Uterine Sarcomas in the Elderly

  • Robert G. MakiEmail author
  • Afsheen Iqbal


Uterine tumors other than carcinoma represent a minority of tumors of this anatomic site. They demonstrate significant variety in terms of molecular pathogenesis and options used for their therapy. Tumors vary from those with specific translocations such as endometrial stromal sarcoma to those with aneuploid karyotypes such as leiomyosarcoma and carcinosarcoma (and other mixed Müllerian tumors). The treatment choices for uterine sarcomas and related tumors are further tempered by their development in an older population, limiting the ability to give some systemic therapeutics that can be safely given to younger patients. We review herein several of these diagnoses and their management, with emphasis on systemic approaches used in younger and older patients alike.


Leiomyosarcoma Endometrial stromal sarcoma Undifferentiated endometrial sarcoma Carcinosarcoma Rhabdomyosarcoma Perivascular epithelioid cell tumor PEComa 


  1. 1.
    Abeler VM, Nenodovic M. Diagnostic immunohistochemistry in uterine sarcomas: a study of 397 cases. Int J Gynecol Pathol. 2011;30(3):236–43.PubMedGoogle Scholar
  2. 2.
    Abeler VM, Royne 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(3):355–64.PubMedGoogle Scholar
  3. 3.
    Baker P, Oliva E. Endometrial stromal tumours of the uterus: a practical approach using conventional morphology and ancillary techniques. J Clin Pathol. 2007;60(3):235–43.PubMedGoogle Scholar
  4. 4.
    Bay JO, Ray-Coquard I, Fayette J, Leyvraz S, Cherix S, Piperno-Neumann S, et al. Docetaxel and gemcitabine combination in 133 advanced soft-tissue sarcomas: a retrospective analysis. Int J Cancer. 2006;119(3):706–11.PubMedGoogle Scholar
  5. 5.
    Beck AH, Lee CH, Witten DM, Gleason BC, Edris B, Espinosa I, et al. Discovery of molecular subtypes in leiomyosarcoma through integrative molecular profiling. Oncogene. 2010;29(6):845–54.PubMedGoogle Scholar
  6. 6.
    Bell SW, Kempson RL, Hendrickson MR. Problematic uterine smooth muscle neoplasms. A clinicopathologic study of 213 cases. Am J Surg Pathol. 1994;18(6):535–58.PubMedGoogle Scholar
  7. 7.
    Bodner K, Bodner-Adler B, Kimberger O, Czerwenka K, Leodolter S, Mayerhofer K. Estrogen and progesterone receptor expression in patients with uterine leiomyosarcoma and correlation with different clinicopathological parameters. Anticancer Res. 2003;23(1B):729–32.PubMedGoogle Scholar
  8. 8.
    Bonetti F, Pea M, Martignoni G, Zamboni G. PEC and sugar. Am J Surg Pathol. 1992;16(3):307–8.PubMedGoogle Scholar
  9. 9.
    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(1):204–8.PubMedGoogle Scholar
  10. 10.
    Brunello A, Basso U, Rossi E, Stefani M, Ghiotto C, Marino D, et al. Ifosfamide-related encephalopathy in elderly patients: report of five cases and review of the literature. Drugs Aging. 2007;24(11):967–73.PubMedGoogle Scholar
  11. 11.
    Buesa JM, Mouridsen HT, van Oosterom AT, Verweij J, Wagener T, Steward W, et al. High-dose DTIC in advanced soft-tissue sarcomas in the adult. A phase II study of the E.O.R.T.C. Soft Tissue and Bone Sarcoma Group. Ann Oncol. 1991;2(4):307–9.PubMedGoogle Scholar
  12. 12.
    Burke C, Hickey K. Treatment of endometrial stromal sarcoma with a gonadotropin-releasing hormone analogue. Obstet Gynecol. 2004;104(5 Pt 2):1182–4.PubMedGoogle Scholar
  13. 13.
    Chan JK, Kawar NM, Shin JY, Osann K, Chen LM, Powell CB, et al. Endometrial stromal sarcoma: a population-based analysis. Br J Cancer. 2008;99(8):1210–5.PubMedGoogle Scholar
  14. 14.
    Chang KL, Crabtree GS, Lim-Tan SK, Kempson RL, Hendrickson MR. Primary uterine endometrial stromal neoplasms. A clinicopathologic study of 117 cases. Am J Surg Pathol. 1990;14(5):415–38.PubMedGoogle Scholar
  15. 15.
    Chen L, Yang B. Immunohistochemical analysis of p16, p53, and Ki-67 expression in uterine smooth muscle tumors. Int J Gynecol Pathol. 2008;27(3):326–32.PubMedGoogle Scholar
  16. 16.
    Cheng X, Yang G, Schmeler KM, Coleman RL, Tu X, Liu J, et al. Recurrence patterns and prognosis of endometrial stromal sarcoma and the potential of tyrosine kinase-inhibiting therapy. Gynecol Oncol. 2011;121(2):323–7.PubMedGoogle Scholar
  17. 17.
    Chi DS, Mychalczak B, Saigo PE, Rescigno J, Brown CL. The role of whole-pelvic irradiation in the treatment of early-stage uterine carcinosarcoma. Gynecol Oncol. 1997;65(3):493–8.PubMedGoogle Scholar
  18. 18.
    Chiang S, Ali R, Melnyk N, McAlpine JN, Huntsman DG, Gilks CB, et al. Frequency of known gene rearrangements in endometrial stromal tumors. Am J Surg Pathol. 2011;35(9):1364–72.PubMedGoogle Scholar
  19. 19.
    D’Angelo E, Prat J. Uterine sarcomas: a review. Gynecol Oncol. 2010;116(1):131–9.PubMedGoogle Scholar
  20. 20.
    Demetri GD, Chawla SP, von Mehren M, Ritch P, Baker LH, Blay JY, et al. Efficacy and safety of trabectedin in patients with advanced or metastatic liposarcoma or leiomyosarcoma after failure of prior anthracyclines and ifosfamide: results of a randomized phase II study of two different schedules. J Clin Oncol. 2009;27(25):4188–96.PubMedGoogle Scholar
  21. 21.
    Donkers B, Kazzaz BA, Meijering JH. Rhabdomyosarcoma of the corpus uteri. Report of two cases with review of the literature. Am J Obstet Gynecol. 1972;114(8):1025–30.PubMedGoogle Scholar
  22. 22.
    Eriksson M. Histology-driven chemotherapy of soft-tissue sarcoma. Ann Oncol. 2010;21 Suppl 7:vii270–6.PubMedGoogle Scholar
  23. 23.
    Fadare O. Perivascular epithelioid cell tumor (PEComa) of the uterus: an outcome-based clinicopathologic analysis of 41 reported cases. Adv Anat Pathol. 2008;15(2):63–75.PubMedGoogle Scholar
  24. 24.
    Fadare O, Bonvicino A, Martel M, Renshaw IL, Azodi M, Parkash V. Pleomorphic rhabdomyosarcoma of the uterine corpus: a clinicopathologic study of 4 cases and a review of the literature. Int J Gynecol Pathol. 2010;29(2):122–34.PubMedGoogle Scholar
  25. 25.
    Ferguson SE, Tornos C, Hummer A, Barakat RR, Soslow RA. Prognostic features of surgical stage I uterine carcinosarcoma. Am J Surg Pathol. 2007;31(11):1653–61.PubMedGoogle Scholar
  26. 26.
    FIGO Committee on Gynecological Oncology. FIGO staging for uterine sarcomas. Int J Gynecol Obstet. 2009;104(3):179.PubMedGoogle Scholar
  27. 27.
    Fisher B, Costantino JP, Redmond CK, Fisher ER, Wickerham DL, Cronin WM. Endometrial cancer in tamoxifen-treated breast cancer patients: findings from the National Surgical Adjuvant Breast and Bowel Project (NSABP) B-14. J Natl Cancer Inst. 1994;86(7):527–37.PubMedGoogle Scholar
  28. 28.
    Folpe AL. Neoplasms with perivascular epithelioid cell differentiation (PEComas). In: Fletcher CDM, Unni KK, Epstein J, Mertens F, editors. Pathology and genetics of tumours of soft tissue and bone, WHO classification of tumours. Lyon: IARC Press; 2002. p. 221–2.Google Scholar
  29. 29.
    Folpe AL, Mentzel T, Lehr HA, Fisher C, Balzer BL, Weiss SW. Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature. Am J Surg Pathol. 2005;29(12):1558–75.PubMedGoogle Scholar
  30. 30.
    Gadducci A, Cosio S, Romanini A, Genazzani AR. The management of patients with uterine sarcoma: a debated clinical challenge. Crit Rev Oncol Hematol. 2008;65(2):129–42.PubMedGoogle Scholar
  31. 31.
    Gadducci A, Sartori E, Landoni F, Zola P, Maggino T, Urgesi A, et al. Endometrial stromal sarcoma: analysis of treatment failures and survival. Gynecol Oncol. 1996;63(2):247–53.PubMedGoogle Scholar
  32. 32.
    Garcia del Muro X, Lopez-Pousa A, Martin J, Buesa JM, Martinez-Trufero J, Casado A, 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(8):1706–12.PubMedGoogle Scholar
  33. 33.
    Garcia-Del-Muro X, Lopez-Pousa A, Maurel J, Martin J, Martinez-Trufero J, Casado A, 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(18):2528–33.PubMedGoogle Scholar
  34. 34.
    Geoerger B, Chisholm J, Le Deley MC, Gentet JC, Zwaan CM, Dias N, et al. Phase II study of gemcitabine combined with oxaliplatin in relapsed or refractory paediatric solid malignancies: an innovative therapy for children with Cancer European Consortium Study. Eur J Cancer. 2011;47(2):230–8.PubMedGoogle Scholar
  35. 35.
    Gerszten K, Faul C, Kounelis S, Huang Q, Kelley J, Jones MW. The impact of adjuvant radiotherapy on carcinosarcoma of the uterus. Gynecol Oncol. 1998;68(1):8–13.PubMedGoogle Scholar
  36. 36.
    Giuntoli 2nd RL, Metzinger DS, DiMarco CS, Cha SS, Sloan JA, Keeney GL, et al. Retrospective review of 208 patients with leiomyosarcoma of the uterus: prognostic indicators, surgical management, and adjuvant therapy. Gynecol Oncol. 2003;89(3):460–9.PubMedGoogle Scholar
  37. 37.
    Goldstein LS, Kavuru MS, Meli Y, Tuthill RJ, Mehta AC. Uterine rhabdomyosarcoma metastatic to mediastinal lymph nodes: diagnosis by transbronchial needle aspiration. South Med J. 1999;92(1):84–7.PubMedGoogle Scholar
  38. 38.
    Gonzalez Bosquet J, Terstriep SA, Cliby WA, Brown-Jones M, Kaur JS, Podratz KC, et al. The impact of multi-modal therapy on survival for uterine carcinosarcomas. Gynecol Oncol. 2010;116(3):419–23.PubMedGoogle Scholar
  39. 39.
    Gottlieb JA, Benjamin RS, Baker LH, O’Bryan RM, Sinkovics JG, Hoogstraten B, et al. Role of DTIC (NSC-45388) in the chemotherapy of sarcomas. Cancer Treat Rep. 1976;60(2):199–203.PubMedGoogle Scholar
  40. 40.
    Greene LA, Mount SL, Schned AR, Cooper K. Recurrent perivascular epithelioid cell tumor of the uterus (PEComa): an immunohistochemical study and review of the literature. Gynecol Oncol. 2003;90(3):677–81.PubMedGoogle Scholar
  41. 41.
    Harlow BL, Weiss NS, Lofton S. The epidemiology of sarcomas of the uterus. J Natl Cancer Inst. 1986;76(3):399–402.PubMedGoogle Scholar
  42. 42.
    Hensley ML. Adjuvant treatment of high-risk primary uterine leiomyosarcoma with gemcitabine/docetaxel (GT), followed by doxorubicin (D): results of phase II multicenter trial SARC005. J Clin Oncol. 2010;28:15s (suppl; abstr 10021).Google Scholar
  43. 43.
    Hensley M, Wathen K, Maki R. 3-year follow up of SARC005: adjuvant treatment of high risk primary uterine leiomyosarcoma with gemcitabine/docetaxel, followed by doxorubicin. In: Proceedings of the connective tissue oncology society, 2011. Abstract 78.Google Scholar
  44. 44.
    Hensley ML. Role of chemotherapy and biomolecular therapy in the treatment of uterine sarcomas. Best Pract Res Clin Obstet Gynaecol. 2011;25(6):773–82.PubMedGoogle Scholar
  45. 45.
    Hensley ML, Blessing JA, Degeest K, Abulafia O, Rose PG, Homesley HD. 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(3):323–8.PubMedGoogle Scholar
  46. 46.
    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(3):329–34.PubMedGoogle Scholar
  47. 47.
    Hensley ML, Ishill N, Soslow R, Larkin J, Abu-Rustum N, Sabbatini P, 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(3):563–7.PubMedGoogle Scholar
  48. 48.
    Hensley ML, Maki R, Venkatraman E, Geller G, Lovegren M, Aghajanian C, et al. Gemcitabine and docetaxel in patients with unresectable leiomyosarcoma: results of a phase II trial. J Clin Oncol. 2002;20(12):2824–31.PubMedGoogle Scholar
  49. 49.
    Homesley HD, Filiaci V, Markman M, Bitterman P, Eaton L, Kilgore LC, et al. Phase III trial of ifosfamide with or without paclitaxel in advanced uterine carcinosarcoma: a Gynecologic Oncology Group Study. J Clin Oncol. 2007;25(5):526–31.PubMedGoogle Scholar
  50. 50.
    Huh WK, Sill MW, Darcy KM, Elias KM, Hoffman JS, Boggess JF, et al. Efficacy and safety of imatinib mesylate (Gleevec) and immunohistochemical expression of c-Kit and PDGFR-beta in a Gynecologic Oncology Group Phase Il Trial in women with recurrent or persistent carcinosarcomas of the uterus. Gynecol Oncol. 2010;117(2):248–54.PubMedGoogle Scholar
  51. 51.
    Italiano A, Delcambre C, Hostein I, Cazeau AL, Marty M, Avril A, et al. Treatment with the mTOR inhibitor temsirolimus in patients with malignant PEComa. Ann Oncol. 2010;21(5):1135–7.PubMedGoogle Scholar
  52. 52.
    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(4):820–30.PubMedGoogle Scholar
  53. 53.
    Karpathiou G, Sivridis E, Giatromanolaki A. Myxoid leiomyosarcoma of the uterus: a diagnostic challenge. Eur J Gynaecol Oncol. 2010;31(4):446–8.PubMedGoogle Scholar
  54. 54.
    Koss LG, Spiro RH, Brunschwig A. Endometrial stromal sarcoma. Surg Gynecol Obstet. 1965;121:531–7.PubMedGoogle Scholar
  55. 55.
    Kulka EW, Douglas GW. Rhabdomyosarcoma of the corpus uteri; report of a case, associated with adenocarcinoma of the cervix, with review of the literature. Cancer. 1952;5(4):727–36.PubMedGoogle Scholar
  56. 56.
    Leath 3rd CA, Huh WK, Hyde Jr J, Cohn DE, Resnick KE, Taylor NP, et al. A multi-institutional review of outcomes of endometrial stromal sarcoma. Gynecol Oncol. 2007;105(3):630–4.PubMedGoogle Scholar
  57. 57.
    Lee C, Marino-Enriquez A, Van de Rijn M, Gilks C, Debiec-Rychter M, Dal Cin P, et al. The histologic features of endometrial stromal sarcomas characterized by YWHAE rearrangement – distinction from usual low-grade endometrial stromal sarcoma with JAZF1 rearrangement. Proceedings of the USCAP; 255a (abstract 1081), 2010.Google Scholar
  58. 58.
    Lee CH, Ali RH, Rouzbahman M, Marino-Enriquez A, Zhu M, Guo X, et al. Cyclin D1 as a Diagnostic Immunomarker for Endometrial Stromal Sarcoma With YWHAE-FAM22 Rearrangement. Am J Surg Pathol. 2012;36(10):1562–70.Google Scholar
  59. 59.
    Lee CH, Ou WB, Mariño-Enriquez A, Zhu M, Mayeda M, Wang Y, et al. 14-3-3 fusion oncogenes in high-grade endometrial stromal sarcoma. Proc Natl Acad Sci USA. 2012;109(3):929–34. Lee CH, Mariño-Enriquez A, Ou W, Zhu M, Ali RH, Chiang S, et al. The clinicopathologic features of YWHAE-FAM22 endometrial stromal sarcomas: a histologically high-grade and clinically aggressive tumor. Am J Surg Pathol. 2012;36(5):641–53Google Scholar
  60. 60.
    Lee CL, Chang TC, Shueh S, Soong YK. Rhabdomyosarcoma following radiation therapy for cervical carcinoma–a case report. Changgeng Yi Xue Za Zhi. 1990;13(1):65–71.PubMedGoogle Scholar
  61. 61.
    Major FJ, Blessing JA, Silverberg SG, Morrow CP, Creasman WT, Currie JL, et al. Prognostic factors in early-stage uterine sarcoma. A Gynecologic Oncology Group study. Cancer. 1993;71(4 Suppl):1702–9.PubMedGoogle Scholar
  62. 62.
    Maki RG, Wathen JK, Patel SR, Priebat DA, Okuno SH, Samuels B, 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(19):2755–63.PubMedGoogle Scholar
  63. 63.
    Makker V, Abu-Rustum NR, Alektiar KM, Aghajanian CA, Zhou Q, Iasonos A, et al. A retrospective assessment of outcomes of chemotherapy-based versus radiation-only adjuvant treatment for completely resected stage I-IV uterine carcinosarcoma. Gynecol Oncol. 2008;111(2):249–54.PubMedGoogle Scholar
  64. 64.
    Malouf GG, Duclos J, Rey A, Duvillard P, Lazar V, Haie-Meder C, et al. Impact of adjuvant treatment modalities on the management of patients with stages I-II endometrial stromal sarcoma. Ann Oncol. 2010;21(10):2102–6.PubMedGoogle Scholar
  65. 65.
    Meredith RF, Eisert DR, Kaka Z, Hodgson SE, Johnston Jr GA, Boutselis JG. An excess of uterine sarcomas after pelvic irradiation. Cancer. 1986;58(9):2003–7.PubMedGoogle Scholar
  66. 66.
    Miller BE, Blessing JA, Stehman FB, Shahin MS, Yamada SD, Secord AA, et al. A phase II evaluation of weekly gemcitabine and docetaxel for second-line treatment of recurrent carcinosarcoma of the uterus: a gynecologic oncology group study. Gynecol Oncol. 2010;118(2):139–44.PubMedGoogle Scholar
  67. 67.
    Miller DS, Blessing JA, Schilder J, Munkarah A, Lee YC. Phase II evaluation of topotecan in carcinosarcoma of the uterus: a Gynecologic Oncology Group study. Gynecol Oncol. 2005;98(2):217–21.PubMedGoogle Scholar
  68. 68.
    Morice P, Rodrigues A, Pautier P, Rey A, Camatte S, Atallah D, et al. Surgery for uterine sarcoma: review of the literature and recommendations for the standard surgical procedure. Gynecol Obstet Fertil. 2003;31(2):147–50.PubMedGoogle Scholar
  69. 69.
    National Comprehensive Cancer Network (NCCN) guidelines. Available at: Accessed on 15 Nov 2011.
  70. 70.
    Nemani D, Mitra N, Guo M, Lin L. Assessing the effects of lymphadenectomy and radiation therapy in patients with uterine carcinosarcoma: a SEER analysis. Gynecol Oncol. 2008;111(1):82–8.PubMedGoogle Scholar
  71. 71.
    Nucci MR, Harburger D, Koontz J, Dal Cin P, Sklar J. Molecular analysis of the JAZF1-JJAZ1 gene fusion by RT-PCR and fluorescence in situ hybridization in endometrial stromal neoplasms. Am J Surg Pathol. 2007;31(1):65–70.PubMedGoogle Scholar
  72. 72.
    O’Cearbhaill R, Zhou Q, Iasonos A, Soslow RA, Leitao MM, Aghajanian C, et al. Treatment of advanced uterine leiomyosarcoma with aromatase inhibitors. Gynecol Oncol. 2010;116(3):424–9.PubMedGoogle Scholar
  73. 73.
    Okada DH, Rowland JB, Petrovic LM. Uterine pleomorphic rhabdomyosarcoma in a patient receiving tamoxifen therapy. Gynecol Oncol. 1999;75(3):509–13.PubMedGoogle Scholar
  74. 74.
    Oliva E, Young RH, Amin MB, Clement PB. An immunohistochemical analysis of endometrial stromal and smooth muscle tumors of the uterus: a study of 54 cases emphasizing the importance of using a panel because of overlap in immunoreactivity for individual antibodies. Am J Surg Pathol. 2002;26(4):403–12.PubMedGoogle Scholar
  75. 75.
    Omura GA, Blessing JA, Major F, Lifshitz S, Ehrlich CE, Mangan C, et al. A randomized ­clinical trial of adjuvant adriamycin in uterine sarcomas: a Gynecologic Oncology Group Study. J Clin Oncol. 1985;3(9):1240–5.PubMedGoogle Scholar
  76. 76.
    O’Neill CJ, McBride HA, Connolly LE, McCluggage WG. Uterine leiomyosarcomas are characterized by high p16, p53 and MIB1 expression in comparison with usual leiomyomas, leiomyoma variants and smooth muscle tumours of uncertain malignant potential. Histopathology. 2007;50(7):851–8.PubMedGoogle Scholar
  77. 77.
    Pautier P, Bui B, Penel B, Piperno-Neumann S, Delcambre-Lair C, Bompas E, et al. Final results of a FNCLCC French Sarcoma Group multicenter randomized phase II study of ­gemcitabine versus gemcitabine and docetaxel in patients with metastatic or relapsed leiomyosarcoma (suppl; abstr 10527). J Clin Oncol. 2009; 27:15s.Google Scholar
  78. 78.
    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(2):257–60.PubMedGoogle Scholar
  79. 79.
    Podczaski E, Sees J, Kaminski P, Sorosky J, Larson JE, DeGeest K, et al. Rhabdomyosarcoma of the uterus in a postmenopausal patient. Gynecol Oncol. 1990;37(3):439–42.PubMedGoogle Scholar
  80. 80.
    Pothuri B, Ramondetta L, Eifel P, Deavers MT, Wilton A, Alektiar K, et al. Radiation-associated endometrial cancers are prognostically unfavorable tumors: a clinicopathologic comparison with 527 sporadic endometrial cancers. Gynecol Oncol. 2006;103(3):948–51.PubMedGoogle Scholar
  81. 81.
    Pothuri B, Ramondetta L, Martino M, Alektiar K, Eifel PJ, Deavers MT, et al. Development of endometrial cancer after radiation treatment for cervical carcinoma. Obstet Gynecol. 2003;101(5 Pt 1):941–5.PubMedGoogle Scholar
  82. 82.
    Powell MA, Filiaci VL, Rose PG, Mannel RS, Hanjani P, Degeest K, et al. Phase II evaluation of paclitaxel and carboplatin in the treatment of carcinosarcoma of the uterus: a Gynecologic Oncology Group study. J Clin Oncol. 2010;28(16):2727–31.PubMedGoogle Scholar
  83. 83.
    Prat J. FIGO staging for uterine sarcomas. Int J Gynaecol Obstet. 2009;104(3):177–8.PubMedGoogle Scholar
  84. 84.
    Raji R, Guzzo F, Carrara L, Varughese J, Cocco E, Bellone S, et al. Uterine and ovarian ­carcinosarcomas overexpressing Trop-2 are sensitive to hRS7, a humanized anti-Trop-2 antibody. J Exp Clin Cancer Res. 2011;30(1):106.PubMedGoogle Scholar
  85. 85.
    Reed NS, Mangioni C, Malmstrom H, Scarfone G, Poveda A, Pecorelli S, 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(6):808–18.PubMedGoogle Scholar
  86. 86.
    Regauer S, Emberger W, Reich O, Pfragner R. Cytogenetic analyses of two new cases of endometrial stromal sarcoma–non-random reciprocal translocation t(10;17)(q22;p13) correlates with fibrous ESS. Histopathology. 2008;52(6):780–3.PubMedGoogle Scholar
  87. 87.
    Reich O, Regauer S. Hormonal therapy of endometrial stromal sarcoma. Curr Opin Oncol. 2007;19(4):347–52.PubMedGoogle Scholar
  88. 88.
    Reich O, Regauer S, Urdl W, Lahousen M, Winter R. Expression of oestrogen and progesterone receptors in low-grade endometrial stromal sarcomas. Br J Cancer. 2000;82(5):1030–4.PubMedGoogle Scholar
  89. 89.
    Sampath S, Schultheiss TE, Ryu JK, Wong JY. The role of adjuvant radiation in uterine sarcomas. Int J Radiat Oncol Biol Phys. 2010;76(3):728–34.PubMedGoogle Scholar
  90. 90.
    Schwartz SM, Weiss NS, Daling JR, Gammon MD, Liff JM, Watt J, et al. Exogenous sex hormone use, correlates of endogenous hormone levels, and the incidence of histologic types of sarcoma of the uterus. Cancer. 1996;77(4):717–24.PubMedGoogle Scholar
  91. 91.
    Shah JP, Bryant CS, Kumar S, Ali-Fehmi R, Malone Jr JM, Morris RT. Lymphadenectomy and ovarian preservation in low-grade endometrial stromal sarcoma. Obstet Gynecol. 2008;112(5):1102–8.PubMedGoogle Scholar
  92. 92.
    Sherman ME, Devesa SS. Analysis of racial differences in incidence, survival, and mortality for malignant tumors of the uterine corpus. Cancer. 2003;98(1):176–86.PubMedGoogle Scholar
  93. 93.
    Silverberg SG, Major FJ, Blessing JA, Fetter B, Askin FB, Liao SY, 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):1–19.PubMedGoogle Scholar
  94. 94.
    Slayton RE, Blessing JA, DiSaia PJ, Christopherson WA. Phase II trial of etoposide in the management of advanced or recurrent mixed mesodermal sarcomas of the uterus: a Gynecologic Oncology Group Study. Cancer Treat Rep. 1987;71(6):661–2.PubMedGoogle Scholar
  95. 95.
    Spano JP, Soria JC, Kambouchner M, Piperno-Neuman S, Morin F, Morere JF, et al. Long-term survival of patients given hormonal therapy for metastatic endometrial stromal sarcoma. Med Oncol. 2003;20(1):87–93.PubMedGoogle Scholar
  96. 96.
    Sutton G, Blessing JA, Park R, DiSaia PJ, Rosenshein N. Ifosfamide treatment of recurrent or metastatic endometrial stromal sarcomas previously unexposed to chemotherapy: a study of the Gynecologic Oncology Group. Obstet Gynecol. 1996;87(5 Pt 1):747–50.PubMedGoogle Scholar
  97. 97.
    Sutton G, Brunetto VL, Kilgore L, Soper JT, McGehee R, Olt G, et al. A phase III trial of ifosfamide with or without cisplatin in carcinosarcoma of the uterus: a Gynecologic Oncology Group Study. Gynecol Oncol. 2000;79(2):147–53.PubMedGoogle Scholar
  98. 98.
    Sutton GP, Blessing JA, Homesley HD, Malfetano JH. A phase II trial of ifosfamide and mesna in patients with advanced or recurrent mixed mesodermal tumors of the ovary previously treated with platinum-based chemotherapy: a Gynecologic Oncology Group study. Gynecol Oncol. 1994;53(1):24–6.PubMedGoogle Scholar
  99. 99.
    Sutton GP, Blessing JA, Rosenshein N, Photopulos G, DiSaia PJ. Phase II trial of ifosfamide and mesna in mixed mesodermal tumors of the uterus (a Gynecologic Oncology Group study). Am J Obstet Gynecol. 1989;161(2):309–12.PubMedGoogle Scholar
  100. 100.
    Szlosarek PW, Lofts FJ, Pettengell R, Carter P, Young M, Harmer C. Effective treatment of a patient with a high-grade endometrial stromal sarcoma with an accelerated regimen of carboplatin and paclitaxel. Anticancer Drugs. 2000;11(4):275–8.PubMedGoogle Scholar
  101. 101.
    Tavassoli FA, Devilee P, editors. World Health Organization classification of tumours. In: Pathology and genetics of tumours of the breast and female genital organs. IARC Press: Lyon; 2003.Google Scholar
  102. 102.
    Temkin SM, Hellmann M, Lee YC, Abulafia O. Early-stage carcinosarcoma of the uterus: the significance of lymph node count. Int J Gynecol Cancer. 2007;17(1):215–9.PubMedGoogle Scholar
  103. 103.
    Thigpen JT, Blessing JA, Beecham J, Homesley H, Yordan E. Phase II trial of cisplatin as first-line chemotherapy in patients with advanced or recurrent uterine sarcomas: a Gynecologic Oncology Group study. J Clin Oncol. 1991;9(11):1962–6.PubMedGoogle Scholar
  104. 104.
    Thigpen JT, Blessing JA, Orr Jr JW, DiSaia PJ. Phase II trial of cisplatin in the treatment of patients with advanced or recurrent mixed mesodermal sarcomas of the uterus: a Gynecologic Oncology Group Study. Cancer Treat Rep. 1986;70(2):271–4.PubMedGoogle Scholar
  105. 105.
    Vang R, Kempson RL. Perivascular epithelioid cell tumor (‘PEComa’) of the uterus: a subset of HMB-45-positive epithelioid mesenchymal neoplasms with an uncertain relationship to pure smooth muscle tumors. Am J Surg Pathol. 2002;26(1):1–13.PubMedGoogle Scholar
  106. 106.
    Wagner AJ, Malinowska-Kolodziej I, Morgan JA, Qin W, Fletcher CD, Vena N, et al. Clinical activity of mTOR inhibition with sirolimus in malignant perivascular epithelioid cell tumors: targeting the pathogenic activation of mTORC1 in tumors. J Clin Oncol. 2010;28(5):835–40.PubMedGoogle Scholar
  107. 107.
    Wagner-Bohn A, Paulussen M, Vieira Pinheiro JP, Gerss J, Stoffregen C, Boos J. Phase II study of gemcitabine in children with solid tumors of mesenchymal and embryonic origin. Anticancer Drugs. 2006;17(7):859–64.PubMedGoogle Scholar
  108. 108.
    Wickerham DL, Fisher B, Wolmark N, Bryant J, Costantino J, Bernstein L, et al. Association of tamoxifen and uterine sarcoma. J Clin Oncol. 2002;20(11):2758–60.PubMedGoogle Scholar
  109. 109.
    Wolfson AH, Brady MF, Rocereto T, Mannel RS, Lee YC, Futoran RJ, et al. A gynecologic oncology group randomized phase III trial of whole abdominal irradiation (WAI) vs. cisplatin-ifosfamide and mesna (CIM) as post-surgical therapy in stage I-IV carcinosarcoma (CS) of the uterus. Gynecol Oncol. 2007;107(2):177–85.PubMedGoogle Scholar
  110. 110.
    Wysowski DK, Honig SF, Beitz J. Uterine sarcoma associated with tamoxifen use. N Engl J Med. 2002;346(23):1832–3.PubMedGoogle Scholar
  111. 111.
    Yamawaki T, Shimizu Y, Hasumi K. Treatment of stage IV “high-grade” endometrial stromal sarcoma with ifosfamide, adriamycin, and cisplatin. Gynecol Oncol. 1997;64(2):265–9.PubMedGoogle Scholar
  112. 112.
    Yi-Shin Kuo D, Timmins P, Blank SV, Fields AL, Goldberg GL, Murgo A, et al. Phase II trial of thalidomide for advanced and recurrent gynecologic sarcoma: a brief communication from the New York Phase II consortium. Gynecol Oncol. 2006;100(1):160–5.PubMedGoogle Scholar
  113. 113.
    Zelmanowicz A, Hildesheim A, Sherman ME, Sturgeon SR, Kurman RJ, Barrett RJ, et al. Evidence for a common etiology for endometrial carcinomas and malignant mixed mullerian tumors. Gynecol Oncol. 1998;69(3):253–7.PubMedGoogle Scholar
  114. 114.
    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(3):660–9.PubMedGoogle Scholar

Copyright information

© Springer-Verlag London 2013

Authors and Affiliations

  1. 1.Departments of Medicine, Pediatrics, and OrthopaedicsThe Mount Sinai HospitalNew YorkUSA
  2. 2.Department of MedicineThe Mount Sinai HospitalNew YorkUSA

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