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Therapy

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Soft Tissue Tumors
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Abstract

The treatment of soft tissue tumors can be challenging and in many cases requires surgery (including different subspecialties) as a primary approach. Radiotherapy and medical therapy (including chemotherapy, targeted therapy and immunotherapy) are indicated both in high-risk cases (in the adjuvant or neoadjuvant setting) and in locally advanced (non-operable) as well as metastatic disease. Given the rarity and at the same time the multiplicity of nosological entities (each one with its specific biological behavior and therapeutic sensitivity), it is universally accepted that soft tissue tumors should be referred to dedicated centers, where each case can be discussed within the frame of a multidisciplinary tumor board and all the specialists potentially involved in the management of these patients are available (e.g., pathologist, radiologist, surgical oncologist, radiotherapist, medical oncologist, orthopedic, vascular surgeon, urologist, neurosurgeon, molecular biologist).

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  • Albertsmeier (2018) External beam radiation therapy for resectable soft tissue sarcoma: a systematic review and meta-analysis. Ann Surg Oncol 25(3):754–767

    Article  PubMed  Google Scholar 

  • Andreou (2013) Sentinel node biopsy in soft tissue sarcoma subtypes with a high propensity for regional lymphatic spread—results of a large prospective trial. Ann Oncol 24(5):1400–1405

    Article  CAS  PubMed  Google Scholar 

  • Assi (2019) Neoadjuvant isolated limb perfusion in newly diagnosed untreated patients with locally advanced soft tissue sarcomas of the extremities: the Gustave Roussy experience. Clin Transl Oncol 21(9):1135–1141

    Article  CAS  PubMed  Google Scholar 

  • Ayodele (2020) Immunotherapy in soft-tissue sarcoma. Curr Oncol 27(Suppl 1):17–23

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Baumann (2016) Efficacy and safety of stereotactic body radiation therapy for the treatment of pulmonary metastases from sarcoma: a potential alternative to resection. J Surg Oncol 114(1):65–69

    Article  PubMed  Google Scholar 

  • Blay (2017) Improved survival using specialized multidisciplinary board in sarcoma patients. Ann Oncol 28(11):2852–2859

    Article  PubMed  PubMed Central  Google Scholar 

  • Blay (2019) Surgery in reference centers improves survival of sarcoma patients: a nationwide study. Ann Oncol 30(7):1143–1153

    Article  PubMed  PubMed Central  Google Scholar 

  • Bonvalot (2005) Randomized trial of cytoreduction followed by intraperitoneal chemotherapy versus cytoreduction alone in patients with peritoneal sarcomatosis. Eur J Surg Oncol 31(8):917–923

    Article  CAS  PubMed  Google Scholar 

  • Bonvalot (2019a) STRASS (EORTC 62092): a phase III randomized study of preoperative radiotherapy plus surgery versus surgery alone for patients with retroperitoneal sarcoma. J Clin Oncol 37(15_suppl):11001

    Article  Google Scholar 

  • Bonvalot (2019b) NBTXR3, a first-in-class radioenhancer hafnium oxide nanoparticle, plus radiotherapy versus radiotherapy alone in patients with locally advanced soft-tissue sarcoma (Act.In.Sarc): a multicentre, phase 2-3, randomised, controlled trial. Lancet Oncol 20(8):1148–1159

    Article  CAS  PubMed  Google Scholar 

  • Casali (2018) Soft tissue and visceral sarcomas: ESMO-EURACAN clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol 29(Suppl 4):iv51–iv67

    Article  CAS  PubMed  Google Scholar 

  • Chen (2020) Genomic-guided precision therapy for soft tissue sarcoma. ESMO Open 5(2):e000626

    Article  PubMed  PubMed Central  Google Scholar 

  • Chowdhary (2019) Does the addition of chemotherapy to neoadjuvant radiotherapy impact survival in high-risk extremity/trunk soft-tissue sarcoma? Cancer 125(21):3801–3809

    Article  CAS  PubMed  Google Scholar 

  • Chudgar (2017a) Is repeat pulmonary metastasectomy indicated for soft tissue sarcoma? Ann Thorac Surg 104(6):1837–1845

    Article  PubMed  PubMed Central  Google Scholar 

  • Chudgar (2017b) Pulmonary metastasectomy with therapeutic intent for soft-tissue sarcoma. J Thorac Cardiovasc Surg 154(1):319–330

    Article  PubMed  PubMed Central  Google Scholar 

  • Comandone (2017) Salvage therapy in advanced adult soft tissue sarcoma: a systematic review and meta-analysis of randomized trials. Oncologist 22(12):1518–1527

    Article  PubMed  PubMed Central  Google Scholar 

  • D’Angelo (2018) Nivolumab with or without ipilimumab treatment for metastatic sarcoma (Alliance A091401): two open-label, non-comparative, randomised, phase 2 trials. Lancet Oncol 19:416–446

    Article  PubMed  PubMed Central  Google Scholar 

  • Dangoor (2016) UK guidelines for the management of soft tissue sarcomas. Clin Sarcoma Res 6:20. eCollection 2016

    Article  PubMed  PubMed Central  Google Scholar 

  • Dei Tos (2020) Evolution in the management of soft tissue sarcoma: classification, surgery and use of radiotherapy. Expert Rev Anticancer Ther 20(suppl 1):3–13

    Article  CAS  PubMed  Google Scholar 

  • Demetri (2013) Results of an international randomized phase III trial of the mammalian target of rapamycin inhibitor ridaforolimus versus placebo to control metastatic sarcomas in patients after benefit from prior chemotherapy. J Clin Oncol 31(19):2485–2492

    Article  CAS  PubMed  Google Scholar 

  • Demetri (2016) 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 34(8):786–793

    Article  CAS  PubMed  Google Scholar 

  • Drilon (2018) Efficacy of larotrectinib in TRK fusion–positive cancers in adults and children. N Engl J Med 378(8):731–739

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Frustaci (2001) Adjuvant chemotherapy for adult soft tissue sarcomas of the extremities and girdles: results of the Italian randomized cooperative trial. J Clin Oncol 19(5):1238–1247

    Article  CAS  PubMed  Google Scholar 

  • Gamboa (2020) Soft-tissue sarcoma in adults: an update on the current state of histiotype-specific management in an era of personalized medicine. CA Cancer J Clin 70(3):200–229

    Article  PubMed  Google Scholar 

  • George (2019) Developments in systemic therapy for soft tissue and bone sarcomas. J Natl Compr Cancer Netw 17(5.5):625–628

    CAS  Google Scholar 

  • Gortzak (2001) A randomised phase II study on neo-adjuvant chemotherapy for ‘high-risk’ adult soft-tissue sarcoma. Eur J Cancer 37(9):1096–1103

    Article  CAS  PubMed  Google Scholar 

  • Gronchi (2012) Short, full-dose adjuvant chemotherapy in high-risk adult soft tissue sarcomas: a randomized clinical trial from the Italian Sarcoma Group and the Spanish Sarcoma Group. J Clin Oncol 30(8):850–856

    Article  CAS  PubMed  Google Scholar 

  • Gronchi (2017) Histotype-tailored neoadjuvant chemotherapy versus standard chemotherapy in patients with high-risk soft-tissue sarcomas (ISG-STS 1001): an international, open-label, randomised, controlled, phase 3, multicentre trial. Lancet Oncol 18(6):812–822

    Article  CAS  PubMed  Google Scholar 

  • Issels (2018) Effect of neoadjuvant chemotherapy plus regional hyperthermia on long-term outcomes among patients with localized high-risk soft tissue sarcoma: the EORTC 62961-ESHO 95 randomized clinical trial. JAMA Oncol 4(4):483–492

    Article  PubMed  PubMed Central  Google Scholar 

  • Judson (2014) Doxorubicin alone versus intensified doxorubicin plus ifosfamide for first-line treatment of advanced or metastatic soft-tissue sarcoma: a randomised controlled phase 3 trial. Lancet Oncol 15(4):415–423

    Article  CAS  PubMed  Google Scholar 

  • Kadle (2019) Flap reconstruction of sarcoma defects in the setting of neoadjuvant and adjuvant radiation. J Reconstr Microsurg 35(4):287–293

    Article  PubMed  Google Scholar 

  • Kelly (2020) Objective response rate among patients with locally advanced or metastatic sarcoma treated with talimogene laherparepvec in combination with pembrolizumab: a phase 2 clinical trial. JAMA Oncol 6(3):402–408

    Article  PubMed  PubMed Central  Google Scholar 

  • Keung (2018) Defining the incidence and clinical significance of lymph node metastasis in soft tissue sarcoma. Eur J Surg Oncol 44(1):170–177

    Article  PubMed  Google Scholar 

  • Le Cesne (2014) Doxorubicin-based adjuvant chemotherapy in soft tissue sarcoma: pooled analysis of two STBSG-EORTC phase III clinical trials. Ann Oncol 25(12):2425–2432

    Article  CAS  PubMed  Google Scholar 

  • Loi (2018) Stereotactic body radiotherapy for oligometastatic soft tissue sarcoma. Radiol Med 123(11):871–878

    Article  PubMed  Google Scholar 

  • MacNeill (2017) Randomized controlled trials in soft tissue sarcoma: we are getting there! Surg Oncol Clin N Am 26(4):531–544

    Article  PubMed  Google Scholar 

  • Mahmoud (2017) Overall survival advantage of chemotherapy and radiotherapy in the perioperative management of large extremity and trunk soft tissue sarcoma; a large database analysis. Radiother Oncol 124(2):277–284

    Article  PubMed  Google Scholar 

  • Mir (2016) Safety and efficacy of regorafenib in patients with advanced soft tissue sarcoma (REGOSARC): a randomised, double-blind, placebo-controlled, phase 2 trial. Lancet Oncol 17(12):1732–1742

    Article  CAS  PubMed  Google Scholar 

  • Nakano (2018) Current molecular targeted therapies for bone and soft tissue sarcomas. Int J Mol Sci 19(3):E739

    Article  PubMed  CAS  Google Scholar 

  • Nakano (2020) Precision medicine in soft tissue sarcoma treatment. Cancers (Basel) 12(1):221

    Article  CAS  Google Scholar 

  • Neuwirth (2017) Isolated limb perfusion and infusion for extremity soft tissue sarcoma: a contemporary systematic review and meta-analysis. Ann Surg Oncol 24(13):3803–3810

    Article  PubMed  Google Scholar 

  • O’Sullivan (2002) Preoperative versus postoperative radiotherapy in soft-tissue sarcoma of the limbs: a randomised trial. Lancet 359(9325):2235–2241

    Article  PubMed  Google Scholar 

  • Osgood (2017) FDA approval summary: eribulin for patients with unresectable or metastatic liposarcoma who have received a prior anthracycline-containing regimen. Clin Cancer Res 23(21):6384–6389

    Article  CAS  PubMed  Google Scholar 

  • Pang (2016) Contemporary therapy for advanced soft-tissue sarcomas in adults: a review. JAMA Oncol 2(7):941–947

    Article  PubMed  Google Scholar 

  • Patel (2019) Overall survival and histology-specific subgroup analyses from a phase 3, randomized controlled study of trabectedin or dacarbazine in patients with advanced liposarcoma or leiomyosarcoma. Cancer 125(15):2610–2620

    Article  CAS  PubMed  Google Scholar 

  • Pervaiz (2008) A systematic meta-analysis of randomized controlled trials of adjuvant chemotherapy for localized resectable soft-tissue sarcoma. Cancer 113(3):573–581

    Article  PubMed  Google Scholar 

  • Petitprez (2020) B cells are associated with survival and immunotherapy response in sarcoma. Nature 577(7791):556–560

    Article  CAS  PubMed  Google Scholar 

  • Pisters (1996) Long-term results of a prospective randomized trial of adjuvant brachytherapy in soft tissue sarcoma. J Clin Oncol 14(3):859–868

    Article  CAS  PubMed  Google Scholar 

  • Pisters (2007) Long-term results of prospective trial of surgery alone with selective use of radiation for patients with T1 extremity and trunk soft tissue sarcomas. Ann Surg 246(4):675–681

    Article  PubMed  Google Scholar 

  • Pollack (2018) Emerging targeted and immune-based therapies in sarcoma. J Clin Oncol 36(2):125–135

    Article  CAS  PubMed  Google Scholar 

  • Quiroga (2020) Activity of PD1 inhibitor therapy in advanced sarcoma: a single-center retrospective analysis. BMC Cancer 20(1):527

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Reed (2008) Phase III randomised study to evaluate the role of adjuvant pelvic radiotherapy in the treatment of uterine sarcomas stages I and II: an European Organisation for Research and Treatment of Cancer Gynaecological Cancer Group Study (protocol 55874). Eur J Cancer 44(6):808–818

    Article  CAS  PubMed  Google Scholar 

  • Rehmani (2020) Adjuvant radiation therapy for thoracic soft tissue sarcomas: a population-based analysis. Ann Thorac Surg 109(1):203–210

    Article  PubMed  Google Scholar 

  • Roeder (2017) Intraoperative radiation therapy (IORT) in soft-tissue sarcoma. Radiat Oncol 12(1):20

    Article  PubMed  PubMed Central  Google Scholar 

  • Rosenberg (1982) The treatment of soft-tissue sarcomas of the extremities: prospective randomized evaluations of limb-sparing surgery plus radiation therapy compared with amputation and the role of adjuvant chemotherapy. Ann Surg 196(3):305–315

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Rosenthal (2020) Nodal metastases of soft tissue sarcomas: risk factors, imaging findings, and implications. Skeletal Radiol 49(2):221–229

    Article  PubMed  Google Scholar 

  • Rossi (2013). Adherence to treatment guidelines for primary sarcomas affects patient survival: a side study of the European CONnective TIssue CAncer NETwork (CONTICANET). Ann Oncol 2013;24(6):1685-1691

    Google Scholar 

  • Ryan (2016) PICASSO III: a phase III, placebo-controlled study of doxorubicin with or without palifosfamide in patients with metastatic soft tissue sarcoma. J Clin Oncol 34(32):3898–3905

    Article  CAS  PubMed  Google Scholar 

  • Salah (2018) Tumor necrosis and clinical outcomes following neoadjuvant therapy in soft tissue sarcoma: a systematic review and meta-analysis. Cancer Treat Rev 69:1–10

    Article  PubMed  Google Scholar 

  • Savina (2017) Patterns of care and outcomes of patients with METAstatic soft tissue SARComa in a real-life setting: the METASARC observational study. BMC Med 15(1):78

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  • Schöffski (2016) Eribulin versus dacarbazine in previously treated patients with advanced liposarcoma or leiomyosarcoma: a randomised, open-label, multicentre, phase 3 trial. Lancet 387(10028):1629–1637

    Article  PubMed  CAS  Google Scholar 

  • Seddon (2017) Gemcitabine and docetaxel versus doxorubicin as first-line treatment in previously untreated advanced unresectable or metastatic soft-tissue sarcomas (GeDDiS): a randomised controlled phase 3 trial. Lancet Oncol 18(10):1397–1410

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Slump (2019) Risk factors for postoperative wound complications after extremity soft tissue sarcoma resection: a systematic review and meta-analyses. J Plast Reconstr Aesthet Surg 72(9):1449–1464

    Article  PubMed  Google Scholar 

  • Smrke (2020) Update on systemic therapy for advanced soft-tissue sarcoma. Curr Oncol 27(Suppl 1):25–33

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Spunt (2020) A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children’s Oncology Group prospective study. Lancet Oncol 21(1):145–161

    Article  CAS  PubMed  Google Scholar 

  • Subbiah (2018) Multimodality treatment of desmoplastic small round cell tumor: chemotherapy and complete cytoreductive surgery improve patient survival. Clin Cancer Res 24(19):4865–4873

    Article  PubMed  PubMed Central  Google Scholar 

  • Tap (2017) Doxorubicin plus evofosfamide versus doxorubicin alone in locally advanced, unresectable or metastatic soft-tissue sarcoma (TH CR-406/SARC021): an international, multicentre, open-label, randomised phase 3 trial. Lancet Oncol 18(8):1089–1103

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Tap (2019) ANNOUNCE: a randomized, placebo (PBO)-controlled, double-blind, phase (Ph) III trial of doxorubicin (dox) + olaratumab versus dox + PBO in patients (pts) with advanced soft tissue sarcomas (STS). J Clin Oncol 37(18_suppl):LBA3

    Article  Google Scholar 

  • Tawbi (2017) Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (sarc028): a multicentre, two-cohort, single-arm, open-label, phase 2 trial. Lancet Oncol 18:1493–1501

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Trédan (2019) Molecular screening program to select molecular-based recommended therapies for metastatic cancer patients: analysis from the ProfiLER trial. Ann Oncol 30(5):757–765

    Article  PubMed  Google Scholar 

  • van der Graaf Winette (2012) Pazopanib for metastatic soft-tissue sarcoma (PALETTE): a randomised, double-blind, placebo-controlled phase 3 trial. Lancet 379:1879–1886

    Article  CAS  PubMed  Google Scholar 

  • van Houdt (2017) Treatment of retroperitoneal sarcoma: current standards and new developments. Curr Opin Oncol 29(4):260–267

    Article  CAS  PubMed  Google Scholar 

  • Vieira (2020) Response to anti-PD1 immunotherapy in patients with metastatic cutaneous sarcoma: case reports and literature review. Oxf Med Case Reports 2020(1):omz138

    Article  PubMed  PubMed Central  Google Scholar 

  • von Mehren (2018) Soft tissue sarcoma, version 2.2018, NCCN clinical practice guidelines in oncology. J Natl Compr Cancer Netw 16(5):536–563

    Article  Google Scholar 

  • Voss (2017) Adherence to national comprehensive cancer network guidelines is associated with improved survival for patients with stage 2A and stages 2B and 3 extremity and superficial trunk soft tissue sarcoma. Ann Surg Oncol 24(11):3271–3278

    Article  PubMed  PubMed Central  Google Scholar 

  • Wagner (2017) Detection of lymph node metastases in pediatric and adolescent/young adult sarcoma: sentinel lymph node biopsy versus fludeoxyglucose positron emission tomography imaging-A prospective trial. Cancer 123(1):155–160

    Article  CAS  PubMed  Google Scholar 

  • Wang (2015) 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 33(20):2231–2238

    Article  PubMed  PubMed Central  Google Scholar 

  • Wardelmann (2016) Evaluation of response after neoadjuvant treatment in soft tissue sarcomas; the European Organization for Research and Treatment of Cancer-Soft Tissue and Bone Sarcoma Group (EORTC-STBSG) recommendations for pathological examination and reporting. Eur J Cancer 53:84–95

    Article  CAS  PubMed  Google Scholar 

  • Wilding (2019) The landscape of tyrosine kinase inhibitors in sarcomas: looking beyond pazopanib. Expert Rev Anticancer Ther 19(11):971–991

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Wilky (2019) Axitinib plus pembrolizumab in patients with advanced sarcomas including alveolar soft-part sarcoma: a single-centre, single-arm, phase 2 trial. Lancet Oncol 20:837–848

    Article  CAS  PubMed  Google Scholar 

  • Wisdom (2018) Rationale and emerging strategies for immune checkpoint blockade in soft tissue sarcoma. Cancer 124(19):3819–3829

    Article  PubMed  Google Scholar 

  • Woll (2012) Adjuvant chemotherapy with doxorubicin, ifosfamide, and lenograstim for resected soft-tissue sarcoma (EORTC 62931): a multicentre randomised controlled trial. Lancet Oncol 13(10):1045–1054

    Article  CAS  PubMed  Google Scholar 

  • Wright (2012) The role of sentinel lymph node biopsy in select sarcoma patients: a meta-analysis. Am J Surg 204(4):428–433

    Article  PubMed  Google Scholar 

  • Yang (1998) Randomized prospective study of the benefit of adjuvant radiation therapy in the treatment of soft tissue sarcomas of the extremity. J Clin Oncol 16(1):197–203

    Article  CAS  PubMed  Google Scholar 

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Mocellin, S. (2021). Therapy. In: Soft Tissue Tumors . Springer, Cham. https://doi.org/10.1007/978-3-030-58710-9_7

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