Uterine Sarcomas – a Review
Authors:
M. Klačko 1; P. Babala 1; P. Mikloš 1; P. Zuzák 2; M. Chorvath 3; M. Ondrušová 4; D. Ondruš 5
Authors‘ workplace:
Klinika gynekologickej onkológie, OUSA a SZU Bratislava, Slovenská republika
1; Interná onkologická klinika, OUSA a TU Bratislava, Slovenská republika
2; Klinika stereotaktickej rádiochirurgie, OUSA a VŠZaSP sv. Alžbety Bratislava, Slovenská republika
3; II. onkologická klinika, LF UK a NOÚ Bratislava, Slovenská republika
4; I. onkologická klinika, LF UK a OUSA Bratislava, Slovenská republika
5
Published in:
Klin Onkol 2012; 25(5): 340-345
Category:
Reviews
Overview
Uterine sarcomas are a heterogeneous group, which constitutes about 8% of malignant uterine tumors. This heterogeneousness and rare occurrence were the main cause of non-uniform therapeutical management. In previously published papers, there were mainly retrospective assessments of the experience of individual centres. The basis of relevant conclusions of the studies, beside their prospectiveness, is the use of unified classification criteria. Currently, a completely new classification of uterine sarcomas is being used, which consists of leiomyosarcoma, endometrial stromal sarcomas and adenosarcomas. For classification of carcinosarcomas, there are valid new criteria of endometrial cancer classification. The basic therapeutic approach of leiomyosarcoma and endometrial stromal sarcomas is a surgical intervention. The gold standard is hysterectomy and salpingooophorectomy. Justifiability of lymphadenectomy is being discussed. For carcinosarcomas, the same recommendations as for the surgical treatment of prognostically unfavourable endometrial carcinoma are valid – hysterectomy, salpingooophorectomy, pelvic and paraaortal lymph node dissection and omentectomy. It is necessary to implement the new classification into clinical practice, to publish and evaluate existing papers, which take into account their basic thesis. Only then it will be possible to create unified therapies. They should be aimed to improve patients’ survival.
Key words:
sarcoma – uterus – epidemiology – classification – terminology – treatment
Submitted:
4. 4. 2012
Accepted:
21. 5. 2012
Sources
1. Sobin LH, Gospodarowicz MK, Wittekind C. TNM classification of malignant tumours. 7th ed. Oxford: Wiley-Blackwell 2009.
2. Freitag P. Sarkomy dělohy. Česk Gynek 1997; 62(6): 362–365.
3. Ondrušová M, Pleško I, Safaei-Diba C et al. Komplexná analýza výskytu a úmrtnosti na zhubné nádory v Slovenskej republike (online). Bratislava: Národný onkologický register SR NCZI 2007. Dostupné na: http: www.nor-sk.org.
4. Ondrušová M. Epidemiológia nádorov (prednáška). Kurz špecializačného štúdia v špecializačnom odbore ošetrovateľská starostlivosť v onkológii. Bratislava: SZU 20. 10. 2006.
5. Ueda SM, Cheung MK, Osann K et al. Factors responsible for the increase in death rate of women diagnosed with uterine corpus cancer. In: Proceedings of the Society of Gynecologic Oncologists 38th annual meeting. San Diego CA, USA 2007. Abstract 644.
6. Tavassoli FA, Devilee P. Pathology and genetics of tumours of the breast and female genital organs. WHO classification of tumours. Lyon: IARC Press 2003.
7. Hacker NF. Uterine Cancer. Budapest: European School of Oncology 1966.
8. Dafapoulos A, Tsikouras P, Dimitraki M et al. The role of lymphadenectomy in uterine leiomyosarcoma: review of the literature and recommendations for the standard surgical procedure. Arch Gynecol Obstet 2010; 282(3): 292–300.
9. Prat J. FIGO staging for uterine sarcomas. Int J Gynaecol Obstet 2009; 104(3): 177–178.
10. 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(3): 460–469.
11. Sobin L, Wittekind C. TNM classification of malignant tumours. 6th ed. New York: Wiley-Liss 2002.
12. Mittal K, Joutovsky A. Areas with benign morphologic and immunohistochemical features are associated with some uterine leiomyosarcomas. Gynecol Oncol 2007; 104(2): 362–365.
13. Bell SW, Kempson RL, Hendrickson MR. Problematic uterine smooth muscle neoplasms. A clinicopathologic study of 213 cases. Am J Surg Pathol 1994; 18(6): 535–558.
14. Wang WL, Soslow RA, Zannoni GF et al. The utility of tumor cell necrosis in the diagnosis of primary leiomyosarcoma of the uterus: an analysis of 77 cases. Mod Pathol 2006; 19 (Suppl 1): 201A.
15. Perrone T, Dehner LP. Prognostically favorable „mitotically active“ smooth-muscle tumors of the uterus. A clinicopathologic study of ten cases. Am J Surg Pathol 1988; 12(1): 1–8.
16. O’Connor DM, Norris HJ. Mitotically active leiomyomas of the uterus. Hum Pathol 1990; 21(2): 223–227.
17. Zhang P, Zhang C, Hao J et al. Use of X-chromosome inactivation pattern to determine clonal origins of uterine leiomyoma and leiomyosarcoma. Hum Pathol 2006; 37(10): 1350–1356.
18. Atkins KA, Arronte N, Darus CJ et. Al. The Use of p16 in enhancing the histologic classification of uterine smooth muscle tumors. Am J Surg Pathol 2008; 32(1): 98–102.
19. Bodner-Adler B, Bodner K, Czerwenka K et al. Expression of p16 protein in patients with uterine smooth muscle tumors: an immunohistochemical analysis. Gynecol Oncol 2005; 96(1): 62–66.
20. 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): 326–332.
21. O’Neill CJ, McBride HA, Connolly LE et al. 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): 851–858.
22. Akhan SE, Yavuz E, Tecer A et al. The expression of Ki-67, p53, estrogen and progesterone receptors affecting survival in uterine leiomyosarcomas. A clinocopathologic study. Gynecol Oncol 2005; 99(1): 36–42.
23. Raspollini MR, Pinzani P, Simi L et al. Uterine leiomyosarcomas express KIT protein but lack mutation(s) in exon 9 of c-KIT. Gynecol Oncol 2005; 98(2): 334–335.
24. Amant F, Coosemans A, Debiec-Rychter M et al. Clinical management of uterine sarcomas. Lancet Oncol 2009; 10(12): 1188–1198.
25. Benoit L, Arnould L, Cheynel M et al. The role of surgery and treatment trends in uterine sarcoma. Eur J Surg Oncol 2005; 31(4): 434–442.
26. Morice P, Rodriques A, Pautier P et al. Surgery for uterine sarcoma: review of the literature and recommendations for the standard surgical procedure. Gynecol Obstet Fertil 2003; 31(2): 147–150.
27. Gaducci A, Landoni F, Sartori E et al. Uterine leiomyosarcoma: analysis of treatment failures and survival. Gynecol Oncol 1996; 62(1): 25–32.
28. Günthert AR. Sarcomas and mixed mesodermal tumors ot he uterus. Ther Umsch 2011; 68(10): 559–564.
29. Giuntoli RL 2nd, Bristow RE. Uterine leiomyosarcoma: present management. Curr Opin Oncol 2004; 16(4): 324–327.
30. Major FJ, Blessing JA, Silverberg SG et al. Prognostic factors in early-stage uterine sarcoma. A Gynecologic Oncology Group study. Cancer 1993; 71 (Suppl 4): 1702–1709.
31. 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(1): 105–109.
32. Leibsohn S, d’Ablaing G, Mishell DR Jr et al. Leiomyosarcoma in a series of hysterectomies performed for presumed uterine leiomyomas. Am J Obstet Gynecol 1990; 162(4): 968–974.
33. Gard GB, Mulvany NJ, Quinn MA. Management of uterine leiomyosarcoma in Australia. Aust N Z J Obstet Gynaecol 1999; 39(1): 93–98.
34. Kapp DS, Shin JY, Chan JK. Prognostic factors and survival in 1,396 patients with uterine leiomyosarcomas: emphasis on impact of lymphadenectomy and oophorectomy. Cancer 2008; 112(4): 820–830.
35. Riopel J, Plante M, Renaud MC et al. Lymph node metastases in low-grade endometrial stromal sarcoma. Gynecol Oncol 2005; 96(2): 402–406.
36. Reich O, Winter R, Regauer S. Should lymphadenectomy be performed in patients with endometrial stromal sarcoma? Gynecol Oncol 2005; 97(3): 982–983.
37. Ramondetta L, Bodurka D, Deavers M et al. Uterine Sarcomas. In: Eifel PJ, Gershenson DM, Kavanagh JJ et al (eds). MD. Anderson Cancer Care Series, Gynecologic Cancer. New York: Springer 2006: 125–147.
38. Gadducci A, Cosio S, Romanini A et al. The management of patients with uterine sarcoma: a debated clinical challenge. Crit Rev Oncol Hematol 2008; 65(2): 129–142.
39. D’Angelo E, Prat J. Uterine sarcomas: a review. Gynecol Oncol 2010; 116(1): 131–139.
40. Spano JP, Soria JC, Kambouchner M et al. Long-term survival of patients given hormonal therapy for metastatic endometrial stromal sarcoma. Med Oncol 2003; 20(1): 87–93.
41. Shah JP, Bryant CS, Kumar S et al. Lymphadenectomy and ovarian preservation on low-grade endometrial stromal sarcoma. Obstet Gynecol 2008; 112(5): 1102–1108.
42. Kohler GH, Evert M. Handbuch und Bildatlas der uterinen Sarkome und Misch tumoren. Belin: De-Gruyter 2009.
43. Zagouri F, Dimopoulos AM, Fotiou S. Treatment of early uterine sarcomas: disentangling adjuvant modalities. World J Surg Oncol 2009; 7: 38.
44. 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): 1–19.
45. Sharma NK, Sorosky JI, Bender D et al. Malignant mixed müllerian tumor (MMMT) of the cervix. Gynecol Oncol 2005; 97(2): 442–445.
46. Wright JD, Rosenblum K, Huettner PC et al. Cervical sarcomas: an analysis of incidence and outcome. Gynecol Oncol 2005; 99(2): 348-351.
47. Abeloff MD, Armitage JO, Niederhuber JE et al. Abeloff’s Clinical Oncology. 4th ed. Philadelphia: Churchill Livingstone Elsevier 2008.
48. 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(9): 1240–1245.
49. Omura GA, Major FJ, Blessing JA et al. A randomized study of adriamycin with and without dimethyl triazenoimidazole carboxamide in advanced uterine sarcomas. Cancer 1983; 52(4): 626–632.
50. 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(2): 644–647.
51. Judson I, Radford JA, Harris M et al. Randomised phase II trial of pegylated liposomal doxorubicin (DOXIL/CAELYX) versus doxorubicin in the treatment of advanced or metastatic soft tissue sarcoma: a study by the EORTC Soft Tissue and Bone Sarcoma Group. Eur J Cancer 2001; 37(7): 870–877.
52. 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(3): 749–752.
53. Gallup DG, Blessing JA, Andersen W et al. Evaluation of paclitaxel in previously treated leiomyosarcoma of the uterus: a gynecologic oncology group study. Gynecol Oncol 2003; 89(1): 48–51.
54. Hensley ML, Maki R, Venkatraman E et al. Gemcitabine and docetaxel in patients with unresectable leiomyosarcoma: results of a phase II trial. J Clin Oncol 2002; 20(12): 2824–2831.
55. Currie J, Blessing JA, Muss HB et al. Combination chemotherapy with hydroxyurea, dacarbazine (DTIC), and etoposide in the treatment of uterine leiomyosarcoma: a Gynecologic Oncology Group study. Gynecol Oncol 1996; 61(1): 27–30.
56. Demetri GD, Chawla SP, von Mehren M 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): 4188–4196.
57. McMeekin DS, Sill MW, Darcy KM et al. A phase II trial of thalidomide in patients with refractory leiomyosarcoma of the uterus and correlation with biomarkers of angiogenesis: a Gynecologic Oncology Group study. Gynecol Oncol 2007; 106(3): 596–603.
58. Hensley ML, Sill MW, Scribner DR Jr et al. Sunitinib malate in the treatment of recurrent or persistent uterine leiomyosarcoma: a Gynecologic Oncology Group phase II study. Gynecol Oncol 2009; 115(3): 460–465.
59. DeVita VT. Principles of Medical Oncology, In: DeVita VT, Lawrence TS, Rosenberg SA (eds). DeVita, Hellman, and Rosenberg’s Cancer: Principles & Practice of Oncology. 8th ed. Philadelphia: Lippincott Williams & Wilkins 2008.
60. NCCN Guidelines, Version 2.2012, Uterine neoplasms.
61. Sutton G, Blessing JA, Park R et al. 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): 747–750.
62. Sutton G, Kauderer J, Carson LF et al. Adjuvant ifosfamide and cisplatin in patients with completely resected stage I or II carcinosarcomas (mixed mesodermal tumors) of the uterus: a Gynecologic Oncology Group study. Gynecol Oncol 2005; 96(3): 630–634.
63. Homesley HD, Filiaci V, Markman M 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): 526–531.
64. Powell MA, Filiaci VL, Rose PG 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): 2727–2731.
65. Huh WK, Sill MW, Darcy KM et al. Efficacy and safety of imatinib mesylate (Gleevec) and immunohistochemical expression of c-Kit and PDGFR-beta in a Gynecologic Oncology Group Phase II Trial in women with recurrent or persistent carcinosarcomas of the uterus. Gynecol Oncol 2010; 117(2): 248–254.
66. Yi-Shin Kuo D, Timmins P, Blank SV 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): 160–165.
67. Bermudez RS, Huang K, Hsu IC. Endometrial Cancer. In: Hansen EK, Roach M (eds). Handbook of evidence-based radiation oncology. 2nd ed. New York: Springer Science + Business Media 2010: 513–526.
68. Schick U, Bolukbasi Y, Thariat J et al. Outcome and prognostic factors in endometrial stromal tumors: a rare cancer network study. Int J Radiat Oncol Biol Phys 2012; 82(5): e757–e763.
69. Reed NS, Mangioni C, Malstrom H et al. Phase III randomised study to evaluate role of adjuvant pelvic radiotherapy in the treatment of uterine sarcomas stages I and II: an EORTC Gynaecological Group Study (protocol 55874). Eur J Cancer 2008, 44(6): 808–818.
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Paediatric clinical oncology Surgery Clinical oncologyArticle was published in
Clinical Oncology
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