Myxoid\Round Cell Liposarcoma (MRCLS) Revisited: An Analysis of 418 Primarily Managed Cases
Rent the article at a discountRent now
* Final gross prices may vary according to local VAT.Get Access
Objectives of this study were to evaluate oncologic outcomes and to provide guidelines for the management of primary myxoid (MLS) and round cell liposarcoma (RCLS).
A multicenter, retrospective study of 418 cases of MRCLS primarily managed by Canadian multidisciplinary sarcoma teams.
Study included 418 cases (MLS: 311 patients and RCLS: 107; > 5% round cell) with a median age of 45 years and a median follow-up of 5.2 years. Median tumor size was 10 cm, and 81% were deep and 90% were in lower limb. The majority of patients underwent surgical resection and radiotherapy, with a small percentage (6%) receiving chemotherapy. The overall 10-year local control rate was 93% with no differences between MLS and RCLS. Radiotherapy was significant in preventing local relapse and reducing tumor diameter (median = 18%) and improving microscopic margin status, but did not impact survival. Radiotherapy and the margin status were independent predictors of local recurrence. The 5- and 10-year metastatic-free survivals were 84 and 77% respectively for MLS and 69 and 46% for RCLS. The initial site of metastasis was found in multiple locations (34%) and bone involvement was frequent (40%) with predilection for spine (79%). Round cell percent (> 5%) and tumor diameter (> 10 cm) correlated with increased risk for metastasis and death.
MLS and RCLS showed different metastatic risk but equally good local control. Radiotherapy was effective in preventing local recurrence and should be delivered as neoadjuvant. New staging strategies are to be defined to account for the unusual metastatic pattern.
- Weiss SW, Goldblum JR. Enzinger and Weiss’s soft tissue tumors, 5th edn. St. Louis: Mosby; 2007.
- World Health Organization. International histological classification of tumours, 2nd edn. Berlin: Springer-Verlag; 2002.
- Antonescu CR, Elahi A, Humphrey M, et al. Specificity of TLS-CHOP rearrangement for classic myxoid/round cell liposarcoma. J Molec Diagn. 2000;2(3):132–8. CrossRef
- Antonescu CR, Tschernyavsky SJ, Decuseara R, et al. Prognostic impact of P53 status, TLS-CHOP fusion transcript structure, and histological grade in myxoid liposarcoma: a molecular and clinicopathologic study of 82 cases. Clin Cancer Res. 2001;7:3977–87.
- Panagopoulos I, Hoglund M, Mertens F, Mandahl N, Mitelman F, Aman P. Fusion of the EWS and CHOP genes in myxoid liposarcoma. Oncogene. 1996;12:489–94.
- Dal Cin P, Sciot R, Panagopoulos I, et al. Additional evidence of a variant translocation t(12;22) with EWS-CHOP fusion in myxoid liposarcoma: clinicopathologic features. J Pathol. 1997;182:437–41. CrossRef
- Fiore M, Grosso F, Lo Vullo S, et al. Myxoid/round cell and pleomorphic liposarcomas: prognostic factors and survival in a series of patients treated at a single institution. Cancer. 2007;109(12):2522–31. CrossRef
- Nishida Y, Tsukushi S, Nakashima H, Ishiguro N. Clinicopathologic prognostic factors of pure myxoid liposarcoma of the extremities and trunk wall. Clin Orthop Rel Res. 2010;468(11):3041–6. CrossRef
- Kilpatrick SE, Doyon J, Choong PF, Sim FH, Nascimento AG. The clinicopathologic spectrum of myxoid and round cell liposarcoma. Cancer. 1996;77(8):1450–8. CrossRef
- Alaggio R, Coffin CM, Weiss SW, Bridge JA, Issakov J, Oliveira AM, Folpe AL. Liposarcomas in young patients: a study of 82 cases occurring in patients younger than 22 years of age. Am J Surg Pathol. 2009;33(5):645–58. CrossRef
- Smith TA, Easley KA, Goldblum JR. Myxoid/round cell liposarcoma of the extremities: a clinicopathologic study of 29 cases with particular attention to extent of round cell liposarcoma. Am J Surg Pathol. 1996;20(2):171–80. CrossRef
- Singer S, Antonescu CR, Riedel E, Brennan MF. Histologic subtype and margin of resection predict pattern of recurrence and survival for retroperitoneal liposarcoma. Ann Surg. 2003;238(3):358–70.
- Ten Heuvel SE, Hoekstra HJ, van Ginkel RJ, Bastiaannet E, Suurmeijer AJ. Clinicopathologic prognostic factors in myxoid liposarcoma: a retrospective study of 49 patients with long-term follow-up. Ann Surg Oncol. 2007;14(1):222–9. CrossRef
- Spillane AJ, Fisher C, Thomas JM. Myxoid liposarcoma–frequency and the natural history of nonpulmonary soft tissue metastases. Ann Surg Oncol. 1999;6(4):389–94. CrossRef
- Sheah K, Ouellette HA, Torriani M, Nielsen GP, Kattapuram S, Bredella MA. Metastatic myxoid liposarcomas: imaging and histopathologic findings. Skeletal Radiol. 2008;37(3):251–8. CrossRef
- Zagars GK, Goswitz MS, Pollack A. Liposarcoma: outcome and prognostic factors following conservation surgery and radiation therapy. Int J Radiat Oncol. 1996;36(2):311–9. CrossRef
- Pisters PW, Sondack VK. Metastatic patterns of extremity liposarcoma and their outcome. J Surg Oncol. 2002;80(2):94–5. CrossRef
- Estourgie SH, Nielsen GP, Ott MJ. Metastatic patterns of extremity myxoid liposarcoma and their outcome. J Surg Oncol. 2002;80(2):89–93. CrossRef
- Schwab JH, Boland PJ, Antonescu C, Bilsky MH, Healey JH. Spinal metastases from myxoid liposarcoma warrant screening with magnetic resonance imaging. Cancer. 2007;110(8):1815–22. CrossRef
- 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. CrossRef
- O’Sullivan B, Davis AM, Turcotte RE, et al. Preoperative versus postoperative radiotherapy in soft-tissue sarcoma of the limbs: a randomised trial. Lancet. 2002;359(9325):2235–41. CrossRef
- Guadagnolo BA, Zagars GK, Ballo MT, Patel SR, Lewis VO, Benjamin RS, Pollock RE. Excellent local control rates and distinctive patterns of failure in myxoid liposarcoma treated with conservation surgery and radiotherapy. Int J Radiat Oncol Biol Phys. 2008;70(3):760–5. CrossRef
- Engstrom K. Bergh P. Cederlund CG, et al. Irradiation of myxoid/round cell liposarcoma induces volume reduction and lipoma-like morphology. Acta Oncologica. 2007;46(6):838–45. CrossRef
- Jones RL, Fisher C, Al-Muderis O, Judson IR. Differential sensitivity of liposarcoma subtypes to chemotherapy. Eur J Cancer. 2005;41(18):2853–60. CrossRef
- Blay JY, Von Mehren M, Samuels, BL, et al. Phase 1 combination study of trabectedin and doxorubicin in patients with soft-tissue sarcoma. Clinical Cancer Res. 2008;14:6656–62. CrossRef
- 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;27:4188–96. CrossRef
- Germano G, Frapolli R, Simone M, et al. Antitumor and anti-inflammatory effects of trabectedin on human myxoid liposarcoma cells. Cancer Res. 2010;70:2235–44. CrossRef
- de Vreeze RS, de Jong D, Tielen IH, Ruijter HJ, Nederlof PM, Haas RL, van Coevorden F. Primary retroperitoneal myxoid liposarcoma is a non-existing disease: an immunohistochemical and molecular biological analysis. Mod Pathol. 2009;22(2):223–31. CrossRef
- Trojani M, Contesso G, Coindre JM, et al. Soft tissue sarcomas of adults: study of pathological prognostic variables and definition of a histopathologic grading system. Int J Cancer. 1984;33:37–42. CrossRef
- Edge SB, Byrd DR, Compton CC, Fritz AG, Greene FL, Trotti A, eds. American Joint Committee on Cancer staging manual, 7th edn. New York: Springer; 2009.
- Jager PL, Hoekstra HJ, Leeuw J, van Der Graaf WT, de Vries EG, Piers D. Routine bone scintigraphy in primary staging of soft tissue sarcoma. Is it worthwhile? Cancer. 2000;89(8):1726–31. CrossRef
- Engström K, Bergh P, Gustafson P, et al. Liposarcoma: outcome based on the Scandinavian Sarcoma Group register. Cancer. 2008;113:1649–56. CrossRef
- Roberge D, Skamene T, Nahal A, Turcotte RE, Powell T, Freeman C. Radiological and pathological response following pre-operative radiotherapy for soft-tissue sarcoma. Radiother Oncol. 2010;97(3):404–7. CrossRef
- Pitson G, Robinson P, Wilke D, et al. Radiation response: an additional unique signature of myxoid liposarcoma. Int J Radiat Oncol Biol Phys. 2004;60(2):522–6. CrossRef
- Davis AM, O’Sullivan B, Turcotte RE, et al. Late radiation morbidity following preoperative versus postoperative radiotherapy in extremity soft tissue sarcoma. Radiother Oncol. 2005;75(1):48–53. CrossRef
- Ghert MA, Abudu A, Driver N, et al. The indications for and the prognostic significance of amputation as the primary surgical procedure for localized soft tissue sarcoma of the extremity. Ann Surg Oncol. 2005;75(1):48–53.
- Myxoid\Round Cell Liposarcoma (MRCLS) Revisited: An Analysis of 418 Primarily Managed Cases
Annals of Surgical Oncology
Volume 19, Issue 4 , pp 1081-1088
- Cover Date
- Print ISSN
- Online ISSN
- Additional Links
- liposarcoma myxoid
- Metastasis bone
- Industry Sectors
- Author Affiliations
- 1. Orthopaedic Surgery, McGill University Health Centre, Montreal, Quebec, Canada
- 2. Orthopaedic Surgery, Mount Sinai Hospital, Toronto, Ontario, Canada
- 3. Orthopaedic Surgery, Princess Margaret Hospital, Toronto, Ontario, Canada
- 4. Orthopaedics, University of British Columbia, Vancouver, Canada
- 5. Surgical Oncology, British Columbia Cancer Agency, Vancouver, Canada
- 6. Orthopaedic Surgery, Vancouver General Hospital, Vancouver, British Columbia, Canada
- 7. Orthopaedic Surgery, Hôpital Maisonneuve-Rosemont, Montreal, Quebec, Canada
- 8. Orthopaedic Surgery, Hôtel-Dieu, Quebec City, Quebec, Canada
- 9. Orthopaedic Surgery, The Ottawa Hospital, Ottawa, Ontario, Canada
- 10. Orthopaedic Surgery, Juravinski Cancer Centre, Hamilton, Ontario, Canada