A 35 year old gentleman presented to a medical centre with backache and palpitation for a month. He was referred to our centre for right atrium(RA) mass. He underwent open heart surgery via median sternotomy. The large tumour was raised from interatrial septum, extending into the IVC and the postero-inferior part of RA. The inferior vena cava (IVC) was fixed posteriorly due to the tumour invasion. Debulking surgery was performed. Histopathology and immunohistochemical study confirmed a malignant hemangioendothelioma. He has completed a palliative course of tomotherapy. He is treating with Tazemetostat, an inhibitor of EZH2 methyltransferase. He remains asymptomatic from cardiac aspect after 1 year of surgery.

1. Wu, X., Li, B., Zheng, C., & He, X. (2019). Clinical characteristics of epithelioid hemangioendothelioma: A single-center retrospective study. Medicine, 98(19), Article e15480. https://doi.org/10.1097/MD.0000000000015480
2. Ciliberti, M. P., Caponio, R., Pascali, A., & Lioce, M. (2015). A rare case of intravascular epithelioid hemangioendothelioma of the cephalic vein treated with surgery and postoperative radiation therapy: A case report and review of the literature. Journal of Medical Case Reports, 9, Article 81. https://doi.org/10.1186/s13256-015-0555-5
3. Bruckner, B. A., & Reardon, M. (2010). Benign cardiac tumors: A review. Methodist DeBakey Cardiovascular Journal, 6(3), 20–26. https://doi.org/10.14797/mdcj-6-3-20
4. Yin, K., Luo, R., Wei, Y., & Dobrilovic, N. (2020). Survival outcomes in patients with primary cardiac sarcoma in the United States. The Journal of Thoracic and Cardiovascular Surgery, 162(3), 879–887. https://doi.org/10.1016/j.jtcvs.2020.01.036
5. Patel, J., & Sheppard, M. N. (2009). Pathological study of primary cardiac and pericardial tumours in a specialist UK centre: Surgical and autopsy series. Cardiovascular Pathology, 18(6), 347–352. https://doi.org/10.1016/j.carpath.2008.09.001
6. Weiss, S. W., & Enzinger, F. M. (1982). Epithelioid hemangioendothelioma: A vascular tumor often mistaken for a carcinoma. Cancer, 50(5), 970–981. https://doi.org/10.1002/1097-0142(19820901)50:5<970::aid-cncr2820500527>3.0.co;2-z
7. Lisy, M., Beierlein, W., Müller, H., & Ziemer, G. (2007). Left atrial epithelioid hemangioendothelioma. The Journal of Thoracic and Cardiovascular Surgery, 133(4), 1105–1107. https://doi.org/10.1016/j.jtcvs.2006.11.030
8. Brčić, I., & Liegl-Atzwanger, B. (2021). Epithelioid hemangioendothelioma. PathologyOutlines.com. https://www.pathologyoutlines.com/topic/softtissuehemangioendotheliomaepithelioid.html
9. Kahlout, M., Al-Mulla, A., Chaikhouni, A., & Al-Bozom, I. (2009). Unusual presentation of a rare tumor: Cardiac epithelioid hemangioepithelioma presenting as cardiac tamponade. Heart Views, 10(3), 132–135.
10. Cottini, M., Lappa, A., & Donfrancesco, S. (2017). Primary cardiac hemangioendothelioma in the adult and cardiac involvement in patient with non-cardiac hemangioendothelioma: Review of the literature. Cancer Studies and Therapeutics Journal, 2(5), 1–6. https://doi.org/10.15133/j.cstj.2017.02.005