Annals of Surgical Oncology

, Volume 21, Issue 1, pp 269–276 | Cite as

Epithelioid Sarcoma: Need for a Multimodal Approach to Maximize the Chances of Curative Conservative Treatment

  • Antonin Levy
  • Cécile Le Péchoux
  • Philippe Terrier
  • Ryan Bouaita
  • Julien Domont
  • Olivier Mir
  • Sarah Coppola
  • Charles Honoré
  • Axel Le Cesne
  • Sylvie BonvalotEmail author
Bone and Soft Tissue Sarcomas



This study was designed to evaluate the impact of multimodal management on a series of epithelioid sarcoma (ES) patients treated with curative intent.


Data were collected on 69 consecutive patients treated from 1982 to 2012. Univariate and multivariate analyses were performed for tumor control and overall survival (OS).


In total, 54 (78 %) patients had localized ES (M0 group). In the M0 group, 85 % of patients received multimodal management (surgery n = 50, radiotherapy n = 37, chemotherapy n = 30). Among 42 patients with limb ES, 9 (21 %) underwent amputation, and isolated limb perfusion (ILP) was required in 17 (40.5 %) to allow conservative management. Among the 45 patients who underwent conservative surgery, flap reconstructions were required in 13 (28.8 %). The median follow-up was 5.7 years. The 5-year actuarial OS rates were 54, 62, and 24 % in the entire group and the M0 and M1 groups, respectively. In the M0 group, the 5-year actuarial distant control, local control (LC), and locoregional control rates were 67, 75, and 66 %, respectively. Prognostic factors for poor OS in the multivariate analysis were tumors that were deep to the fascia (p = 0.04) and grade 3 (p = 0.005). In the univariate analysis, age <30 years (p = 0.04), the T2 stage (p = 0.04), and mass presentation (p = 0.03) correlated with decreased LC, whereas patients who underwent ILP had a significantly higher LC rate (hazard ratio 3; 95 % confidence interval 0.9–9.4; p = 0.05).


Multimodal management including ILP and flap reconstruction is necessary to achieve optimal conservative LC. High rates of metastasis and lymphatic spread require innovative systemic treatments.


Overall Survival Sentinel Lymph Node Biopsy Flap Reconstruction Isolate Limb Perfusion Epithelioid Sarcoma 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.



The authors declare no financial support and no funds received.

Supplementary material

10434_2013_3247_MOESM1_ESM.doc (26 kb)
Supplementary material 1 (DOC 26 kb)
10434_2013_3247_MOESM2_ESM.doc (28 kb)
Supplementary material 2 (DOC 27 kb)


  1. 1.
    Mastrangelo G, Coindre JM, Ducimetière F, et al. Incidence of soft tissue sarcoma and beyond: a population-based prospective study in 3 European regions. Cancer. 2012;118:5339–48.PubMedCrossRefGoogle Scholar
  2. 2.
    Laskowski J. Sarcoma aponeuroticum. Nowotory. 1961;11:61–7.Google Scholar
  3. 3.
    Enzinger FM. Epitheloid sarcoma. A sarcoma simulating a granuloma or a carcinoma. Cancer. 1970;26:1029–41.PubMedCrossRefGoogle Scholar
  4. 4.
    Armah HB, Parwani AV. Epithelioid sarcoma. Arch Pathol Lab Med. 2009;133:814–9.PubMedGoogle Scholar
  5. 5.
    Dei Tos AP, Wagner AJ, Modena P, et al. Epithelioid soft tissue tumors. Semin Oncol. 2009; 36:347–57.PubMedCrossRefGoogle Scholar
  6. 6.
    Spillane AJ, Thomas JM, Fisher C. Epithelioid sarcoma: the clinicopathological complexities of this rare soft tissue sarcoma. Ann Surg Oncol. 2000;7:218–25.PubMedCrossRefGoogle Scholar
  7. 7.
    de Visscher SA, van Ginkel RJ, Wobbes T, et al. Epithelioid sarcoma: still an only surgically curable disease. Cancer. 2006;107:606–12.PubMedCrossRefGoogle Scholar
  8. 8.
    Zagars GK, Ballo MT, Pisters PW, et al. Prognostic factors for patients with localized soft-tissue sarcoma treated with conservation surgery and radiation therapy: an analysis of 1,225 patients. Cancer. 2003; 97:2530–43.PubMedCrossRefGoogle Scholar
  9. 9.
    ESMO/European Sarcoma Network Working Group. Soft tissue and visceral sarcomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2012;23S:vii92–9.Google Scholar
  10. 10.
    Kraybill WG, Harris J, Spiro IJ, et al. Long-term results of a phase 2 study of neoadjuvant chemotherapy and radiotherapy in the management of high-risk, high-grade, soft tissue sarcomas of the extremities and body wall: Radiation Therapy Oncology Group Trial 9514. Cancer. 2010;116:4613–21.PubMedCentralPubMedCrossRefGoogle Scholar
  11. 11.
    Grunhagen DJ, de Wilt JH, Graveland WJ, et al. Outcome and prognostic factor analysis of 217 consecutive isolated limb perfusions with tumor necrosis factor-alpha and melphalan for limb-threatening soft tissue sarcoma. Cancer. 2006;106:1776.PubMedCrossRefGoogle Scholar
  12. 12.
    Bonvalot S, Laplanche A, Lejeune F, Stoeckle E, Le Péchoux C, Vanel D, Terrier P, Lumbroso J, Ricard M, Antoni G, Cavalcanti A, Robert C, Lassau N, Blay JY, Le Cesne A. Limb salvage with isolated perfusion for soft tissue sarcoma: could less TNF-alpha be better? Ann Oncol. 2005;16:1061–8.PubMedCrossRefGoogle Scholar
  13. 13.
    Bonvalot S, Rimareix F, Causeret S, et al. Hyperthermic isolated limb perfusion in locally advanced soft tissue sarcoma and progressive desmoid-type fibromatosis with TNF 1 mg and melphalan (T1-M HILP) is safe and efficient. Ann Surg Oncol. 2009;16:3350.PubMedCrossRefGoogle Scholar
  14. 14.
    McGee L, Indelicato DJ, Dagan R, et al. Long-term results following postoperative radiotherapy for soft tissue sarcomas of the extremity. Int J Radiat Oncol Biol Phys. 2012;84:1003–9.PubMedCrossRefGoogle Scholar
  15. 15.
    Shimm DS, Suit HD. Radiation therapy of epithelioid sarcoma. Cancer. 1983;52:1022–5.PubMedCrossRefGoogle Scholar
  16. 16.
    Wolf PS, Flum DR, Tanas MR, et al. Epithelioid sarcoma: The University of Washington experience. Am J Surg. 2008;196:407–12.PubMedCrossRefGoogle Scholar
  17. 17.
    Callister MC, Ballo MT, Pisters PWT, et al. Epithelioid sarcoma: results of conservative surgery and radiotherapy. Int J Rad Oncol Biol Phys. 2001;51:384–91.CrossRefGoogle Scholar
  18. 18.
    Baratti D, Pennacchioli E, Casali PG, et al. Epithelioid sarcoma: prognostic factors and survival in a series of patients treated at a single institution. Ann Surg Oncol. 2007;14:3542–51.PubMedCrossRefGoogle Scholar
  19. 19.
    Edge SB, Byrd DR, Compton CC, et al. Soft tissue sarcoma. AJCC cancer staging manual, 7th edn. New York: Springer, 2010, pp 291–6.Google Scholar
  20. 20.
    Enzinger FM, Weiss SW. Soft tissue tumors, 3rd edn. St. Louis: Mosby, 1994.Google Scholar
  21. 21.
    Trojani M, Contesso G, Coindre JM, et al. Soft-tissue sarcomas of adults; study of pathological prognostic variables and definition of a histopathological grading system. Int J Cancer. 1984;33:37–42.PubMedCrossRefGoogle Scholar
  22. 22.
    Tumor of bone and soft tissues. R classification. In: Sobin LH, Wittekind Ch. TNM classification of malignant tumours UICC, 6th edn, 2002. New York: Wiley Liss, p 110.Google Scholar
  23. 23.
    Therasse P, Arbuck SG, Eisenhauer EA, et al. New guidelines to evaluate the response to treatment in solid tumors. European Organization for Research and Treatment of Cancer, National Cancer Institute of the United States, National Cancer Institute of Canada. J Natl Cancer Inst. 2000;92:205–16.PubMedCrossRefGoogle Scholar
  24. 24.
    Ross HM, Lewis JJ, Woodruff JM, et al. Epithelioid sarcoma: clinical behavior and prognostic factors of survival. Ann Surg Oncol. 1997;4:491–5.PubMedCrossRefGoogle Scholar
  25. 25.
    Guzzetta AA, Montgomery EA, Lyu H, et al. Epithelioid sarcoma: one institution’s experience with a rare sarcoma. J Surg Res. 2012;177:116–22.PubMedCentralPubMedCrossRefGoogle Scholar
  26. 26.
    Halling AC, Wollan PC, Pritchard DJ, et al. Epithelioid sarcoma: a clinicopathologic review of 55 cases. Mayo Clin Proc. 1996;71:636–42.PubMedCrossRefGoogle Scholar
  27. 27.
    Grunhagen DJ, de Wilt JH, Graveland WJ, et al. Outcome and prognostic factor analysis of 217 consecutive isolated limb perfusions with tumor necrosis factor-alpha and melphalan for limb-threatening soft tissue sarcoma. Cancer. 2006;106:1776.PubMedCrossRefGoogle Scholar
  28. 28.
    Deroose JP, van Geel AN, Burger JW, et al. Isolated limb perfusion with TNF-alpha and melphalan for distal parts of the limb in soft tissue sarcoma patients. J Surg Oncol. 2012;105:563–9.PubMedCrossRefGoogle Scholar
  29. 29.
    Olofsson R, Bergh P, Berlin O, et al. Long-term outcome of isolated limb perfusion in advanced soft tissue sarcoma of the extremity. Ann Surg Oncol. 2012;19:1800–7.PubMedCrossRefGoogle Scholar
  30. 30.
    Bonvalot S, Dunant A, Le Pechoux C, et al. Quality of surgical margins and local recurrence in primary extremity soft tissue sarcoma (STS). J Clin Oncol. 2010;28:15s (suppl; abstr 10068).Google Scholar
  31. 31.
    Pennacchioli E, Deraco M, Mariani L, Fiore M, Mussi C, Collini P, Olmi P, Casali PG, Santinami M, Gronchi A. Advanced extremity soft tissue sarcoma: prognostic effect of isolated limb perfusion in a series of 88 patients treated at a single institution. Ann Surg Oncol. 2007;14:553–9.PubMedCrossRefGoogle Scholar
  32. 32.
    Stoeckle E, Gardet H, Coindre JM, Kantor G, Bonichon F, Milbéo Y, Thomas L, Avril A, Bui BN. Prospective evaluation of quality of surgery in soft tissue sarcoma. Eur J Surg Oncol. 2006;32:1242–8.PubMedCrossRefGoogle Scholar
  33. 33.
    Bhangu A, Broom L, Nepogodiev D, Gourevitch D, Desai A. Outcomes of isolated limb perfusion in the treatment of extremity soft tissue sarcoma: a systematic review. Eur J Surg Oncol. 2013;39:311–9.PubMedCrossRefGoogle Scholar
  34. 34.
    Zagars GK, Ballo MT, Pisters PW, Pollock RE, Patel SR, Benjamin RS. Surgical margins and reresection in the management of patients with soft tissue sarcoma using conservative surgery and radiation therapy. Cancer. 2003;97:2544–53.PubMedCrossRefGoogle Scholar
  35. 35.
    Steinberg BD, Gelberman RH, Mankin HJ, et al. Epithelioid sarcoma in the upper extremity. J Bone Joint Surg. 1992;74:28–35.PubMedGoogle Scholar
  36. 36.
    Maduekwe UN, Hornicek FJ, Springfield DS, et al. Role of sentinel lymph node biopsy in the staging of synovial, epithelioid, and clear cell sarcomas. Ann Surg Oncol. 2009;16:1356–63.PubMedCrossRefGoogle Scholar
  37. 37.
    Xie X, Ghadimi MP, Young ED, et al. Combining EGFR and mTOR blockade for the treatment of epithelioid sarcoma. Clin Cancer Res. 2011;17:5901–12.PubMedCentralPubMedCrossRefGoogle Scholar

Copyright information

© Society of Surgical Oncology 2013

Authors and Affiliations

  • Antonin Levy
    • 1
    • 2
  • Cécile Le Péchoux
    • 1
    • 2
  • Philippe Terrier
    • 2
    • 3
  • Ryan Bouaita
    • 1
    • 2
  • Julien Domont
    • 2
    • 4
  • Olivier Mir
    • 2
    • 4
  • Sarah Coppola
    • 2
    • 5
  • Charles Honoré
    • 2
    • 5
  • Axel Le Cesne
    • 2
    • 4
  • Sylvie Bonvalot
    • 2
    • 5
    Email author
  1. 1.Department of Radiation Oncology, Institut Gustave-RoussyUniversité Paris SudVillejuifFrance
  2. 2.Department of Soft Tissue and Bone Sarcoma Multidisciplinary UnitInstitut Gustave-RoussyVillejuifFrance
  3. 3.Department of PathologyInstitut Gustave-RoussyVillejuifFrance
  4. 4.Department of Medical OncologyInstitut Gustave-RoussyVillejuifFrance
  5. 5.Department of SurgeryInstitut Gustave-RoussyVillejuifFrance

Personalised recommendations