[en] Cardiac intimal sarcoma is extremely rare and aggressive primary malignant cardiac tumors. Here, we reported the case of a young man initially operated for a tumor of the left atrium, causing a dynamic obstruction of the mitral valve and (mis-)diagnosed as a myxoma at the histopathological analysis. Patient presented a local recurrence at 3 months and was reoperated. Pathology revealed this time the presence of an intimal sarcoma. Patient received adjuvant chemotherapy. Despite a good local control, the 1-year follow-up positron emission tomography scan revealed the presence of a metastasis in the left adrenal gland that was surgically resected. This article aims to highlight the risk of misdiagnosis in case of cardiac tumors, the hypothetical concept of malignant transformation of a cardiac myxoma, the aggressive course of the extremely rare cardiac intimal sarcoma, and the therapeutic modalities available to treat this pathology.
Thuraisingam A, Fletcher N, Mckay G. Primary cardiac intimal sarcoma. Heart, Lung Circ. 2019;28:S104.
Valecha G, Pau D, Nalluri N, Liu Y, Mohammad F, Atallah JP. Primary intimal sarcoma of the left atrium: an incidental finding on routine echocardiography. Rare Tumors. 2016;8(4):6389. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5136772/
Mir IA, Ahangar AG. Atrial myxoma: a review. Int J Commun Med Public Health. 2017;3(1):23-29.
Vito AD, Mignogna C, Donato G. The mysterious pathways of cardiac myxomas: a review of histogenesis, pathogenesis and pathology. Histopathology. 2015;66(3):321-332.
Amano J, Kono T, Wada Y, et al. Cardiac myxoma: its origin and tumor characteristics. Ann Thorac Cardiovasc Surg. 2003;9(4):215-221.
Hudzik B, Miszalski-Jamka K, Glowacki J, et al. Malignant tumors of the heart. Cancer Epidemiol. 2015;39(5):665-672.
Kasugai T, Sakurai M, Yutani C, et al. Sequential malignant transformation of cardiac myxoma. Acta Pathol Jpn. 1990;40(9):687-692.
Nguyen A, Awad WI. Cardiac sarcoma arising from malignant transformation of a preexisting atrial myxoma. Ann Thorac Surg. 2016;101(4):1571-1573.
Bin Wang, He Li, Yuji Xie, et al. Undifferentiated cardiac sarcomas originating from malignant transformation of atrial myxoma. Circulation: Cardiovascular Imaging. 2020;13(5):e010277.
Lian D, Zhiyun X, Zhigang L. Relapse of a cardiac tumor from myxoma to low-grade malignant myofibroblastic sarcoma: malignant transformation or metachronous lesion? J Med Coll PLA. 2010;25(1):50-53.
Kaynak K, Beşirli K, Arslan C, Ozgüroğlu M, Oz B. Metastatic cardiac myxoma. Ann Thorac Surg. 2001;72(2):623-625.
Kumary VS, Madhavan S, Akhil PC, Jayaprakash K, George R. Two-time recurrence of a right atrial myxoma. Proc (Bayl Univ Med Cent). 2015;28(4):507-508.
Wang Z, Chen S, Zhu M, et al. Risk prediction for emboli and recurrence of primary cardiac myxomas after resection. J Cardiothorac Surg. 2016;11(1):22.
Mendoza C, Bernstein E, Ferreira A. Multiple Recurrences of Nonfamilial Cardiac Myxomas. Tex Heart Inst J. 2007;34(2):236-239.
Shah IK, Dearani JA, Daly RC, et al. Cardiac myxomas: a 50-year experience with resection and analysis of risk factors for recurrence. Ann Thorac Surg. 2015;100(2):495-500.
Ogechukwu C-N, Christopher N, Christoph S, Etinosasere O, Jose B. The insidious cardiac tumor: a primary left atrium intimal cardiac sarcoma in a young patient. Case Rep Cardiol. 2019;2019(1):e7245676. https://www.hindawi.com/journals/cric/2019/7245676/
Zhang PJ, Brooks JS, Goldblum JR, et al. 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):1385-1395.
Marques Mendes E, Ferreira A, Felgueiras P, et al. Primary intimal sarcoma of the left atrium presenting with constitutional symptoms. Oxf Med Case Rep. 2017;2017(7):omx031. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5497514/
Nakamura K, Nakamura T, Iino T, et al. Expression of interleukin-6 and the interleukin-6 receptor predicts the clinical outcomes of patients with soft tissue sarcomas. Cancers (Basel). 2020;12(3):585. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7139480/
Deguchi H, Sato B, Ohshima M, et al. A case of interleukin-6-producing malignant fibrous histiocytoma originating in the heart. Jpn J Clin Oncol. 1999;29(8):382-386.
Burke AP, Cowan D, Virmani R. Primary sarcomas of the heart. Cancer. 1992;69(2):387-395.
Sarcoma Meta-analysis Collaboration. Adjuvant chemotherapy for localised resectable soft-tissue sarcoma of adults: meta-analysis of individual data. Lancet. 1997;350(9092):1647-1654.
Li H, Yang S, Chen H, et al. Survival after heart transplantation for non-metastatic primary cardiac sarcoma. J Cardiothorac Surg. 2016;11(1):145.
Mansencal N, Revault-d'Allonnes L, Pelage J-P, Farcot J-C, Lacombe P, Dubourg O. Usefulness of contrast echocardiography for assessment of intracardiac masses. Arch Cardiovasc Dis. 2009;102(3):177-183.
Motwani M, Kidambi A, Herzog BA, Uddin A, Greenwood JP, Plein S. MR Imaging of cardiac tumors and masses: a review of methods and clinical applications. Radiology. 2013;268(1):26-43.
Meng J, Zhao H, Liu Y, et al. Assessment of cardiac tumors by 18F-FDG PET/CT imaging: Histological correlation and clinical outcomes [published online ahead of print January 13, 2020]. J Nucl Cardiol. 2020. https://doi.org/10.1007/s12350-019-02022-1
Cohen R, Singh G, Mena D, Garcia CA, Loarte P, Mirrer B. Atrial myxoma: a case presentation and review. Cardiol Res. 2012;3(1):41-44.
Kusumi T, Minakawa M, Fukui K, et al. Cardiac tumor comprising two components including typical myxoma and atypical hypercellularity suggesting a malignant change. Cardiovasc Pathol. 2009;18(6):369-374.
Leduc C, Maleszewski JJ. Misconceptions about malignant metamorphosis in myxoma. Ann Thorac Surg. 2016;102(5):1766.
