Advertisement
Review Article| Volume 34, ISSUE 1, P161-175, February 2020

Malignant Soft-Tissue Sarcomas

Published:October 28, 2019DOI:https://doi.org/10.1016/j.hoc.2019.08.022

      Keywords

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribers receive full online access to your subscription and archive of back issues up to and including 2002.

      Content published before 2002 is available via pay-per-view purchase only.

      Subscribe:

      Subscribe to Hematology/Oncology Clinics
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Noone A.
        • Howlader N.
        • Krapcho M.
        • et al.
        SEER cancer statistics review, 1975-2015.
        National Cancer Institute, Bethesda (MD)2018
        • Siegel R.L.
        • Miller K.D.
        • Jemal A.
        Cancer statistics, 2019.
        CA Cancer J Clin. 2019; 69: 7-34
        • Gustafson P.
        • Dreinhofer K.E.
        • Rydholm A.
        Soft tissue sarcoma should be treated at a tumor center. A comparison of quality of surgery in 375 patients.
        Acta Orthop Scand. 1994; 65: 47-50
        • Hoda S.A.
        Enzinger and Weiss’s soft tissue tumors, 6th edition.
        Adv Anat Pathol. 2014; 21: 216
        • Farid M.
        • Ngeow J.
        Sarcomas associated with genetic cancer predisposition syndromes: a review.
        Oncologist. 2016; 21: 1002-1013
        • Ballinger M.L.
        • Goode D.L.
        • Ray-Coquard I.
        • et al.
        Monogenic and polygenic determinants of sarcoma risk: an international genetic study.
        Lancet Oncol. 2016; 17: 1261-1271
        • Rydholm A.
        • Berg N.O.
        • Gullberg B.
        • et al.
        Epidemiology of soft-tissue sarcoma in the locomotor system. A retrospective population-based study of the inter-relationships between clinical and morphologic variables.
        Acta Pathol Microbiol Immunol Scand A. 1984; 92: 363-374
        • Aisen A.M.
        • Martel W.
        • Braunstein E.M.
        • et al.
        MRI and CT evaluation of primary bone and soft-tissue tumors.
        AJR Am J Roentgenol. 1986; 146: 749-756
        • Demas B.E.
        • Heelan R.T.
        • Lane J.
        • et al.
        Soft-tissue sarcomas of the extremities: comparison of MR and CT in determining the extent of disease.
        AJR Am J Roentgenol. 1988; 150: 615-620
        • McKenzie A.F.
        The role of magnetic resonance imaging.
        Acta orthopaedica Scand. 1997; 68: 21-24
        • Panicek D.M.
        • Gatsonis C.
        • Rosenthal D.I.
        • et al.
        CT and MR imaging in the local staging of primary malignant musculoskeletal neoplasms: report of the Radiology Diagnostic Oncology Group.
        Radiology. 1997; 202: 237-246
        • Roberge D.
        • Vakilian S.
        • Alabed Y.Z.
        • et al.
        FDG PET/CT in initial staging of adult soft-tissue sarcoma.
        Sarcoma. 2012; 2012: 960194
        • Italiano A.
        • Di Mauro I.
        • Rapp J.
        • et al.
        Clinical effect of molecular methods in sarcoma diagnosis (GENSARC): a prospective, multicentre, observational study.
        Lancet Oncol. 2016; 17: 532-538
        • American Joint Committee on Cancer
        AJCC cancer staging manual.
        8th edition. Springer, Cham (Switzerland)2017
        • Fisher S.B.
        • Chiang Y.-J.
        • Feig B.W.
        • et al.
        Comparative performance of the 7th and 8th editions of the American Joint Committee on cancer staging systems for soft tissue sarcoma of the trunk and extremities.
        Ann Surg Oncol. 2018; 25: 1126-1132
        • Enneking W.F.
        • Spanier S.S.
        • Goodman M.A.
        A system for the surgical staging of musculoskeletal sarcoma.
        Clin Orthop Relat Res. 1980; : 106-120
        • Hajdu S.I.
        • Shiu M.H.
        • Brennan M.F.
        The role of the pathologist in the management of soft tissue sarcomas.
        World J Surg. 1988; 12: 326-331
        • Gustafson P.
        • Akerman M.
        • Alvegard T.A.
        • et al.
        Prognostic information in soft tissue sarcoma using tumour size, vascular invasion and microscopic tumour necrosis––the SIN-system.
        Eur J Cancer. 2003; 39: 1568-1576
        • Spiro I.J.
        • Gebhardt M.C.
        • Jennings L.C.
        • et al.
        Prognostic factors for local control of sarcomas of the soft tissues managed by radiation and surgery.
        Semin Oncol. 1997; 24: 540-546
        • Wunder J.S.
        • Healey J.H.
        • Davis A.M.
        • et al.
        A comparison of staging systems for localized extremity soft tissue sarcoma.
        Cancer. 2000; 88: 2721-2730
        • Guillou L.
        • Coindre J.M.
        • Bonichon F.
        • et al.
        Comparative study of the National Cancer Institute and French Federation of Cancer Centers Sarcoma Group grading systems in a population of 410 adult patients with soft tissue sarcoma.
        J Clin Oncol. 1997; 15: 350-362
        • Angervall L.
        • Kindblom L.G.
        • Rydholm A.
        • et al.
        The diagnosis and prognosis of soft tissue tumors.
        Semin Diagn Pathol. 1986; 3: 240-258
        • Kandel R.A.
        • Bell R.S.
        • Wunder J.S.
        • et al.
        Comparison between a 2- and 3-grade system in predicting metastatic-free survival in extremity soft-tissue sarcoma.
        J Surg Oncol. 1999; 72: 77-82
        • Gutierrez J.C.
        • Perez E.A.
        • Franceschi D.
        • et al.
        Outcomes for soft-tissue sarcoma in 8249 cases from a large state cancer registry.
        J Surg Res. 2007; 141: 105-114
        • Ferrari A.
        • Miceli R.
        • Meazza C.
        • et al.
        Soft tissue sarcomas of childhood and adolescence: the prognostic role of tumor size in relation to patient body size.
        J Clin Oncol. 2009; 27: 371-376
        • American Joint Committee on Cancer
        AJCC cancer staging handbook.
        7th edition. Springer-Verlag, New York2010
        • Tsujimoto M.
        • Aozasa K.
        • Ueda T.
        • et al.
        Multivariate analysis for histologic prognostic factors in soft tissue sarcomas.
        Cancer. 1988; 62: 994-998
        • Kattan M.W.
        • Leung D.H.
        • Brennan M.F.
        Postoperative nomogram for 12-year sarcoma-specific death.
        J Clin Oncol. 2002; 20: 791-796
        • Stojadinovic A.
        • Leung D.H.Y.
        • Hoos A.
        • et al.
        Analysis of the prognostic significance of microscopic margins in 2,084 localized primary adult soft tissue sarcomas.
        Ann Surg. 2002; 235: 424-434
        • Maki R.G.
        • Moraco N.
        • Antonescu C.R.
        • et al.
        Toward better soft tissue sarcoma staging: building on American Joint Committee on cancer staging systems versions 6 and 7.
        Ann Surg Oncol. 2013; 20: 3377-3383
        • Justin M.M.C.
        The AJCC 8th edition staging system for soft tissue sarcoma of the extremities or trunk: a cohort study of the SEER database.
        J Natl Compr Canc Netw. 2018; 16: 144-152
        • Chibon F.
        • Lagarde P.
        • Salas S.
        • et al.
        Validated prediction of clinical outcome in sarcomas and multiple types of cancer on the basis of a gene expression signature related to genome complexity.
        Nat Med. 2010; 16: 781-787
        • Kim J.R.
        • Moon Y.J.
        • Kwon K.S.
        • et al.
        Expression of SIRT1 and DBC1 is associated with poor prognosis of soft tissue sarcomas.
        PLoS One. 2013; 8: e74738
        • Kandel R.A.
        • Yao X.
        • Dickson B.C.
        • et al.
        Molecular analyses in the diagnosis and prediction of prognosis in non-GIST soft tissue sarcomas: a systematic review and meta-analysis.
        Cancer Treat Rev. 2018; 66: 74-81
        • Machado I.
        • Noguera R.
        • Pellin A.
        • et al.
        Molecular diagnosis of Ewing sarcoma family of tumors: a comparative analysis of 560 cases with FISH and RT-PCR.
        Diagn Mol Pathol. 2009; 18: 189-199
        • Williamson D.
        • Missiaglia E.
        • de Reynies A.
        • et al.
        Fusion gene-negative alveolar rhabdomyosarcoma is clinically and molecularly indistinguishable from embryonal rhabdomyosarcoma.
        J Clin Oncol. 2010; 28: 2151-2158
        • Skapek S.X.
        • Anderson J.
        • Barr F.G.
        • et al.
        PAX-FOXO1 fusion status drives unfavorable outcome for children with rhabdomyosarcoma: a Children's Oncology Group report.
        Pediatr Blood Cancer. 2013; 60: 1411-1417
        • Crago A.M.
        • Dickson M.A.
        Liposarcoma: multimodality management and future targeted therapies.
        Surg Oncol Clin N Am. 2016; 25: 761-773
        • DeLaney T.F.
        • Gebhardt M.C.
        • Ryan C.W.
        Overview of multimodality treatment for primary soft tissue sarcoma of the extremities and chest wall.
        in: Maki R. Pollock R.E. UpToDate. 2018 (Available at:) (Accessed September 1, 2019)
        • Alvegard T.A.
        • Sigurdsson H.
        • Mouridsen H.
        • et al.
        Adjuvant chemotherapy with doxorubicin in high-grade soft tissue sarcoma: a randomized trial of the Scandinavian Sarcoma Group.
        J Clin Oncol. 1989; 7: 1504-1513
        • Trovik C.S.
        • Bauer H.C.F.
        • Alvegård T.A.
        • et al.
        Surgical margins, local recurrence and metastasis in soft tissue sarcomas: 559 surgically-treated patients from the Scandinavian Sarcoma Group Register.
        Eur J Cancer. 2000; 36: 710-716
        • White L.M.
        • Wunder J.S.
        • Bell R.S.
        • et al.
        Histologic assessment of peritumoral edema in soft tissue sarcoma.
        Int J Radiat Oncol Biol Phys. 2005; 61: 1439-1445
        • McGee L.
        • Indelicato D.J.
        • 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: 1003-1009
        • Gannon N.P.
        • King D.M.
        • Ethun C.G.
        • et al.
        The role of radiation therapy and margin width in localized soft-tissue sarcoma: analysis from the US Sarcoma Collaborative.
        J Surg Oncol. 2019; 120: 325-331
        • O'Sullivan B.
        • Davis A.M.
        • Turcotte R.
        • et al.
        Preoperative versus postoperative radiotherapy in soft-tissue sarcoma of the limbs: a randomised trial.
        Lancet. 2002; 359: 2235-2241
        • Lin A.
        • Kim H.M.
        • Terrell J.E.
        • et al.
        Quality of life after parotid-sparing IMRT for head-and-neck cancer: a prospective longitudinal study.
        Int J Radiat Oncol Biol Phys. 2003; 57: 61-70
        • Nutting C.M.
        • Morden J.P.
        • Harrington K.J.
        • et al.
        Parotid-sparing intensity modulated versus conventional radiotherapy in head and neck cancer (PARSPORT): a phase 3 multicentre randomised controlled trial.
        Lancet Oncol. 2011; 12: 127-136
        • Peng G.
        • Wang T.
        • Yang K.Y.
        • et al.
        A prospective, randomized study comparing outcomes and toxicities of intensity-modulated radiotherapy vs. conventional two-dimensional radiotherapy for the treatment of nasopharyngeal carcinoma.
        Radiother Oncol. 2012; 104: 286-293
        • Zelefsky M.J.
        • Levin E.J.
        • Hunt M.
        • et al.
        Incidence of late rectal and urinary toxicities after three-dimensional conformal radiotherapy and intensity-modulated radiotherapy for localized prostate cancer.
        Int J Radiat Oncol Biol Phys. 2008; 70: 1124-1129
        • Lin Y.
        • Chen K.
        • Lu Z.
        • et al.
        Intensity-modulated radiation therapy for definitive treatment of cervical cancer: a meta-analysis.
        Radiat Oncol. 2018; 13: 177
        • Fredman E.T.
        • Abdel-Wahab M.
        • Kumar A.M.S.
        Influence of radiation treatment technique on outcome and toxicity in anal cancer.
        J Radiat Oncol. 2017; 6: 413-421
        • Chun S.G.
        • Hu C.
        • Choy H.
        • et al.
        Impact of intensity-modulated radiation therapy technique for locally advanced non-small-cell lung cancer: a secondary analysis of the NRG oncology RTOG 0617 randomized clinical trial.
        J Clin Oncol. 2017; 35: 56-62
        • Lin S.H.
        • Wang L.
        • Myles B.
        • et al.
        Propensity score-based comparison of long-term outcomes with 3-dimensional conformal radiotherapy vs intensity-modulated radiotherapy for esophageal cancer.
        Int J Radiat Oncol Biol Phys. 2012; 84: 1078-1085
        • O'Sullivan B.
        • Griffin A.M.
        • Dickie C.I.
        • et al.
        Phase 2 study of preoperative image-guided intensity-modulated radiation therapy to reduce wound and combined modality morbidities in lower extremity soft tissue sarcoma.
        Cancer. 2013; 119: 1878-1884
        • Folkert M.R.
        • Casey D.L.
        • Berry S.L.
        • et al.
        Femoral fracture in primary soft-tissue sarcoma of the thigh and groin treated with intensity-modulated radiation therapy: observed versus expected risk.
        Ann Surg Oncol. 2019; 26: 1326-1331
        • Gortzak Y.
        • Lockwood G.A.
        • Mahendra A.
        • et al.
        Prediction of pathologic fracture risk of the femur after combined modality treatment of soft tissue sarcoma of the thigh.
        Cancer. 2010; 116: 1553-1559
        • Folkert M.R.
        • Singer S.
        • Brennan M.F.
        • et al.
        Comparison of local recurrence with conventional and intensity-modulated radiation therapy for primary soft-tissue sarcomas of the extremity.
        J Clin Oncol. 2014; 32: 3236-3241
        • Robinson M.H.
        • Gaunt P.
        • Grimer R.
        • et al.
        Vortex trial: a randomized controlled multicenter phase 3 trial of volume of postoperative radiation therapy given to adult patients with extremity soft tissue sarcoma (STS).
        Int J Radiat Oncol Biol Phys. 2016; 96: S1
        • Bujold A.
        • Craig T.
        • Jaffray D.
        • et al.
        Image-guided radiotherapy: has it influenced patient outcomes?.
        Semin Radiat Oncol. 2012; 22: 50-61
        • Wang D.
        • Zhang Q.
        • Eisenberg B.L.
        • et al.
        Significant reduction of late toxicities in patients with extremity sarcoma treated with image-guided radiation therapy to a reduced target volume: results of Radiation Therapy Oncology Group RTOG-0630 Trial.
        J Clin Oncol. 2015; 33: 2231-2238
        • Le Cesne A.
        • Ouali M.
        • Leahy M.G.
        • et al.
        Doxorubicin-based adjuvant chemotherapy in soft tissue sarcoma: pooled analysis of two STBSG-EORTC phase III clinical trials.
        Ann Oncol. 2014; 25: 2425-2432
        • DeLaney T.F.
        • Spiro I.J.
        • Suit H.D.
        • et al.
        Neoadjuvant chemotherapy and radiotherapy for large extremity soft-tissue sarcomas.
        Int J Radiat Oncol Biol Phys. 2003; 56: 1117-1127
        • Mullen J.T.
        • Kobayashi W.
        • Wang J.J.
        • et al.
        Long-term follow-up of patients treated with neoadjuvant chemotherapy and radiotherapy for large, extremity soft tissue sarcomas.
        Cancer. 2012; 118: 3758-3765
        • Lawrence Jr., W.
        • Donegan W.L.
        • Natarajan N.
        • et al.
        Adult soft tissue sarcomas. A pattern of care survey of the American College of Surgeons.
        Ann Surg. 1987; 205: 349-359
        • Linehan D.C.
        • Lewis J.J.
        • Leung D.
        • et al.
        Influence of biologic factors and anatomic site in completely resected liposarcoma.
        J Clin Oncol. 2000; 18: 1637-1643
        • Nathan H.
        • Raut C.P.
        • Thornton K.
        • et al.
        Predictors of survival after resection of retroperitoneal sarcoma: a population-based analysis and critical appraisal of the AJCC staging system.
        Ann Surg. 2009; 250: 970-976
        • Tseng W.
        • Martinez S.R.
        • Tamurian R.M.
        • et al.
        Histologic type predicts survival in patients with retroperitoneal soft tissue sarcoma.
        J Surg Res. 2012; 172: 123-130
        • Abbott A.M.
        • Habermann E.B.
        • Parsons H.M.
        • et al.
        Prognosis for primary retroperitoneal sarcoma survivors.
        Cancer. 2012; 118: 3321-3329
        • Catton C.N.
        • O'Sullivan B.
        • Kotwall C.
        • et al.
        Outcome and prognosis in retroperitoneal soft tissue sarcoma.
        Int J Radiat Oncol Biol Phys. 1994; 29: 1005-1010
        • Lewis J.J.
        • Leung D.
        • Woodruff J.M.
        • et al.
        Retroperitoneal soft-tissue sarcoma: analysis of 500 patients treated and followed at a single institution.
        Ann Surg. 1998; 228: 355-365
        • Heslin M.J.
        • Lewis J.J.
        • Nadler E.
        • et al.
        Prognostic factors associated with long-term survival for retroperitoneal sarcoma: implications for management.
        J Clin Oncol. 1997; 15: 2832-2839
        • Kelly K.J.
        • Yoon S.S.
        • Kuk D.
        • et al.
        Comparison of perioperative radiation therapy and surgery versus surgery alone in 204 patients with primary retroperitoneal sarcoma: a retrospective 2-institution study.
        Ann Surg. 2015; 262: 156-162
        • Nussbaum D.P.
        • Rushing C.N.
        • Lane W.O.
        • et al.
        Preoperative or postoperative radiotherapy versus surgery alone for retroperitoneal sarcoma: a case-control, propensity score-matched analysis of a nationwide clinical oncology database.
        Lancet Oncol. 2016; 17: 966-975
        • Tzeng C.-W.D.
        • Fiveash J.B.
        • Popple R.A.
        • et al.
        Preoperative radiation therapy with selective dose escalation to the margin at risk for retroperitoneal sarcoma.
        Cancer. 2006; 107: 371-379
        • Delaney T.F.
        • Kepka L.
        • Goldberg S.I.
        • et al.
        Radiation therapy for control of soft-tissue sarcomas resected with positive margins.
        Int J Radiat Oncol Biol Phys. 2007; 67: 1460-1469
        • Gieschen H.L.
        • Spiro I.J.
        • Suit H.D.
        • et al.
        Long-term results of intraoperative electron beam radiotherapy for primary and recurrent retroperitoneal soft tissue sarcoma.
        Int J Radiat Oncol Biol Phys. 2001; 50: 127-131
        • DeLaney T.F.
        • Chen Y.-L.
        • Baldini E.H.
        • et al.
        Phase 1 trial of preoperative image guided intensity modulated proton radiation therapy with simultaneously integrated boost to the high risk margin for retroperitoneal sarcomas.
        Adv Radiat Oncol. 2017; 2: 85-93
      1. Proton or Photon RT for Retroperitoneal Sarcomas.
        (Available at:) (Accessed September 1, 2019)