoogle Scholar
  26. 26.
    Nilbert M, Therkildsen C, Nissen A, et al. Sarcomas associated with hereditary nonpolyposis colorectal cancer: broad anatomical and morphological spectrum. Fam Cancer. 2009;8:209–13.CrossRefPubMedGoogle Scholar
  27. 27.
    Sandberg AA. Updates on the cytogenetics and molecular genetics of bone and soft tissue tumors: leiomyosarcoma. Cancer Genet Cytogenet. 2005;161:1–19.CrossRefPubMedGoogle Scholar
  28. 28.
    Dos Santos LA, Garg K, Diaz JP, et al. Incidence of lymph nodes and adnexal metastasis in endometrial stromal sarcoma. Gynecol Oncol. 2011;121:319–22.CrossRefPubMedGoogle Scholar
  29. 29.
    Bansal N, Herzog TJ, Burke W, Cohen CJ, Wright JD. The utility of preoperative endometrial sampling for the detection of endometrial sarcomas. Gynecol Oncol. 2008;110:43–8.CrossRefPubMedGoogle Scholar
  30. 30.
    Major FJ, Blessing JA, Silverberg SG, et al. Prognostic factors in early stage uterine sarcoma. Cancer. 1993;71:1702–9.CrossRefPubMedGoogle Scholar
  31. 31.
    Goto A, Takeuchi S, Sugimura K, et al. Usefulness of Gd-DTPA contrast enhanced dynamic MRI and serum determination of LDH and its isizymes in differential diagnosis of leiomyosarcoma from degenerated leiomyomaof the uterus. Int J Gynecol Cancer. 2002;12:354–61.CrossRefPubMedGoogle Scholar
  32. 32.
    Lim D, Wang WL, Lee CH, et al. Old versus new FIGO staging systems in predicting overall survival in patients with uterine leiomyosarcoma: a study of 86 cases. Gynecol Oncol. 2013;128:322–6.CrossRefPubMedGoogle Scholar
  33. 33.
    Zivanovic O, Jacks LM, Iasonos A, Leitao Jr MM, Soslow RA, Veras E, et al. A nomogram to predict postresection 5-year overall survival for patients with uterine leiomyosarcoma. Cancer. 2012;118:660–9.CrossRefPubMedGoogle Scholar
  34. 34.
    Wang WL, Soslow R, Hensley M, Asad H, Zannoni GF, de Nictolis M, et al. Histopathologic prognostic factors in stage I leiomyosarcoma of the uterus: a detailed analysis of 27 cases. Am J Surg Pathol. 2011;35:522–9.CrossRefPubMedGoogle Scholar
  35. 35.
    Garg G, Shah JP, Kumar S, Bryant CS, Munkarah A, Morris RT. Ovarian and uterine carcinosarcomas: a comparative analysis of prognostic variables and survival outcomes. Int J Gynecol Cancer. 2010;20:888–94.CrossRefPubMedGoogle Scholar
  36. 36.
    Tan PS, Koh E, Pang C, Ong WS, Ngo L, Soh LT, et al. Uterine leiomyosarcoma in Asian patients: validation of the revised federation of gynecology and obstetrics staging system and identification of prognostic classifiers. Oncologist. 2012;17:1286–93.PubMedCentralCrossRefPubMedGoogle Scholar
  37. 37.
    Silverberg SG, Major FJ, Blessing JA, et al. Carcinosarcoma (malignant mixed mesodermal tumor) of the uterus. A gynecologic oncology group pathologic study of 203 cases. Int J Gynecol Pathol. 1990;9:1–19.CrossRefPubMedGoogle Scholar
  38. 38.
    Kaku T, Silverberg SG, Major FJ, et al. Adenosarcoma of the uterus: a gynecologic oncology group clinicopathologic study of 31 cases. Int J Gynecol Pathol. 1992;11:75–88.CrossRefPubMedGoogle Scholar
  39. 39.
    Nordal RN, Kjørstad KE, Stenwig AE, Tropé CG. Leiomyosarcoma (LMS) and endometrial stromal sarcoma (ESS) of the uterus. A survey of patients treated in the Norwegian radium hospital 1976–1985. Int J Gynecol Cancer. 1993;3:110–5.CrossRefPubMedGoogle Scholar
  40. 40.
    Nordal RR. Uterine sarcomas in Norway 1956–1992: an epidemiological and clinicopathological study. A thesis. Oslo: Faculty of Medicine – University of Oslo; 1998.Google Scholar
  41. 41.
    Perry T, Korach J, Sadetzki S, et al. Uterine leimyosarcoma: does the primary surgical procedure matter? Int J Gynecol Cancer. 2009;19:257–60.CrossRefGoogle Scholar
  42. 42.
    Park J, Park S, Kim D, et al. The impact of tumor morcellation during surgery on the prognosis of patients with apparently early low-grade endometrial stromal sarcoma of the uterus. Ann Surg Oncol. 2011;18:3453–61.CrossRefPubMedGoogle Scholar
  43. 43.
    Fox H. Ploidy in gynaecological cancers. Histopathology. 2005;46:121–9.CrossRefPubMedGoogle Scholar
  44. 44.
    Kildal W, Abeler VM, Kristensen GB, Jenstad M, Thoresen SØ, Danielsen HE. The prognostic value of DNA ploidy in a total population of uterine sarcomas. Ann Oncol. 2009;20:1037–41.CrossRefPubMedGoogle Scholar

Copyright information

© Springer India 2015

Authors and Affiliations

  1. 1.Department of Gynecologic OncologyGujarat Cancer Research Institute (GCRI)AhmedabadIndia
  2. 2.Department of Obstetrics & GynecologyUniversity College of Medical Sciences and Guru Teg Bahadur HospitalDelhiIndia

Personalised recommendations