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Der Gynäkologe

, Volume 51, Issue 7, pp 563–571 | Cite as

Update zu uterinen Sarkomen

  • Helmut Plett
  • Mareike Bommert
  • Beyhan Ataseven
  • Florian Heitz
  • Philipp Harter
CME
  • 210 Downloads

Zusammenfassung

Uterine Sarkome bilden eine inhomogene Gruppe seltener Tumoren der Uterusmuskulatur. Es sind meist aggressive Neoplasien, die vom uterinen Bindegewebe oder vom endometrialen Stroma ausgehen. Histologisch werden folgende Subtypen unterschieden: das Leiomyosarkom (LMS), das low-grade endometriale Stromasarkom (LG-ESS) und das high-grade endometriale Stromasarkom (HG-ESS) sowie das undifferenzierte Stromasarkom (UES). Die Inzidenz liegt bei 0,5–3,3 pro 100.000 Frauen pro Jahr; insgesamt bilden sie mit 1–2 % nur einen kleinen Anteil der uterinen Malignome. Eine andere Kategorie bilden uterine Sarkome gemischten Ursprungs, wie Adenosarkome, Karzinosarkome (maligne Müller-Mischtumoren) und andere heterologe Sarkome (z. B. Chondrosarkome, Liposarkome, Fibrosarkome etc.). Im Beitrag wird auf das spezifische Management der häufigen Entitäten (LMS, LG-ESS, HG-ESS und UES) Bezug genommen. Im August 2015 wurde die erste Version einer S2k-Leitlinie zu uterinen Sarkomen herausgegeben.

Schlüsselwörter

Adenosarkom Leiomyosarkom Endometriales Stromasarkom  Karzinosarkom Uterine Neoplasien 

Update on uterine sarcoma

Abstract

Uterine sarcoma is a rare inhomogeneous entity, representing 1–2% of uterine malignancies. Only little is known about the epidemiology, survival and treatment options due to the rareness of this disease and the lack of prospective clinical studies. Compared to other entities sarcomas are among the most lethal gynecological malignancies. Histologically, the following subtypes are distinguished: leiomyosarcoma (uterine smooth muscle), endometrial stromal sarcoma (mesodermal origin) and other sarcomas of mixed histological origins, such as carcinosarcoma, adenosarcoma and fibrosarcoma. In this review article the focus is on the prognosis and current treatment options of leiomyosarcoma as well as high-grade and low-grade endometrial stromal sarcoma.

Keywords

Adenosarcoma Leiomyosarcoma Sarcoma, endometrial stromal Carcinosarcoma Uterine neoplasms 

Notes

Einhaltung ethischer Richtlinien

Interessenkonflikt

H. Plett, M. Bommert, B. Ataseven, F. Heitz und P. Harter geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

Literatur

  1. 1.
    Brooks SE, Zhan M, Cote T, Baquet CR (2004) Surveillance, epidemiology, and end results analysis of 2677 cases of uterine sarcoma 1989–1999. Gynecol Oncol 93:204–208CrossRefPubMedGoogle Scholar
  2. 2.
    D’Angelo E, Prat J (2010) Uterine sarcomas: a review. Gynecol Oncol 116:131–139CrossRefPubMedGoogle Scholar
  3. 3.
    Pietzner K, Buttmann-Schweiger N, Sehouli J, Kraywinkel K (2017) Incidence patterns and survival of gynecological sarcoma in Germany: analysis of population-based cancer registry data on 1066 women. Int J Gynecol Cancer 28:134.  https://doi.org/10.1097/IGC.0000000000001128 CrossRefGoogle Scholar
  4. 4.
    Pelmus M, Penault-Llorca F, Guillou L, Collin F, Bertrand G, Trassard M, Leroux A, Floquet A, Stoeckle E, Thomas L, MacGrogan G (2009) Prognostic factors in early-stage leiomyosarcoma of the uterus. Int J Gynecol Cancer 19:385–390CrossRefPubMedGoogle Scholar
  5. 5.
    Seagle B‑LL, Sobecki-Rausch J, Strohl AE, Shilpi A, Grace A, Shahabi S (2017) Prognosis and treatment of uterine leiomyosarcoma: a national cancer database study. Gynecol Oncol 145:61–70CrossRefPubMedGoogle Scholar
  6. 6.
    FDA_Safety_Communication_Laparoscopic_Morcellation_Hysterectomy.pdf. https://www.basg.gv.at/fileadmin/_migrated/content_uploads/FDA_Safety_Communication_Laparoscopic_Morcellation_Hysterectomy.pdf. Zugegriffen: 14.12.2017
  7. 7.
    Park J‑Y, Park S‑K, Kim D‑Y, Kim J‑H, Kim Y‑M, Kim Y‑T, Nam J‑H (2011) The impact of tumor morcellation during surgery on the prognosis of patients with apparently early uterine leiomyosarcoma. Gynecol Oncol 122:255–259CrossRefPubMedGoogle Scholar
  8. 8.
    Perri T, Korach J, Sadetzki S, Oberman B, Fridman E, Ben-Baruch G (2009) Uterine leiomyosarcoma: does the primary surgical procedure matter? Int J Gynecol Cancer 19:257–260CrossRefPubMedGoogle Scholar
  9. 9.
    Leitao MM, Sonoda Y, Brennan MF, Barakat RR, Chi DS (2003) Incidence of lymph node and ovarian metastases in leiomyosarcoma of the uterus. Gynecol Oncol 91:209–212CrossRefPubMedGoogle Scholar
  10. 10.
    Pautier P, Floquet A, Gladieff L, Bompas E, Ray-Coquard I, Piperno-Neumann S, Selle F, Guillemet C, Weber B, Largillier R, Bertucci F, Opinel P, Duffaud F, Reynaud-Bougnoux A, Delcambre C, Isambert N, Kerbrat P, Netter-Pinon G, Pinto N, Duvillard P, Haie-Meder C, Lhommé C, Rey A (2013) A randomized clinical trial of adjuvant chemotherapy with doxorubicin, ifosfamide, and cisplatin followed by radiotherapy versus radiotherapy alone in patients with localized uterine sarcomas (SARCGYN study). A study of the French Sarcoma Group. Ann Oncol 24:1099–1104CrossRefPubMedGoogle Scholar
  11. 11.
    Reed NS, Mangioni C, Malmström H, Scarfone G, Poveda A, Pecorelli S, Tateo S, Franchi M, Jobsen JJ, Coens C, Teodorovic I, Vergote I, Vermorken JB, European Organisation for Research and Treatment of Cancer Gynaecological Cancer Group (2008) 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 44:808–818CrossRefPubMedGoogle Scholar
  12. 12.
    Hensley ML, Ishill N, Soslow R, Larkin J, Abu-Rustum N, Sabbatini P, Konner J, Tew W, Spriggs D, Aghajanian CA (2009) Adjuvant gemcitabine plus docetaxel for completely resected stages I–IV high grade uterine leiomyosarcoma: results of a prospective study. Gynecol Oncol 112:563–567CrossRefPubMedGoogle Scholar
  13. 13.
    Hensley ML, Wathen JK, Maki RG, Araujo DM, Sutton G, Priebat DA, George S, Soslow RA, Baker LH (2013) Adjuvant therapy for high-grade, uterus-limited leiomyosarcoma: results of a phase 2 trial (SARC 005). Cancer 119:1555–1561CrossRefPubMedGoogle Scholar
  14. 14.
    Omura GA, Blessing JA, Major F, Lifshitz S, Ehrlich CE, Mangan C, Beecham J, Park R, Silverberg S (1985) A randomized clinical trial of adjuvant adriamycin in uterine sarcomas: a Gynecologic Oncology Group Study. J Clin Oncol 3:1240–1245CrossRefPubMedGoogle Scholar
  15. 15.
    Bernstein-Molho R, Grisaro D, Soyfer V, Safra T, Merimsky O (2010) Metastatic uterine leiomyosarcomas: a single-institution experience. Int J Gynecol Cancer 20:255–260CrossRefPubMedGoogle Scholar
  16. 16.
    Giuntoli RL, Garrett-Mayer E, Bristow RE, Gostout BS (2007) Secondary cytoreduction in the management of recurrent uterine leiomyosarcoma. Gynecol Oncol 106:82–88CrossRefPubMedGoogle Scholar
  17. 17.
    Seddon B, Scurr M, Jones RL, Wood Z, Propert-Lewis C, Fisher C, Flanagan A, Sunkersing J, A’Hern R, Whelan J, Judson I (2015) A phase II trial to assess the activity of gemcitabine and docetaxel as first line chemotherapy treatment in patients with unresectable leiomyosarcoma. Clin Sarcoma Res 5:13CrossRefPubMedPubMedCentralGoogle Scholar
  18. 18.
    Seddon B, Strauss SJ, Whelan J, Leahy M, Woll PJ, Cowie F, Rothermundt C, Wood Z, Benson C, Ali N, Marples M, Veal GJ, Jamieson D, Küver K, Tirabosco R, Forsyth S, Nash S, Dehbi H‑M, Beare S (2017) Gemcitabine and docetaxel versus doxorubicin as first-line treatment in previously untreated advanced unresectable or metastatic soft-tissue sarcomas (GeDDiS): a randomised controlled phase 3 trial. Lancet Oncol 18:1397–1410CrossRefPubMedPubMedCentralGoogle Scholar
  19. 19.
    Maki RG, Wathen JK, Patel SR, Priebat DA, Okuno SH, Samuels B, Fanucchi M, Harmon DC, Schuetze SM, Reinke D, Thall PF, Benjamin RS, Baker LH, Hensley ML (2007) 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 25:2755–2763CrossRefPubMedGoogle Scholar
  20. 20.
    Harter P, Sehouli J, Reuss A, Baumann K, Hanker L, Kimmig R, Schröder W, Burges A, Gropp-Meier M, Kurzeder C, Mahner S, Canzler U, Lück H‑J, Meier W, Fehm T, du Bois A (2016) Phase II study evaluating pegLiposomal doxorubicin and carboplatin combination chemotherapy in gynecologic sarcomas and mixed epithelial-mesenchymal tumors A phase II protocol of the Arbeitsgemeinschaft Gynaekologische Onkologie study group (AGO-GYN 7). Int J Gynecol Cancer 26:1636–1641CrossRefPubMedGoogle Scholar
  21. 21.
    Gadducci A, Guerrieri ME (2015) Pharmacological treatment for uterine leiomyosarcomas. Expert Opin Pharmacother 16:335–346CrossRefPubMedGoogle Scholar
  22. 22.
    Hensley ML, Patel SR, von Mehren M, Ganjoo K, Jones RL, Staddon A, Rushing D, Milhem M, Monk B, Wang G, McCarthy S, Knoblauch RE, Parekh TV, Maki RG, Demetri GD (2017) Efficacy and safety of trabectedin or dacarbazine in patients with advanced uterine leiomyosarcoma after failure of anthracycline-based chemotherapy: Subgroup analysis of a phase 3, randomized clinical trial. Gynecol Oncol 146:531–537CrossRefPubMedPubMedCentralGoogle Scholar
  23. 23.
    Demetri GD, von Mehren M, Jones RL, Hensley ML, Schuetze SM, Staddon A, Milhem M, Elias A, Ganjoo K, Tawbi H, Van Tine BA, Spira A, Dean A, Khokhar NZ, Park YC, Knoblauch RE, Parekh TV, Maki RG, Patel SR (2016) 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 34:786–793CrossRefPubMedGoogle Scholar
  24. 24.
    Pautier P, Floquet A, Chevreau C, Penel N, Guillemet C, Delcambre C, Cupissol D, Selle F, Isambert N, Piperno-Neumann S, Thyss A, Bertucci F, Bompas E, Alexandre J, Collard O, Lavau-Denes S, Soulié P, Toulmonde M, Le Cesne A, Lacas B, Duffaud F, French Sarcoma Group (2015) 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 16:457–464CrossRefPubMedGoogle Scholar
  25. 25.
    van der Graaf WTA, Blay J‑Y, Chawla SP, Kim D‑W, Bui-Nguyen B, Casali PG, Schöffski P, Aglietta M, Staddon AP, Beppu Y, Le Cesne A, Gelderblom H, Judson IR, Araki N, Ouali M, Marreaud S, Hodge R, Dewji MR, Coens C, Demetri GD, Fletcher CD, Dei Tos AP, Hohenberger P, Soft Tissue EORTC, EORTC Soft Tissue and Bone Sarcoma Group, PALETTE study group (2012) Pazopanib for metastatic soft-tissue sarcoma (PALETTE): a randomised, double-blind, placebo-controlled phase 3 trial. Lancet 379:1879–1886CrossRefPubMedGoogle Scholar
  26. 26.
    Tap WD, Jones RL, Van Tine BA, Chmielowski B, Elias AD, Adkins D, Agulnik M, Cooney MM, Livingston MB, Pennock G, Hameed MR, Shah GD, Qin A, Shahir A, Cronier DM, Ilaria R, Conti I, Cosaert J, Schwartz GK (2016) Olaratumab and doxorubicin versus doxorubicin alone for treatment of soft-tissue sarcoma: an open-label phase 1b and randomised phase 2 trial. Lancet 388:488–497CrossRefPubMedPubMedCentralGoogle Scholar
  27. 27.
    Ali RH, Rouzbahman M (2015) Endometrial stromal tumours revisited: an update based on the 2014 WHO classification. J Clin Pathol 68:325–332CrossRefPubMedGoogle Scholar
  28. 28.
    Chan JK, Kawar NM, Shin JY, Osann K, Chen L‑M, Powell CB, Kapp DS (2008) Endometrial stromal sarcoma: a population-based analysis. Br J Cancer 99:1210–1215CrossRefPubMedPubMedCentralGoogle Scholar
  29. 29.
    Barney B, Tward JD, Skidmore T, Gaffney DK (2009) Does radiotherapy or lymphadenectomy improve survival in endometrial stromal sarcoma? Int J Gynecol Cancer 19:1232–1238CrossRefPubMedGoogle Scholar
  30. 30.
    Seagle B‑LL, Shilpi A, Buchanan S, Goodman C, Shahabi S (2017) Low-grade and high-grade endometrial stromal sarcoma: A National Cancer Database study. Gynecol Oncol 146:254–262CrossRefPubMedGoogle Scholar
  31. 31.
    Bai H, Yang J, Cao D, Huang H, Xiang Y, Wu M, Cui Q, Chen J, Lang J, Shen K (2014) Ovary and uterus-sparing procedures for low-grade endometrial stromal sarcoma: a retrospective study of 153 cases. Gynecol Oncol 132:654–660CrossRefPubMedGoogle Scholar
  32. 32.
    Signorelli M, Fruscio R, Dell’Anna T, Buda A, Giuliani D, Ceppi L, Milani R (2010) Lymphadenectomy in uterine low-grade endometrial stromal sarcoma: an analysis of 19 cases and a literature review. Int J Gynecol Cancer 20:1363–1366PubMedGoogle Scholar
  33. 33.
    Chu MC, Mor G, Lim C, Zheng W, Parkash V, Schwartz PE (2003) Low-grade endometrial stromal sarcoma: hormonal aspects. Gynecol Oncol 90:170–176CrossRefPubMedGoogle Scholar
  34. 34.
    Gadducci A, Cosio S, Romanini A, Genazzani AR (2008) The management of patients with uterine sarcoma: a debated clinical challenge. Crit Rev Oncol Hematol 65:129–142CrossRefPubMedGoogle Scholar
  35. 35.
    Amant F, Coosemans A, Debiec-Rychter M, Timmerman D, Vergote I (2009) Clinical management of uterine sarcomas. Lancet Oncol 10:1188–1198CrossRefPubMedGoogle Scholar
  36. 36.
    Pautier P, Nam EJ, Provencher DM, Hamilton AL, Mangili G, Siddiqui NA, Westermann AM, Reed NS, Harter P, Ray-Coquard I (2014) Gynecologic Cancer InterGroup (GCIG) consensus review for high-grade undifferentiated sarcomas of the uterus. Int J Gynecol Cancer 24:S73–S77CrossRefPubMedGoogle Scholar
  37. 37.
    Schick U, Bolukbasi Y, Thariat J, Abdah-Bortnyak R, Kuten A, Igdem S, Caglar H, Ozsaran Z, Lössl K, Schleicher U, Zwahlen D, Villette S, Vees H (2012) Outcome and prognostic factors in endometrial stromal tumors: a Rare Cancer Network study. Int J Radiat Oncol Biol Phys 82:e757–e763CrossRefPubMedGoogle Scholar
  38. 38.
    Leath CA, Huh WK, Hyde J, Cohn DE, Resnick KE, Taylor NP, Powell MA, Mutch DG, Bradley WH, Geller MA, Argenta PA, Gold MA (2007) A multi-institutional review of outcomes of endometrial stromal sarcoma. Gynecol Oncol 105:630–634CrossRefPubMedGoogle Scholar
  39. 39.
    Lewis N, Soslow RA, Delair DF, Park KJ, Murali R, Hollmann TJ, Davidson B, Micci F, Panagopoulos I, Hoang LN, Arias-Stella JA, Oliva E, Young RH, Hensley ML, Leitao MM, Hameed M, Benayed R, Ladanyi M, Frosina D, Jungbluth AA, Antonescu CR, Chiang S (2018) ZC3H7B-BCOR high-grade endometrial stromal sarcomas: a report of 17 cases of a newly defined entity. Mod Pathol 31(4):674.  https://doi.org/10.1038/modpathol.2017.162 CrossRefPubMedGoogle Scholar
  40. 40.
    Caudell JJ, Deavers MT, Slomovitz BM, Lu KH, Broaddus RR, Gershenson DM, Ramondetta LM (2005) Imatinib mesylate (gleevec) – targeted kinases are expressed in uterine sarcomas. Appl Immunohistochem Mol Morphol 13:167–170CrossRefPubMedGoogle Scholar
  41. 41.
    Coosemans A, Nik SA, Caluwaerts S, Lambin S, Verbist G, Van Bree R, Schelfhout V, de Jonge E, Dalle I, Jacomen G, Cassiman JJ, Moerman P, Vergote I, Amant F (2007) Upregulation of Wilms’ tumour gene 1 (WT1) in uterine sarcomas. Eur J Cancer 43:1630–1637CrossRefPubMedGoogle Scholar

Copyright information

© Springer Medizin Verlag GmbH, ein Teil von Springer Nature 2018

Authors and Affiliations

  • Helmut Plett
    • 1
  • Mareike Bommert
    • 1
  • Beyhan Ataseven
    • 1
  • Florian Heitz
    • 1
  • Philipp Harter
    • 1
  1. 1.Evangelische Huyssens-Stiftung/Knappschaft GmbH, Klinik für Gynäkologie & Gynäkologische OnkologieKliniken Essen-MitteEssenDeutschland

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