Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub. 2023, 167(2):116-120 | DOI: 10.5507/bp.2022.011

Expansive intracardiac leiomyosarcoma - a combined therapeutic approach resulting in survival with complete remission. A literature review and case report

Jan Juchelka1, Martin Simek1, Pavel Marcian1, Jiri Ehrmann2, Petr Santavy1
1 Department of Cardiac Surgery, University Hospital Olomouc, Czech Republic
2 Department of Clinical and Molecular Pathology, University Hospital Olomouc, Czech Republic

Primary cardiac sarcomas are extremely rare and often with dismal prognosis. Only a few case series and retrospective studies regarding its biological characteristics, diagnostics, and treatment were reported. The multi-modality therapeutic strategy has been discussed in the published literature, but often with contradictory results. There is thus, no consensus on the optimal therapeutic approach to date. We present the case report of the 66-year old female endangered by a large primary leiomyosarcoma expanding in the right-sided heart chambers with imminent risk of acute obstruction of blood flow. The patient was managed by urgent surgical resection. After the histological confirmation of incomplete R1 resection, the treatment was supplemented by adjuvant CT-targeted radiotherapy, resulting in extraordinary survival with complete remission over a 24-month follow-up period. Our case report aims to demonstrate a favorable result of an individually suited complex surgical and oncological treatment to support the multidisciplinary therapeutic approach to these patients. The article is supplemented by a detailed literature review providing a theoretical background and an overview of the acquired knowledge and possible strategies.

Keywords: leiomyosarcoma, cardiac tumour, cardiac surgery, radiotherapy

Received: October 24, 2021; Revised: February 27, 2022; Accepted: March 1, 2022; Prepublished online: March 9, 2022; Published: June 12, 2023  Show citation

ACS AIP APA ASA Harvard Chicago Chicago Notes IEEE ISO690 MLA NLM Turabian Vancouver
Juchelka, J., Simek, M., Marcian, P., Ehrmann, J., & Santavy, P. (2023). Expansive intracardiac leiomyosarcoma - a combined therapeutic approach resulting in survival with complete remission. A literature review and case report. Biomedical papers, 167(2), 116-120. doi: 10.5507/bp.2022.011
Share...
Download citation
  • BibTeX (.bib)
  • Bookends (.ris)
  • EasyBib (.ris)
  • EndNote (.enw)
  • EndNote 8 (.xml)
  • ISI WoS (.isi)
  • Medlars (.medlars)
  • Mendeley (.ris)
  • MODS (.xml)
  • Papers (.ris)
  • RefWorks (.txt)
  • RefManager (.ris)
  • RIS (.ris)
  • MS Word (.xml)
  • Zotero (.ris)
    • Open full article

    References

    1. Butany J, Nair V, Naseemuddin A, Nair GM, Catton C, Yau T. Cardiac tumours: diagnosis and management. Lancet Oncol 2005;6(4):219-28. Go to original source... Go to PubMed...
    2. Neragi-Miandoab S, Kim J, Vlahakes GJ. Malignant tumours of the heart: a review of tumour type, diagnosis and therapy. Clin Oncol (R Coll Radiol) 2007;19(10):748-56. Go to original source... Go to PubMed...
    3. Castillo JG, Silvay G, Boateng P. Characterization of surgical cardiac tumors. Bratisl Lek Listy 2016;117(1):3-10. Go to original source... Go to PubMed...
    4. Zhang PJ, Brooks JS, Goldblum JR, Yoder B, Seethala R, Pawel B, Gorman JH, Gorman RC, Huang JH, Acker M, Narula N. Primary cardiac sarcomas: a clinicopathologic analysis of a series with follow-up information in 17 patients and emphasis on long-term survival. Hum Pathol 2008;39(9):1385-395. Go to original source... Go to PubMed...
    5. Donsbeck AV, Ranchere D, Coindre JM, Le Gall F, Cordier JF, Loire R. Primary cardiac sarcomas: an immunohistochemical and grading study with long-term follow-up of 24 cases. Histopathology 1999;34(4):295-304. Go to original source... Go to PubMed...
    6. Bakaeen FG, Reardon MJ, Coselli JS, Miller CC, Howell JF, Lawrie GM, Espada R, Ramchandani MK, Noon GP, Weilbaecher DG, DeBakey ME. Surgical outcome in 85 patients with primary cardiac tumors. Am J Surg 2003;186(6):641-47. Go to original source... Go to PubMed...
    7. Paraskevaidis IA, Michalakeas CA, Papadopoulos CH, Anastasiou-Nana M. Cardiac tumors. ISRN Oncol 2011;2011:208929. Go to original source... Go to PubMed...
    8. Burke AP, Cowan D, Virmani R. Primary sarcomas of the heart. Cancer 1992;69(2):387-95. Go to original source...
    9. Vander Salm TJ. Unusual primary tumors of the heart. Semin Thorac Cardiovasc Surg 2000;12(2):89-100. Go to original source... Go to PubMed...
    10. Parissis H, Akbar MT, Young V. Primary leiomyosarcoma of the right atrium: a case report and literature update. J Cardiothorac Surg 2010;5:80 doi: 10.1186/1749-8090-5-80 Go to original source... Go to PubMed...
    11. Castillo JG, Silvay G. Characterization and management of cardiac tumors. Semin Cardiothorac Vasc Anesth 2010;14(1):6-20. Go to original source... Go to PubMed...
    12. Grebenc ML, Rosado de Christenson ML, Burke AP, Green CE, Galvin JR. Primary cardiac and pericardial neoplasms: radiologic-pathologic correlation. Radiographics 2000;20(4):1073-112. Go to original source... Go to PubMed...
    13. Faught PR, Waller BF, Hull MT. Spindle cell sarcoma of the heart in childhood: light microscopic, ultrastructural, and immunohistochemical evidence for smooth muscle, endothelial, and fibroblastic differentiation. Pediatr Pathol 1988;8(6):649-56. Go to original source... Go to PubMed...
    14. Jellis C, Doyle J, Sutherland T, Gutman J, Macisaac A. Cardiac epithelioid leiomyosarcoma and the role of cardiac imaging in the differentiation of intracardiac masses. Clin Cardiol 2010;33(6):E6-E9. Go to original source... Go to PubMed...
    15. Yuan SM, Shinfeld A, Lavee J, Kuperstein R, Haizler R, Raanani E. Imaging morphology of cardiac tumours. Cardiol J 2009;16(1):26-35.
    16. Ann SH, Jung SY, Yi JE, et al. A primary left atrial leiomyosarcoma mimicking myxoma: echocardiographic findings. Echocardiography 2012;29(9):E226-E229. Go to original source... Go to PubMed...
    17. Astarcioğlu MA, Kalçik M, Gürsoy MO, Gündüz S, Özkan M. Multimodality imaging of a recurrent case of right-sided cardiac leiomyosarcoma with an unusual clinical course. Turk Kardiyol Dern Ars 2015;43(4):372-75. Go to PubMed...
    18. Araoz PA, Eklund HE, Welch TJ, Breen JF. CT and MR imaging of primary cardiac malignancies. Radiographics 1999;19(6):1421-34. Go to original source... Go to PubMed...
    19. Patel J, Sheppard MN. Pathological study of primary cardiac and pericardial tumours in a specialist UK Centre: surgical and autopsy series. Cardiovasc Pathol 2010;19(6):343-52. Go to original source... Go to PubMed...
    20. Neuville A, Collin F, Bruneval P, Parrens M, Thivolet F, Gomez-Brouchet A, Terrier P, de Montpreville VT, Le Gall F, Hostein I, Lagarde P, Chibon F, Coindre JM. Intimal sarcoma is the most frequent primary cardiac sarcoma: clinicopathologic and molecular retrospective analysis of 100 primary cardiac sarcomas. Am J Surg Pathol 2014;38(4):461-69. Go to original source... Go to PubMed...
    21. Winther C, Timmermans-Wielenga V, Daugaard S, Mortensen SA, Sander K, Andersen CB. Primary cardiac tumors: a clinicopathologic evaluation of four cases. Cardiovasc Pathol 2011;20(1):63-7. Go to original source... Go to PubMed...
    22. Malyshev M, Safuanov A, Gladyshev I, Trushyna V, Abramovskaya L, Malyshev A. Primary left atrial leiomyosarcoma: literature review and lessons of a case. Asian Cardiovasc Thorac Ann 2006;14(5):435-40. Go to original source... Go to PubMed...
    23. Pacini D, Careddu L, Pantaleo A, Parolari A, Leone O, Daprati A, Gargiulo GD, Di Bartolomeo R. Primary malignant tumors of the heart: Outcomes of the surgical treatment. Asian Cardiovasc Thorac Ann 2015;23(6):645-51. Go to original source... Go to PubMed...
    24. Molina JE, Edwards JE, Ward HB. Primary cardiac tumors: experience at the University of Minnesota. Thorac Cardiovasc Surg 1990;38(Suppl 2):183-91. Go to original source... Go to PubMed...
    25. Llombart-Cussac A, Pivot X, Contesso G, Rhor-Alvarado A, Delord JP, Spielmann M, Türsz T, Le Cesne A. Adjuvant chemotherapy for primary cardiac sarcomas: the IGR experience. Br J Cancer 1998;78(12):1624-8. Go to original source... Go to PubMed...
    26. Abu Saleh WK, Ramlawi B, Shapira OM, Al Jabbari O, Ravi V, Benjamin R, Durand JB, Leja MJ, Blackmon SH, Bruckner BA, Reardon MJ. Improved Outcomes With the Evolution of a Neoadjuvant Chemotherapy Approach to Right Heart Sarcoma. Ann Thorac Surg 2017;104(1):90-6. Go to original source... Go to PubMed...
    27. Randhawa JS, Budd GT, Randhawa M, Ahluwalia M, Jia X, Daw H, Spiro T, Haddad A. Primary Cardiac Sarcoma: 25-Year Cleveland Clinic Experience. Am J Clin Oncol 2016;39(6):593-99. Go to original source... Go to PubMed...
    28. Glaoui M, Benbrahim Z, Belbaraka R, Naciri S, Errihani H, Lescene A. An uncommon long-term survival case of primary cardiac leiomyosarcoma. World J Surg Oncol 2014;12:338. doi: 10.1186/1477-7819-12-338 Go to original source... Go to PubMed...
    29. Isambert N, Ray-Coquard I, Italiano A, Rios M, Kerbrat P, Gauthier M, Blouet A, Chaigneau L, Duffaud F, Piperno-Neumann S, Kurtz JE, Girard N, Collard O, Bompas E, Penel N, Bay JO, Guillemet C, Collin F, Blay JY, Le Cesne A, Thariat J. Primary cardiac sarcomas: a retrospective study of the French Sarcoma Group. Eur J Cancer 2014;50(1):128-36. Go to original source... Go to PubMed...
    30. Pins MR, Ferrell MA, Madsen JC, Piubello Q, Dickersin GR, Fletcher CD. Epithelioid and spindle-celled leiomyosarcoma of the heart. Report of 2 cases and review of the literature. Arch Pathol Lab Med 1999;123(9):782-88. Go to original source... Go to PubMed...
    31. Behi K, Ayadi M, Mezni E, Meddeb K, Mokrani A, Yahyaoui Y, Ksontini F, Rais H, Chrait N, Mezlini A. Two years survival of primary cardiac leiomyosarcoma managed by surgical and adjuvant therapy. Clin. Sarcoma Res 2017;7:5. doi: 10.1186/s13569-017-0069-3 Go to original source... Go to PubMed...
    32. Wang JG, Cui L, Jiang T, Li YJ, Wei ZM. Primary cardiac leiomyosarcoma: An analysis of clinical characteristics and outcome patterns. Asian Cardiovascular and Thoracic Annals 2015;23(5):623-30. Go to original source... Go to PubMed...

    This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International License (CC BY 4.0), which permits use, distribution, and reproduction in any medium, provided the original publication is properly cited. No use, distribution or reproduction is permitted which does not comply with these terms.


    Return to the content