• Murray F. Brennan
  • Cristina R. Antonescu
  • Kaled M. Alektiar
  • Robert G. Maki


Liposarcoma is primarily a tumor that occurs) with peak incidence between ages 50 and 70 and equal gender distribution (Fig. 5.1). As described previously (see Chap.  1, Fig.  1.6), liposarcomas account for approximately 20 % of all soft tissue sarcomas in adults. Anatomic distribution of liposarcoma is wide (Fig. 5.2) and is usually considered to manifest in three biological subtypes. The most common type is well-differentiated liposarcoma (sometimes called atypical lipomatous tumor [ALT]), and its high-grade variant dedifferentiated liposarcoma. The second most common is myxoid (low grade) and round cell (high-grade) liposarcoma. The least common is (high-grade) pleomorphic liposarcoma. Each subtype has a very distinctive morphology, natural history, and genetic changes utilized in diagnosis.


Well differentiated liposarcoma Dedifferentiated liposarcoma Myxoid liposarcoma Round cell liposarcoma Pleomorphic liposarcoma MDM2 CDK4 


  1. 1.
    Kooby DA, Antonescu CR, Brennan MF, et al. Atypical lipomatous tumor/well-differentiated liposarcoma of the extremity and trunk wall: importance of histological subtype with treatment recommendations. Ann Surg Oncol. 2004;11(1):78–84.CrossRefPubMedGoogle Scholar
  2. 2.
    Russo P, Brady MS, Conlon K, et al. Adult urological sarcoma. J Urol. 1992;147(4):1032–6. discussion 1036-1037.PubMedGoogle Scholar
  3. 3.
    Shibata D, Lewis JJ, Leung DH, et al. Is there a role for incomplete resection in the management of retroperitoneal liposarcomas? J Am Coll Surg. 2001;193(4):373–9.CrossRefPubMedGoogle Scholar
  4. 4.
    Alektiar KM, Hu K, Anderson L, et al. High-dose-rate intraoperative radiation therapy (HDR-IORT) for retroperitoneal sarcomas. Int J Radiat Oncol Biol Phys. 2000;47(1):157–63.CrossRefPubMedGoogle Scholar
  5. 5.
    Al Yami A, Griffin AM, Ferguson PC, et al. Positive surgical margins in soft tissue sarcoma treated with preoperative radiation: is a postoperative boost necessary? Int J Radiat Oncol Biol Phys. 2010;77(4):1191–7.CrossRefPubMedGoogle Scholar
  6. 6.
    Chung PW, Deheshi BM, Ferguson PC, et al. Radiosensitivity translates into excellent local control in extremity myxoid liposarcoma: a comparison with other soft tissue sarcomas. Cancer. 2009;115(14):3254–61.CrossRefPubMedGoogle Scholar
  7. 7.
    Schwab JH, Boland PJ, Antonescu C, et al. Spinal metastases from myxoid liposarcoma warrant screening with magnetic resonance imaging. Cancer. 2007;110(8):1815–22.CrossRefPubMedGoogle Scholar
  8. 8.
    Schwab JH, Boland P, Guo T, et al. Skeletal metastases in myxoid liposarcoma: an unusual pattern of distant spread. Ann Surg Oncol. 2007;14(4):1507–14.CrossRefPubMedGoogle Scholar
  9. 9.
    Verweij J, Baker LH. Future treatment of soft tissue sarcomas will be driven by histological subtype and molecular aberrations. Eur J Cancer. 2010;46(5):863–8.CrossRefPubMedGoogle Scholar
  10. 10.
    Romeo S, Dei Tos AP. Soft tissue tumors associated with EWSR1 translocation. Virchows Arch. 2010;456(2):219–34.CrossRefPubMedGoogle Scholar
  11. 11.
    Rieker RJ, Weitz J, Lehner B, et al. Genomic profiling reveals subsets of dedifferentiated liposarcoma to follow separate molecular pathways. Virchows Arch. 2010;456(3):277–85.CrossRefPubMedGoogle Scholar
  12. 12.
    Guillou L, Aurias A. Soft tissue sarcomas with complex genomic profiles. Virchows Arch. 2010;456(2):201–17.CrossRefPubMedGoogle Scholar
  13. 13.
    Pires de Camargo V, van de Rijn M, Maestro R, et al. Other targetable sarcomas. Semin Oncol. 2009;36(4):358–71.CrossRefPubMedGoogle Scholar
  14. 14.
    Goransson M, Andersson MK, Forni C, et al. The myxoid liposarcoma FUS-DDIT3 fusion oncoprotein deregulates NF-kappaB target genes by interaction with NFKBIZ. Oncogene. 2009;28(2):270–8.CrossRefPubMedGoogle Scholar
  15. 15.
    Italiano A, Bianchini L, Keslair F, et al. HMGA2 is the partner of MDM2 in well-differentiated and dedifferentiated liposarcomas whereas CDK4 belongs to a distinct inconsistent amplicon. Int J Cancer. 2008;122(10):2233–41.CrossRefPubMedGoogle Scholar
  16. 16.
    Italiano A, Cardot N, Dupre F, et al. Gains and complex rearrangements of the 12q13-15 chromosomal region in ordinary lipomas: the “missing link” between lipomas and liposarcomas? Int J Cancer. 2007;121(2):308–15.CrossRefPubMedGoogle Scholar
  17. 17.
    Antonescu CR. The role of genetic testing in soft tissue sarcoma. Histopathology. 2006;48(1):13–21.CrossRefPubMedGoogle Scholar
  18. 18.
    Dei Tos AP. Liposarcoma: new entities and evolving concepts. Ann Diagn Pathol. 2000;4(4):252–66.CrossRefPubMedGoogle Scholar
  19. 19.
    Mertens F, Fletcher CD, Dal Cin P, et al. Cytogenetic analysis of 46 pleomorphic soft tissue sarcomas and correlation with morphologic and clinical features: a report of the CHAMP Study Group. Chromosomes and MorPhology. Genes Chromosomes Cancer. 1998;22(1):16–25.CrossRefPubMedGoogle Scholar
  20. 20.
    Turc-Carel C, Limon J, Dal Cin P, et al. Cytogenetic studies of adipose tissue tumors. II. Recurrent reciprocal translocation t(12;16)(q13;p11) in myxoid liposarcomas. Cancer Genet Cytogenet. 1986;23(4):291–9.CrossRefPubMedGoogle Scholar
  21. 21.
    Jones RL, Fisher C, Al-Muderis O, et al. Differential sensitivity of liposarcoma subtypes to chemotherapy. Eur J Cancer. 2005;41(18):2853–60.CrossRefPubMedGoogle Scholar
  22. 22.
    Eilber FC, Eilber FR, Eckardt J, et al. The impact of chemotherapy on the survival of patients with high-grade primary extremity liposarcoma. Ann Surg. 2004;240(4):686–95. discussion 695-687.PubMedPubMedCentralGoogle Scholar
  23. 23.
    Patel SR, Burgess MA, Plager C, et al. Myxoid liposarcoma. Experience with chemotherapy. Cancer. 1994;74(4):1265–9.CrossRefPubMedGoogle Scholar
  24. 24.
    Pisters PW, O’Sullivan B, Maki RG. Evidence-based recommendations for local therapy for soft tissue sarcomas. J Clin Oncol. 2007;25(8):1003–8.CrossRefPubMedGoogle Scholar
  25. 25.
    Pervaiz N, Colterjohn N, Farrokhyar F, et al. A systematic meta-analysis of randomized controlled trials of adjuvant chemotherapy for localized resectable soft-tissue sarcoma. Cancer. 2008;113(3):573–81.CrossRefPubMedGoogle Scholar
  26. 26.
    Dalal KM, Kattan MW, Antonescu CR, et al. Subtype specific prognostic nomogram for patients with primary liposarcoma of the retroperitoneum, extremity, or trunk. Ann Surg. 2006;244(3):381–91.PubMedPubMedCentralGoogle Scholar
  27. 27.
    Grosso F, Sanfilippo R, Virdis E, et al. Trabectedin in myxoid liposarcomas (MLS): a long-term analysis of a single-institution series. Ann Oncol. 2009;20(8):1439–44.CrossRefPubMedGoogle Scholar
  28. 28.
    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. 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. 2016;34(8):786–93.CrossRefPubMedGoogle Scholar
  29. 29.
    Schöffski P, Chawla S, Maki RG, Italiano A, et al. Eribulin versus dacarbazine in previously treated patients with advanced liposarcoma or leiomyosarcoma: a randomised, open-label, multicentre, phase 3 trial. Lancet. 2016;387:1629–37.Google Scholar
  30. 30.
    Maki RG, Wathen JK, Patel SR, 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.CrossRefPubMedGoogle Scholar
  31. 31.
    Chawla SP, Tolcher AW, Sankhala KK, et al. Updated interim results of a phase 2 study of the mTOR inhibitor AP23573 in patients with advanced sarcomas of soft tissue or bone. Connective Tissue Oncology Society 15th Annual Meeting, Vol. 15. Boca Raton, 2005. pp. Abstract 447.Google Scholar
  32. 32.
    Chugh R, Wathen JK, Maki RG, et al. Phase II multicenter trial of imatinib in 10 histologic subtypes of sarcoma using a bayesian hierarchical statistical model. J Clin Oncol. 2009;27(19):3148–53.CrossRefPubMedGoogle Scholar
  33. 33.
    Maki RG, D’Adamo DR, Keohan ML, et al. Phase II study of sorafenib in patients with metastatic or recurrent sarcomas. J Clin Oncol. 2009;27(19):3133–40.CrossRefPubMedPubMedCentralGoogle Scholar
  34. 34.
    Sleijfer S, Ray-Coquard I, Papai Z, et al. Pazopanib, a multikinase angiogenesis inhibitor, in patients with relapsed or refractory advanced soft tissue sarcoma: a phase II study from the European Organisation for Research and Treatment of Cancer-Soft Tissue and Bone Sarcoma Group (EORTC study 62043). J Clin Oncol. 2009;27(19):3126–32.CrossRefPubMedGoogle Scholar
  35. 35.
    Dickson MA, Tap WD, Keohan ML, et al. Phase II trial of the CDK4 inhibitor PD0332991 in patients with advanced CDK4-amplified well-differentiated or dedifferentiated liposarcoma. J Clin Oncol. 2013;31(16):2024–8.CrossRefPubMedPubMedCentralGoogle Scholar
  36. 36.
    Tawbi HA-H, Burgess MA, Crowley J, et al. Safety and efficacy of PD-1 lockade using pembrolizumab in patients with advanced soft tissue (STS) and bone sarcomas (BS): Results of SARC028--A multicenter Phase II study. ASCO Meeting Abstracts. 2016;34:11006.Google Scholar
  37. 37.
    Grosso F, Jones RL, Demetri GD, et al. Efficacy of trabectedin (ecteinascidin-743) in advanced pretreated myxoid liposarcomas: a retrospective study. Lancet Oncol. 2007;8(7):595–602.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing Switzerland 2016

Authors and Affiliations

  • Murray F. Brennan
    • 1
  • Cristina R. Antonescu
    • 2
  • Kaled M. Alektiar
    • 3
  • Robert G. Maki
    • 4
  1. 1.International Center Department of SurgeryMemorial Sloan Kettering Cancer CenterNew YorkUSA
  2. 2.Department of PathologyMemorial Sloan Kettering Cancer CenterNew YorkUSA
  3. 3.Department of Radiation OncologyMemorial Sloan Kettering Cancer CenterNew YorkUSA
  4. 4.Monter Cancer CenterHofstra Northwell School of Medicine and Cold Spring Harbor LaboratoryLake SuccessUSA

Personalised recommendations