Cardiac Tumors: Multimodality Approach, Follow-Up, and Prognosis

  • Antonino De Paoli
  • Gian Maria Miolo
  • Angela Buonadonna


Cardiac tumors include mainly primary lymphomas and sarcomas. Cardiac lymphomas are usually treated with chemotherapy (CT), with a good rate of success at least at short-medium term, even if survival is worse than in non-cardiac lymphomas. Surgery may be indicated in selected cases in the presence of life-threatening hemodynamic impairment. Radiation therapy may be considered for palliation in non-response or progressive disease after CT. For cardiac sarcomas the mainstay of treatment is surgery, but CT and radiotherapy (RT) may play a relevant role. Preoperative CT should be considered in unresectable and borderline resectable tumors, to increase the probability of a wide resection with negative margins. Adjuvant CT may be used in high risk tumors. Anthracyclines and ifosfamide are the more active drugs. CT must be planned by a medical oncologist with experience in the management of sarcomas. Postoperative RT may be considered after CT. Combined CT and RT may be considered in unresectable or relapsing tumors to reduce tumor burden and prolong the progression-free survival. Radiation therapy is challenging and must be planned by a radiation oncologist with experience in the management of sarcomas. The collaboration with an experienced cardiologist is recommended. A cardiologic monitoring during treatment should be planned.


  1. 1.
    Antoniades L, Eftychiou C, Petrou PM, et al. Primary cardiac lymphoma: case report and brief review of the literature. Echocardiography. 2009;26:214–9.CrossRefPubMedGoogle Scholar
  2. 2.
    Lilje C, Thiel C, Weil J, et al. Non-surgical management of advanced cardiac lymphoma. J Pediatr. 2008;152:440.CrossRefPubMedGoogle Scholar
  3. 3.
    Mohsen A, Najafi AH, Zhou L, et al. Massive, rapidly growing cardiac lymphoma with rare valvular involvement showing excellent response to chemotherapy. Can J Cardiol. 2013;29:1139. e3-4.CrossRefPubMedGoogle Scholar
  4. 4.
    Oliveira GH, Al-Kindi SG, Hoimes C, Park SJ. Characteristics and survival of malignant cardiac tumors: a 40-year analysis of over 500 patients. Circulation. 2015;132:2395–402.CrossRefPubMedGoogle Scholar
  5. 5.
    Gosev I, Sirić F, Gasparović H, et al. Surgical treatment of a primary cardiac lymphoma presenting with tamponade physiology. J Card Surg. 2006;21:414–6.CrossRefPubMedGoogle Scholar
  6. 6.
    Lestuzzi C, Spina M, Martellotta F, Carbone A. Massive myocardial infiltration by HIV-related non-Hodgkin lymphoma: echocardiographic aspects at diagnosis and at follow-up. J Cardiovasc Med (Hagerstown). 2012;13:836–8.CrossRefGoogle Scholar
  7. 7.
    Tilly H, Dreyling M, ESMO Guidelines Working Group. Diffuse large B-cell non-Hodgkin’s lymphoma: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2010;21 Suppl 5:v172–4.CrossRefPubMedGoogle Scholar
  8. 8.
    Howard SC, Jones DP, Pui CH. The tumor lysis syndrome. N Engl J Med. 2011;364:1844–54.CrossRefPubMedPubMedCentralGoogle Scholar
  9. 9.
    Pi J, Kang Y, Smith M, et al. A review in the treatment of oncologic emergencies. J Oncol Pharm Pract. 2015 Oct 6.Google Scholar
  10. 10.
    Kim MP, Correa AM, Blackmon S, et al. Outcomes after right-side heart sarcoma resection. Ann Thorac Surg. 2011;91:770–6.CrossRefPubMedGoogle Scholar
  11. 11.
    Ramlawi B, Leja MJ, Abu Saleh WK, et al. Surgical treatment of primary cardiac sarcomas: review of a single-institution experience. Ann Thorac Surg. 2015 Oct 14.Google Scholar
  12. 12.
    Patrikidou A, Domont J, Cioffi A, Le Cesne A. Treating soft tissue sarcomas with adjuvant chemotherapy. Curr Treat Options Oncol. 2011;12:21–31.CrossRefPubMedGoogle Scholar
  13. 13.
    Le Cesne A, Ouali M, Leahy MG, et al. Doxorubicin-based adjuvant chemotherapy in soft tissue sarcoma: pooled analysis of two STBSG-EORTC phase III clinical trials. Ann Oncol. 2014;25:2425–32.CrossRefPubMedGoogle Scholar
  14. 14.
    Ravi V, Benjamin RS. Systemic therapy for cardiac sarcomas. MDCVJ. 2010;VI(3):57–60.Google Scholar
  15. 15.
    Schlemmer M, Reichardt P, Verweij J, et al. Paclitaxel in patients with advanced angiosarcomas of soft tissue: a retrospective study of the EORTC soft tissue and bone sarcoma group. Eur J Cancer. 2008;44:2433–6.CrossRefPubMedGoogle Scholar
  16. 16.
    Oxenberg J, Khushalani NI, Salerno KE, et al. Neoadjuvant chemotherapy for primary cutaneous/soft tissue angiosarcoma: determining tumor behavior prior to surgical resection. J Surg Oncol. 2015;111:829–33.CrossRefPubMedGoogle Scholar
  17. 17.
    Liebner DA. The indications and efficacy of conventional chemotherapy in primary and recurrent sarcoma. J Surg Oncol. 2015;111:622–31.CrossRefPubMedGoogle Scholar
  18. 18.
    van der Graaf WT, Blay JY, Chawla SP, EORTC Soft Tissue and Bone Sarcoma Group, PALETTE study group, et al. Pazopanib for metastatic soft-tissue sarcoma (PALETTE): a randomised, double-blind, placebo-controlled phase 3 trial. Lancet. 2012;379:1879–86.CrossRefPubMedGoogle Scholar
  19. 19.
    Basavaraju SR, Easterly CE. Pathophysiological effects of radiation on atherosclerosis development and progression, and the incidence of cardiovascular complications. Med Phys. 2002;29:2391–403.CrossRefPubMedGoogle Scholar
  20. 20.
    Thariat J, Clément-Colmou K, Vogin G, et al. Radiation therapy of cardiac sarcomas. Cancer Radiother. 2014;18:125–31.CrossRefPubMedGoogle Scholar
  21. 21.
    Ipsen S, Blanck O, Oborn B, et al. Radiotherapy beyond cancer: target localization in real-time MRI and treatment planning for cardiac radiosurgery. Med Phys. 2014;41:120702.CrossRefPubMedGoogle Scholar
  22. 22.
    Thariat J, Clément-Colmou K, Vogin G, et al. Radiation therapy of cardiac sarcomas. Cancer Radiother. 2014;18:125–31.CrossRefPubMedGoogle Scholar
  23. 23.
    Roeder F, Nicolay NH, Nguyen T, et al. Intensity modulated radiotherapy (IMRT) with concurrent chemotherapy as definitive treatment of locally advanced esophageal cancer. Radiat Oncol. 2014;9:191.CrossRefPubMedPubMedCentralGoogle Scholar
  24. 24.
    Eisenhauer EA, Therasse P, Bogaerts J, et al. New response evaluation criteria in solid tumours: revised RECIST guideline (version 1.1). Eur J Cancer. 2009;45:228–47.CrossRefPubMedGoogle Scholar
  25. 25.
    Hollebecque A, Adenis A, Taieb S, et al. Inadequacy of size-based response criteria to assess the efficacy of trabectedin among metastatic sarcoma patients. Invest New Drugs. 2010;28:529–30.CrossRefPubMedGoogle Scholar
  26. 26.
    Taieb S, Saada-Bouzid E, Tresch E, French Sarcoma Group, et al. Comparison of response evaluation criteria in solid tumours and Choi criteria for response evaluation in patients with advanced soft tissue sarcoma treated with trabectedin: a retrospective analysis. Eur J Cancer. 2015;51:202–9.CrossRefPubMedGoogle Scholar
  27. 27.
    Choi H, Charnsangavej C, Faria SC, et al. Correlation of computed tomography and positron emission tomography in patients with metastatic gastrointestinal stromal tumor treated at a single institution with imatinib mesylate: proposal of new computed tomography response criteria. J Clin Oncol. 2007;25:1753–9.CrossRefPubMedGoogle Scholar
  28. 28.
    Lencioni R, Llovet JM. Modified RECIST (mRECIST) assessment for hepatocellular carcinoma. Semin Liver Dis. 2010;30:52–60.CrossRefPubMedGoogle Scholar
  29. 29.
    Ueda T, Morioka H, Nishida Y, et al. Objective tumor response to denosumab in patients with giant cell tumor of bone: a multicenter phase II trial. Ann Oncol. 2015;26:2149–54.CrossRefPubMedPubMedCentralGoogle Scholar
  30. 30.
    Evilevitch V, Weber WA, Tap WD, et al. Reduction of glucose metabolic activity is more accurate than change in size at predicting histopathologic response to neoadjuvant therapy in high-grade soft-tissue sarcomas. Clin Cancer Res. 2008;14:715–20.CrossRefPubMedGoogle Scholar
  31. 31.
    Benz MR, Czernin J, Allen-Auerbach MS, et al. FDG-PET/CT imaging predicts histopathologic treatment responses after the initial cycle of neoadjuvant chemotherapy in high-grade soft-tissue sarcomas. Clin Cancer Res. 2009;15:2856–63.CrossRefPubMedPubMedCentralGoogle Scholar
  32. 32.
    Herrmann K, Benz MR, Czernin J, et al. 18F-FDG-PET/CT Imaging as an early survival predictor in patients with primary high-grade soft tissue sarcomas undergoing neoadjuvant therapy. Clin Cancer Res. 2012;18:2024–31.CrossRefPubMedPubMedCentralGoogle Scholar
  33. 33.
    Li H, Xu D, Chen Z, et al. Prognostic analysis for survival after resections of localized primary cardiac sarcomas: a single- institution experience. Ann Thorac Surg. 2014;97:1379–85.CrossRefPubMedGoogle Scholar
  34. 34.
    Isambert N, Ray-Coquard I, Italiano A, et al. Primary cardiac sarcomas: a retrospective study of the French Sarcoma Group. Eur J Cancer. 2014;50:128–36.CrossRefPubMedGoogle Scholar
  35. 35.
    Randhawa JS, Budd GT, Randhawa M, et al. Primary cardiac sarcoma: 25-Year Cleveland clinic experience. Am J Clin Oncol. 2014 Jul 17.Google Scholar

Copyright information

© Springer International Publishing Switzerland 2017

Authors and Affiliations

  • Antonino De Paoli
    • 1
  • Gian Maria Miolo
    • 2
  • Angela Buonadonna
    • 2
  1. 1.Department of Radiation OncologyCRO-National Cancer InstituteAviano (PN)Italy
  2. 2.Department of Medical OncologyCRO-National Cancer InstituteAviano (PN)Italy

Personalised recommendations