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Basic Knowledge in Soft Tissue Sarcoma

Abstract

Sarcoma is rare and heterogenous with various subtypes having a different prognostic. Desmoid is a tumour with a local aggressiveness; GIST with KIT mutation responds massively to target treatment as IMATINIB, whereas soft tissue sarcoma and leiomyosarcoma are very aggressive with poor response to systemic therapies. Interventional radiology plays an important role in the diagnosis of sarcomas with image-guided percutaneous core needle biopsy being the most commonly used biopsy technique in the diagnosis of sarcomas. Biopsy access routes discussed with the surgeon, and skin access is tattooed. Surgery is a mainstay of sarcoma treatment; the resection can be large. Indeed, resection objective is R0 because quality of surgical margins impacts local control and survival. Radiotherapy is possible in neoadjuvant or in adjuvant treatment to improve local control rate. Recently radiotherapy enhancer injected percutaneously in soft tissue sarcoma has proven benefit in increasing the rate of R0 complete surgical resection. Several studies showed better local control rate linked with post-operative radiotherapy. In patients affected by oligometastatic disease, complete surgical resection of all metastatic sites is in fact considered the primary treatment because complete remission is critical for cure. The decision making to use local therapies is complex, depends upon diverse presentations and histologies, and should always be taken in a multidisciplinary discussion. Today, percutaneous image-guided treatments with ablation technologies (radiofrequency ablation, cryotherapy, microwaves ablation) provide high rate of durable local control for small-sized malignant deposit in many organs including lung, liver and bones. Sarcoma must be managed by multimodality treatment in expert reference centres. Such management has a considerable impact on the prognosis.

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References

  1. 1.

    Hui JYC. Epidemiology and etiology of sarcomas. Surg Clin North Am. 2016;96:901–14.

    Article  PubMed  Google Scholar 

  2. 2.

    Gatta G, van der Zwan JM, Casali PG, Siesling S, Dei Tos AP, Kunkler I, et al. Rare cancers are not so rare: the rare cancer burden in Europe. Eur J Cancer Oxf Engl. 1990;2011(47):2493–511.

    Google Scholar 

  3. 3.

    Coindre JM, Terrier P, Bui NB, Bonichon F, Collin F, Le Doussal V, et al. Prognostic factors in adult patients with locally controlled soft tissue sarcoma. A study of 546 patients from the French Federation of Cancer Centers Sarcoma Group. J Clin Oncol. 1996;14:869–77.

    Article  CAS  PubMed  Google Scholar 

  4. 4.

    Farid M, Ngeow J. Sarcomas associated with genetic cancer predisposition syndromes: a review. Oncologist. 2016;21:1002–13.

    Article  PubMed  PubMed Central  Google Scholar 

  5. 5.

    Bourhafour M, Bourhafour I, El Youbi MBA, M’Rabti H, Benjaafar N, Errihani H. Spécificité de la transformation sarcomateuse de la maladie de Recklinghaussen: a propos de deux cas et revue de la littérature. Pan Afr Med J. 2013;15. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3810010/. Accessed 13 May 2018.

  6. 6.

    Kleinerman RA, Schonfeld SJ, Tucker MA. Sarcomas in hereditary retinoblastoma. Clin Sarcoma Res. 2012;2:15.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  7. 7.

    Gandhi S, Chandna S. Radiation-induced inflammatory cascade and its reverberating crosstalks as potential cause of post-radiotherapy second malignancies. Cancer Metastasis Rev. 2017;36:375–93.

    Article  CAS  PubMed  Google Scholar 

  8. 8.

    Lahat G, Lazar A, Lev D. Sarcoma epidemiology and etiology: potential environmental and genetic factors. Surg Clin North Am. 2008;88(451–81):v.

    Google Scholar 

  9. 9.

    Radons J. The role of inflammation in sarcoma. Adv Exp Med Biol. 2014;816:259–313.

    Article  CAS  PubMed  Google Scholar 

  10. 10.

    Coindre J-M. Molecular biology of soft-tissue sarcomas. Bull Cancer (Paris). 2010;97:1337–45.

    Article  CAS  Google Scholar 

  11. 11.

    Blay J-Y, Soibinet P, Penel N, Bompas E, Duffaud F, Stoeckle E, et al. Improved survival using specialized multidisciplinary board in sarcoma patients. Ann Oncol. 2017;28:2852–9.

    Article  PubMed  PubMed Central  Google Scholar 

  12. 12.

    Casali PG, Abecassis N, Bauer S, Biagini R, Bielack S, Bonvalot S, et al. Soft tissue and visceral sarcomas: ESMO–EURACAN clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2018;29:iv51–67.

    Article  CAS  Google Scholar 

  13. 13.

    Bonvalot S, Missana M-C, Le Cesne A, Missenard G, Vanel D, Terrier P, et al. Chirurgie des sarcomes des tissus mous des membres chez l’adulte: historique et avancées thérapeutiques. Cancer/Radiothérapie. 2006;10:416–24.

    Article  CAS  Google Scholar 

  14. 14.

    Gronchi A, Lo Vullo S, Colombo C, Collini P, Stacchiotti S, Mariani L, et al. Extremity soft tissue sarcoma in a series of patients treated at a single institution: local control directly impacts survival. Ann Surg. 2010;251:506–11.

    Article  PubMed  Google Scholar 

  15. 15.

    Nakamura T, Kawai A, Sudo A. Analysis of the patients with soft tissue sarcoma who received additional excision after unplanned excision: report from the Bone and soft tissue tumor registry in Japan. Jpn J Clin Oncol. 2017;47:1055–9.

    Article  PubMed  Google Scholar 

  16. 16.

    Ogura K, Yasunaga H, Horiguchi H, Ohe K, Shinoda Y, Tanaka S, et al. Impact of hospital volume on postoperative complications and in-hospital mortality after musculoskeletal tumor surgery: analysis of a national administrative database. J Bone Jt Surg Am. 2013;95:1684–91.

    Article  Google Scholar 

  17. 17.

    Moureau-Zabotto L, Delannes M, Le Péchoux C, Sunyach MP, Kantor G, Sargos P, et al. Prise en charge des sarcomes des tissus mous des membres par radiothérapie externe. Cancer/Radiothérapie. 2016;20:133–40.

    Article  CAS  Google Scholar 

  18. 18.

    First-in-Class NBTXR3 Provides a New Option for Preoperative Treatment in Patients with Locally Advanced STS (Act-In-Sarc)|ESMO. https://www.esmo.org/Oncology-News/NBTXR3-locally-advance-soft-tissue-sarcoma-Bonvalot. Accessed 11 Dec 2018.

  19. 19.

    Casali PG, Abecassis N, Bauer S, Biagini R, Bielack S, Bonvalot S, et al. Soft tissue and visceral sarcomas: ESMO–EURACAN clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2018;29:iv51–67.

    Article  CAS  PubMed  Google Scholar 

  20. 20.

    Gronchi A, Ferrari S, Quagliuolo V, Broto JM, Pousa AL, Grignani G, et al. 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. 2017;18:812–22.

    Article  CAS  Google Scholar 

  21. 21.

    Neuwirth MG, Song Y, Sinnamon AJ, Fraker DL, Zager JS, Karakousis GC. Isolated limb perfusion and infusion for extremity soft tissue sarcoma: a contemporary systematic review and meta-analysis. Ann Surg Oncol. 2017;24:3803–10.

    Article  PubMed  Google Scholar 

  22. 22.

    Issels RD, Lindner LH, Verweij J, Wust P, Reichardt P, Schem B-C, et al. Neo-adjuvant chemotherapy alone or with regional hyperthermia for localised high-risk soft-tissue sarcoma: a randomised phase 3 multicentre study. Lancet Oncol. 2010;11:561–70.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. 23.

    Hellman S, Weichselbaum RR. Oligometastases. J Clin Oncol. 1995;13:8–10.

    Article  CAS  Google Scholar 

  24. 24.

    Kissel M, Helou J, Thariat J. Nouvelles définitions de la maladie oligométastatique et nouveaux concepts de prise en charge globale de la maladie métastatique. Bull Cancer (Paris). 2018;105:696–706.

    Article  Google Scholar 

  25. 25.

    ESMO/European Sarcoma Network Working Group. Bone sarcomas: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2014;25(Suppl 3):iii113–23.

    Article  Google Scholar 

  26. 26.

    Falk AT, Moureau-Zabotto L, Ouali M, Penel N, Italiano A, Bay J-O, et al. Effect on survival of local ablative treatment of metastases from sarcomas: a study of the French sarcoma group. Clin Oncol R Coll Radiol G B. 2015;27:48–55.

    Article  CAS  Google Scholar 

  27. 27.

    Briccoli A, Rocca M, Salone M, Guzzardella GA, Balladelli A, Bacci G. High grade osteosarcoma of the extremities metastatic to the lung: long-term results in 323 patients treated combining surgery and chemotherapy, 1985–2005. Surg Oncol. 2010;19:193–9.

    Article  PubMed  Google Scholar 

  28. 28.

    Penel N, Italiano A, Ray-Coquard I, Chaigneau L, Delcambre C, Robin YM, et al. Metastatic angiosarcomas: doxorubicin-based regimens, weekly paclitaxel and metastasectomy significantly improve the outcome. Ann Oncol. 2012;23:517–23.

    Article  CAS  PubMed  Google Scholar 

  29. 29.

    Stanelle EJ, Christison-Lagay ER, Wolden SL, Meyers PA, La Quaglia MP. Pulmonary metastasectomy in pediatric/adolescent patients with synovial sarcoma: an institutional review. J Pediatr Surg. 2013;48:757–63.

    Article  PubMed  Google Scholar 

  30. 30.

    Yevich S, Gaspar N, Tselikas L, Brugières L, Pacquement H, Schleiermacher G, et al. Percutaneous computed tomography-guided thermal ablation of pulmonary osteosarcoma metastases in children. Ann Surg Oncol. 2016;23:1380–6.

    Article  PubMed  Google Scholar 

  31. 31.

    Judson I, Verweij J, Gelderblom H, Hartmann JT, Schöffski P, Blay J-Y, et al. 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. 2014;15:415–23.

    Article  CAS  PubMed  Google Scholar 

  32. 32.

    Mir O, Brodowicz T, Italiano A, Wallet J, Blay J-Y, Bertucci F, et al. Safety and efficacy of regorafenib in patients with advanced soft tissue sarcoma (REGOSARC): a randomised, double-blind, placebo-controlled, phase 2 trial. Lancet Oncol. 2016;17:1732–42.

    Article  CAS  PubMed  Google Scholar 

  33. 33.

    Gounder MM, Somaiah N, Attia S, Chawla SP, Villalobos VM, Chmielowski B, et al. Phase 2 results of selinexor in advanced de-differentiated (DDLS) liposarcoma (SEAL) study: a phase 2/3, randomized, double blind, placebo controlled cross-over study. J Clin Oncol. 2018;36:11512.

    Article  Google Scholar 

  34. 34.

    Bourcier K, Italiano A. Newer therapeutic strategies for soft-tissue sarcomas. Pharmacol Ther. 2018;188:118–23.

    Article  CAS  PubMed  Google Scholar 

  35. 35.

    Gounder MM, Stacchiotti S, Schöffski P, Attia S, Italiano A, Jones R, et al. Phase 2 multicenter study of the EZH2 inhibitor tazemetostat in adults with INI1 negative epithelioid sarcoma (NCT02601950). J Clin Oncol. 2017;35:11058.

    Article  Google Scholar 

  36. 36.

    Kelly CM, Bowler TG, Munhoz RR, Chi P, Dickson MA, Gounder MM, et al. A phase II study of talimogene laherparepvec (T-VEC) and pembrolizumab in patients with metastatic sarcoma. J Clin Oncol. 2018;36:11516.

    Article  Google Scholar 

  37. 37.

    de Baère T, Aupérin A, Deschamps F, Chevallier P, Gaubert Y, Boige V, et al. Radiofrequency ablation is a valid treatment option for lung metastases: experience in 566 patients with 1037 metastases. Ann Oncol. 2015;26:987–91.

    Article  PubMed  PubMed Central  Google Scholar 

  38. 38.

    Jung J-H, Won HJ, Shin YM, Kim PN. Safety and efficacy of radiofrequency ablation for hepatic metastases from gastrointestinal stromal tumor. J Vasc Interv Radiol JVIR. 2015;26:1797–802.

    Article  PubMed  Google Scholar 

  39. 39.

    Jun HY, Ryu J-H, Byun SJ, Jeong CW, Kim T-H, Lee YH, et al. Combined radiofrequency ablation and double anti-angiogenic protein therapy to increase coagulation efficacy: an experimental study in a murine renal carcinoma model. Korean J Radiol. 2015;16:776–82.

    Article  PubMed  PubMed Central  Google Scholar 

  40. 40.

    Deschamps F, Farouil G, Ternes N, Gaudin A, Hakime A, Tselikas L, et al. Thermal ablation techniques: a curative treatment of bone metastases in selected patients? Eur Radiol. 2014;24:1971–80.

    Article  CAS  PubMed  Google Scholar 

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Correspondence to Thierry de Baere.

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Bourcier, K., Le Cesne, A., Tselikas, L. et al. Basic Knowledge in Soft Tissue Sarcoma. Cardiovasc Intervent Radiol 42, 1255–1261 (2019). https://doi.org/10.1007/s00270-019-02259-w

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Keywords

  • Sarcoma
  • Soft tissue sarcoma
  • Stromal tumor
  • Interventional oncology