Seminars in Oncology
Volume 36, Issue 4 , Pages 324-337 , August 2009

Sarcomas With Spindle Cell Morphology

  • Paola Collini

      Affiliations

    • Department of Anatomic Pathology, IRCCS Foundation, Istituto Nazionale Tumori, Milan, Italy
    • These authors contributed equally to this work.
  • ,
  • Poul H.B. Sorensen

      Affiliations

    • Department of Molecular Oncology, BC Cancer Research Centre, Vancouver, BC, Canada
    • These authors contributed equally to this work.
  • ,
  • Shreyaskumar Patel

      Affiliations

    • Sarcoma Center, Department of Sarcoma Medical Oncology, The University of Texas M.D. Anderson Cancer Center, Houston, TX
    • These authors contributed equally to this work.
  • ,
  • Jean-Yves Blay

      Affiliations

    • Centre Leon Berard, Hôpital Edouard Herriot, pav. E and INSERM U590, Lyon, France
    • These authors contributed equally to this work.
  • ,
  • Rolf D. Issels

      Affiliations

    • University Hospital Medical Center Grosshadern, Medical Clinic III, Munich, Germany; and HelmholtzZentrum München, German Research Center for Environmental Health, CCG Hyperthermia, Neuherberg, Germany
    • These authors contributed equally to this work.
  • ,
  • Robert G. Maki

      Affiliations

    • Sarcoma Program, Memorial Sloan-Kettering Cancer Center, New York, NY
    • These authors contributed equally to this work.
  • ,
  • Mikael Eriksson

      Affiliations

    • Department of Oncology, University Hospital, Lund, Sweden
    • These authors contributed equally to this work.
  • ,
  • Xavier Garcia del Muro

      Affiliations

    • Instituto Catalán de Oncología, Barcelona, Spain
    • These authors contributed equally to this work.
    • Corresponding Author InformationAddress correspondence to Xavier Garcia del Muro, MD, Instituto Catalán de Oncología, c/Viladomat 319 Atico 1, Barcelona 08029, Spain

References 

  1. Fletcher CDM, Unni KK, Mertens F World Health Organization classification of tumours. Pathology and genetics of tumours of soft tissue and bone. Lyon, France: IARC Press; 2002;p. 427
  2. Kempson RL, Fletcher CDM, Evans HL, Hendrickson MR, Sibley RK. Atlas of tumor pathology: tumors of soft tissue. Washington, DC: Armed Forces Institute of Pathology; 2001;
  3. Weiss SW, Goldblum JR. Soft tissue tumors. 5th ed.. Philadelphia: Mosby-Elsevier; 2008;p. 1258
  4. McArthur GA, Demetri GD, van Oosterom A, Heinrich MC, Debiec-Rychter M, Corless CL, et al. Molecular and clinical analysis of locally advanced dermatofibrosarcoma protuberans treated with imatinib: Imatinib Target Exploration Consortium Study B2225. J Clin Oncol. 2005;23:866–873
  5. Maki RG, Awan RA, Dixon RH, Jhanwar S, Antonescu CR. Differential sensitivity to imatinib of 2 patients with metastatic sarcoma arising from dermatofibrosarcoma protuberans. Int J Cancer. 2002;100:623–626
  6. Rubin BP, Schuetze SM, Eary JF, Norwood TH, Mirza S, Conrad EU, et al. Molecular targeting of platelet-derived growth factor B by imatinib mesylate in a patient with metastatic dermatofibrosarcoma protuberans. J Clin Oncol. 2002;20:3586–3591
  7. Bennicelli JL, Barr FG. Chromosomal translocations and sarcomas. Curr Opin Oncol. 2002;14:412–419
  8. Mitelman F. Recurrent chromosome aberrations in cancer. Mutat Res. 2000;462:247–253
  9. Ladanyi M, Bridge JA. Contribution of molecular genetic data to the classification of sarcomas. Hum Pathol. 2000;31:532–538
  10. de Alava E, Kawai A, Healey JH, Fligman I, Meyers PA, Huvos AG, et al. EWS-FLI1 fusion transcript structure is an independent determinant of prognosis in Ewing's sarcoma. J Clin Oncol. 1998;16:1248–1255
  11. Ladanyi M, Antonescu CR, Leung DH, Woodruff JM, Kawai A, Healey JH, et al. Impact of SYT-SSX fusion type on the clinical behavior of synovial sarcoma: a multi-institutional retrospective study of 243 patients. Cancer Res. 2002;62:135–140
  12. Sorensen PH, Lynch JC, Qualman SJ, Tirabosco R, Lim JF, Maurer HM, et al. PAX3-FKHR and PAX7-FKHR gene fusions are prognostic indicators in alveolar rhabdomyosarcoma: a report from the Children's Oncology Group. J Clin Oncol. 2002;20:2672–2679
  13. Zoubek A, Dockhorn-Dworniczak B, Delattre O, Christiansen H, Niggli F, Gatterer-Menz I, et al. Does expression of different EWS chimeric transcripts define clinically distinct risk groups of Ewing tumor patients?. J Clin Oncol. 1996;14:1245–1251
  14. Barr FG. Translocations, cancer and the puzzle of specificity. Nat Genet. 1998;19:121–124
  15. van de Rijn M, Barr FG, Xiong QB, Salhany KE, Fraker DL, Fisher C. Radiation-associated synovial sarcoma. Hum Pathol. 1997;28:1325–1328
  16. Olsen RJ, Tarantolo SR, Hinrichs SH. Molecular approaches to sarcoma therapy. Sarcoma. 2002;6:27–42
  17. Rabbitts TH. Chromosomal translocation master genes, mouse models and experimental therapeutics. Oncogene. 2001;20:5763–5777
  18. Tomescu O, Barr FG. Chromosomal translocations in sarcomas: prospects for therapy. Trends Mol Med. 2001;7:554–559
  19. Borden EC, Baker LH, Bell RS, Bramwell V, Demetri GD, Eisenberg BL, et al. Soft tissue sarcomas of adults: state of the translational science. Clin Cancer Res. 2003;9:1941–1956
  20. Kawai A, Woodruff J, Healey JH, Brennan MF, Antonescu CR, Ladanyi M. SYT-SSX gene fusion as a determinant of morphology and prognosis in synovial sarcoma. N Engl J Med. 1998;338:153–160
  21. Rabbitts TH, Forster A, Larson R, Nathan P. Fusion of the dominant negative transcription regulator CHOP with a novel gene FUS by translocation t(12;16) in malignant liposarcoma. Nat Genet. 1993;4:175–180
  22. Knezevich SR, McFadden DE, Tao W, Lim JF, Sorensen PH. A novel ETV6-NTRK3 gene fusion in congenital fibrosarcoma. Nat Genet. 1998;18:184–187
  23. Knezevich SR, Garnett MJ, Pysher TJ, Beckwith JB, Grundy PE, Sorensen PH. ETV6-NTRK3 gene fusions and trisomy 11 establish a histogenetic link between mesoblastic nephroma and congenital fibrosarcoma. Cancer Res. 1998;58:5046–5048
  24. Morrison KB, Tognon CE, Garnett MJ, Deal C, Sorensen PH. ETV6-NTRK3 transformation requires insulin-like growth factor 1 receptor signaling and is associated with constitutive IRS-1 tyrosine phosphorylation. Oncogene. 2002;21:5684–5695
  25. Martin MJ, Melnyk N, Pollard M, Bowden M, Leong H, Podor TJ, et al. The insulin-like growth factor I receptor is required for Akt activation and suppression of anoikis in cells transformed by the ETV6-NTRK3 chimeric tyrosine kinase. Mol Cell Biol. 2006;26:1754–1769
  26. Park MS, Lazar AJ, Trent JC, Conrad CA, Ludwig JA, Wang W, et al. Combination therapy with temozolomide and bevacizumab in the treatment of hemangiopericytoma/solitary fibrous tumor: an updated analysis [abstract]. Proc Connective Tissue Oncol Soc. 2008;14:35064
  27. Borden EC, Amato DA, Rosenbaum C, Enterline HT, Shiraki MJ, Creech RH, et al. Randomized comparison of three adriamycin regimens for metastatic soft tissue sarcomas. J Clin Oncol. 1987;5:840–850
  28. Maki RG, Wathen JK, Patel SR, Priebat DA, Okuno SH, Samuels B, 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. J Clin Oncol. 2007;25:2755–2763
  29. Penel N, Bui BN, Bay JO, Cupissol D, Ray-Coquard I, Piperno-Neumann S, et al. Phase II trial of weekly paclitaxel for unresectable angiosarcoma: the ANGIOTAX study. J Clin Oncol. 2008;26:5269–5274
  30. Rosen G, Forscher C, Lowenbraun S, Eilber F, Eckardt J, Holmes C, et al. Synovial sarcoma (Uniform response of metastases to high dose ifosfamide). Cancer. 1994;73:2506–2511
  31. Garcia-Carbonero R, Supko JG, Maki RG, Manola J, Ryan DP, Harmon D, et al. Ecteinascidin-743 (ET-743) for chemotherapy-naive patients with advanced soft tissue sarcomas: multicenter phase II and pharmacokinetic study. J Clin Oncol. 2005;23:5484–5492
  32. Garcia-Carbonero R, Supko JG, Manola J, Seiden MV, Harmon D, Ryan DP, et al. Phase II and pharmacokinetic study of ecteinascidin 743 in patients with progressive sarcomas of soft tissues refractory to chemotherapy. J Clin Oncol. 2004;22:1480–1490
  33. Grosso F, Jones RL, Demetri GD, Judson IR, Blay JY, Le Cesne A, et al. Efficacy of trabectedin (ecteinascidin-743) in advanced pretreated myxoid liposarcomas: a retrospective study. Lancet Oncol. 2007;8:595–602
  34. Meazza C, Casanova M, Zaffignani E, Luksch R, Podda M, Favini F, et al. Efficacy of topotecan plus vincristine and doxorubicin in children with recurrent/refractory rhabdomyosarcoma. Med Oncol. 2009;26:67–72
  35. Hansmann A, Adolph C, Vogel T, Unger A, Moeslein G. High-dose tamoxifen and sulindac as first-line treatment for desmoid tumors. Cancer. 2004;100:612–620
  36. Gadducci A, Cosio S, Romanini A, Genazzani AR. The management of patients with uterine sarcoma: a debated clinical challenge. Crit Rev Oncol Hematol. 2008;65:129–142
  37. Heinrich MC, McArthur GA, Demetri GD, Joensuu H, Bono P, Herrmann R, et al. Clinical and molecular studies of the effect of imatinib on advanced aggressive fibromatosis (desmoid tumor). J Clin Oncol. 2006;24:1195–1203
  38. D'Adamo DR, Anderson SE, Albritton K, Yamada J, Riedel E, Scheu K, et al. Phase II study of doxorubicin and bevacizumab for patients with metastatic soft-tissue sarcomas. J Clin Oncol. 2005;23:7135–7142
  39. Wunder JS, Nielsen TO, Maki RG, O'Sullivan B, Alman BA. Opportunities for improving the therapeutic ratio for patients with sarcoma. Lancet Oncol. 2007;8:513–524
  40. Mita MM, Mita AC, Chu QS, Rowinsky EK, Fetterly GJ, Goldston M, et al. Phase I trial of the novel mammalian target of rapamycin inhibitor deforolimus (AP23573; MK-8669) administered intravenously daily for 5 days every 2 weeks to patients with advanced malignancies. J Clin Oncol. 2008;26:361–367
  41. Sarcoma Meta-Analysis Collaboration. Adjuvant chemotherapy for localised resectable soft-tissue sarcoma of adults: meta-analysis of individual data. Lancet. 1997;350:1647–1654
  42. Frustaci S, Gherlinzoni F, De Paoli A, Bonetti M, Azzarelli A, Comandone A, et al. Adjuvant chemotherapy for adult soft tissue sarcomas of the extremities and girdles: results of the Italian randomized cooperative trial. J Clin Oncol. 2001;19:1238–1247
  43. Frustaci S, De Paoli A, Bidoli E, La Mura N, Berretta M, Buonadonna A, et al. Ifosfamide in the adjuvant therapy of soft tissue sarcomas. Oncology. 2003;65(Suppl 2):80–84
  44. Woll PJ, Van Glabbeke M, Hohenberger P, Le Cesne A, Gronchi A, Hoekstra HJ, et al. Adjuvant chemotherapy with doxorubicin and ifosfamide in resected soft tissue sarcoma: interim analysis of a randomised phase III trial [abstract]. J Clin Oncol. 2007;25:10008
  45. Pervaiz N, Colterjohn N, Farrokhyar F, Tozer R, Figueredo A, Ghert M. A systematic meta-analysis of randomized controlled trials of adjuvant chemotherapy for localized resectable soft-tissue sarcoma. Cancer. 2008;113:573–581
  46. Pinedo HM, Vendrik CP, Bramwell VH, van Slooten EA, Deakin DP, van Unnik JA, et al. Evaluation of adjuvant therapy in soft tissue sarcoma (A collaborative multidisciplinary approach). EORTC protocol 62771 Eur J Cancer. 1979;15:811–820
  47. Le Cesne A, Van Glabbeke M, Woll PJ, Bramwell VH, Casali PG, Hoekstra HJ, et al. The end of adjuvant chemotherapy era with doxorubicin-based regimen in resected high-grade soft tissue sarcoma: pooled analysis of the two STBSG-EORTC phase III clinical trials [abstract]. J Clin Oncol. 2008;26:10525
  48. Schöffski P, Casali PG, Taron M, Van Oosterom AT, Judson IR, Grosso F, et al. DNA repair functionality modulates the clinical outcome of patients with advanced sarcoma treated with trabectedin (ET-743) [abstract]. J Clin Oncol. 2006;24:9522
  49. Gortzak E, Azzarelli A, Buesa J, Bramwell VH, van Coevorden F, van Geel AN, et al. A randomised phase II study on neo-adjuvant chemotherapy for ‘high-risk’ adult soft-tissue sarcoma. Eur J Cancer. 2001;37:1096–1103
  50. Lorigan P, Verweij J, Papai Z, Rodenhuis S, Le Cesne A, Leahy MG, et al. Phase III trial of two investigational schedules of ifosfamide compared with standard-dose doxorubicin in advanced or metastatic soft tissue sarcoma: a European Organisation for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group study. J Clin Oncol. 2007;25:3144–3150
  51. Wust P, Hildebrandt B, Sreenivasa G, et al. Hyperthermia in combined treatment of cancer. Lancet Oncol. 2002;3:487–497
  52. Milani V, Noessner E. Effects of thermal stress on tumor antigenicity and recognition by immune effector cells. Cancer Immunol Immunother. 2006;55:312–319
  53. Wust P, Cho CH, Hildebrandt B, et al. Thermal monitoring: Invasive, minimal-invasive and non-invasive approaches. Int J Hyperthermia. 2006;22:255–262
  54. Gellermann J, Hildebrandt B, Issels R, Ganter H, Wlodarczyk W, Budach V, et al. Noninvasive magnetic resonance thermography of soft tissue sarcomas during regional hyperthermia: correlation with response and direct thermometry. Cancer. 2006;107:1373–1382
  55. Issels RD, Bosse D, Abdel-Rahman S, et al. Preoperative systemic etoposide/ifosfamide/doxorubicin chemotherapy combined with regional hyperthermia in high-risk sarcoma: a pilot study. Cancer Chemother Pharmacol. 1993;31(Suppl 2):S233–S237
  56. Issels RD, Abdel-Rahman S, Wendtner CM, et al. Neoadjuvant chemotherapy combined with regional hyperthermia (RHT) for locally advanced primary or recurrent high-risk adult soft-tissue sarcomas (STS) of adults: long-term results of a phase II study. Eur J Cancer. 2001;37:1599-08
  57. Wendtner CM, Abdel-Rahman S, Krych M, et al. Response to neoadjuvant chemotherapy combined with regional hyperthermia predicts long-term survival for adult patients with retroperitoneal and visceral high-risk soft tissue sarcomas. J Clin Oncol. 2002;20:3156–3164
  58. Multhoff G, Botzler C, Jennen L, et al. Heat shock protein 72 on tumor cells (A recognition structure for natural killer cells). J Immunol. 1997;158:4341–4350
  59. Dewey WC. Arrhenius relationships from the molecule and cell to the clinic. Int J Hyperthermia. 1994;10:457–483
  60. Issels RD, Lindner LH, Wust P, et al. Regional hyperthermia (RHT) improves response and survival when combined with systemic chemotherapy in the management of locally advanced, high grade soft tissue sarcomas (STS) of the extremities, the body wall and the abdomen: A phase III randomised prospective trial (EORTC-ESHO intergroup trial). J Clin Oncol. 2007;25(18S):10009
  61. Lindner L, Mella O, Kuhlencordt M, et al. Early progression in patients with high-risk soft tissue sarcomas (STS): a phase III randomized prospective trial (EORTC/ESHO intergroup trial) of neoadjuvant chemotherapy with or without regional hyperthermia (RHT) [abstract 7504]. Eur J Cancer. 2007;5(Suppl 4):403
  62. Issels RD. Regional hyperthermia in high-risk soft tissue sarcomas. Curr Opin Oncol. 2008;20:438–443

 Jean-Yves Blay is supported by CONTICANET Network of Excellence of the 6th FP of the European Commission; Poul H.B. Sorensen has received grants from the Canadian Institutes for Health Research; and Robert G. Maki is supported by Program Project Grant No. P01-CA47179; Cycle for Survival.

Disclosures Jean-Yves Blay: Research support, consulting fees (Pfizer, Novartis, Roche, Glaxo Smith Kline)Robert G. Maki: Research support (Pfizer); honoraria (Ziopharm); testimony (Roche)

PII: S0093-7754(09)00107-9

doi: 10.1053/j.seminoncol.2009.06.007

Seminars in Oncology
Volume 36, Issue 4 , Pages 324-337 , August 2009