Primary Ewing Sarcoma in the Right Ventricle in an Adult Patient: A Case Report
Abstract
Ewing sarcoma, a highly aggressive round-cell neoplasm of unknown origin, rarely occurs as a primary cardiac tumor. In this intriguing case, we describe an instance of primary Ewing sarcoma in an adult patient’s right ventricle (RV).
The patient, a 27-year-old man, presented with symptoms including epigastric pain, fever, tachycardia, nausea, vomiting, shortness of breath, and a dry cough. Imaging studies, including a computed tomography scan and echocardiogram, revealed an anterior mediastinal mass arising from the free wall of the RV and extending into the pericardium. The mass exerted pressure on the RV outflow tract and the pulmonary artery.
Surgical intervention was deemed necessary based on the clinical presentation and paraclinical findings. The mass was meticulously dissected from the cardiac tissue, and the RV outflow tract was resected, along with the pulmonary valve and the main pulmonary artery. These structures were replaced with a composite Dacron graft, anastomosed to the bifurcation of the main pulmonary artery. Additionally, the mass near the superior vena cava and the pulmonary vein region was carefully excised.
The patient was successfully weaned off the cardiopulmonary bypass pump in stable condition and transferred to the ICU. Histopathological analysis confirmed the diagnosis of small round cell sarcoma, specifically Ewing sarcoma. Following recovery, the patient was discharged and scheduled for regular follow-up appointments.
Following discharge, the patient was referred to a radiotherapy center and underwent radiotherapy. During a follow-up examination 1 year later, no signs of disease recurrence or progression were observed, offering a promising outlook. This case underscores the importance of timely referral and treatment for cardiac Ewing sarcoma, which may significantly improve prognosis.
2. Abboud A, Masrouha K, Saliba M, Haidar R, Saab R, Khoury N, et al. Extraskeletal Ewing sarcoma: Diagnosis, management and prognosis. Oncology letters 2021; 21: 1-6.
3. Iwamoto Y. Diagnosis and treatment of Ewing's sarcoma. Japanese journal of clinical oncology 2007; 37: 79-89.
4. Ushigusa J, Mukae Y, Takamatsu M, Nogami E, Furutachi A, Itoh M, et al. Adult-onset primary Ewing’s sarcoma of the right atrium: a case report. Surgical Case Reports 2019; 5: 1-4.
5. Angervall L, Enzinger F. Extraskeletal neoplasm resembling Ewing's sarcoma. Cancer 1975; 36: 240-51.
6. van den Berg H, Heinen RC, van der Pal HJ, Merks JH. Extra-osseous Ewing sarcoma. Pediatric Hematology and Oncology 2009; 26: 175-85.
7. Cotterill SJ, Ahrens S, Paulussen M, Jürgens HF, Voûte PA, Gadner H, et al. Prognostic factors in Ewing's tumor of bone: analysis of 975 patients from the European Intergroup Cooperative Ewing's Sarcoma Study Group. J Clin Oncol 2000; 18: 3108-14. doi: 10.1200/jco.2000.18.17.3108
8. Huh J, Noh CI, Kim YW, Choi JY, Yun YS, Shin HY, et al. Secondary cardiac tumor in children. Pediatr Cardiol 1999; 20: 400-3. doi: 10.1007/s002469900500
9. Applebaum MA, Worch J, Matthay KK, Goldsby R, Neuhaus J, West DC, et al. Clinical features and outcomes in patients with extraskeletal Ewing sarcoma. Cancer 2011; 117: 3027-32.
10. Petrovic M, Zhao B, Thangam M, Loyalka P, Buja LM, Kar B, et al. Ewing sarcoma in the right ventricle. Texas Heart Institute Journal 2016; 43: 458-60.
11. Dotson J, Urella M, Shenouda M, Abu-Hashyeh A, Lebowicz Y. Extraosseous Ewing sarcoma presenting with inferior ST-elevation myocardial infarction and systemic emboli due to tumor thrombus and invasion of the left atrium. Case Reports in Orthopedics 2020; 2020.
12. Kulkarni SK, Moorthy N, Somanna S, Cholenahally Nanjappa M. A rare case of Ewing's sarcoma/primitive neuroectodermal tumor metastasizing to left atrium through left inferior pulmonary vein. Echocardiography 2018; 35: 885-8. doi: 10.1111/echo.13897
13. Faim D, Francisco A, Pires A. Cardiac tumours in children: a single-centre experience and literature review. Cardiol Young 2022; 1-10. doi: 10.1017/s1047951122000531
14. Murad R, Bakkar R, Sukkar R, Mahmoud F, Al-Dairy A. A Case of Ewing Sarcoma Presenting With Cardiac Metastasis. World J Pediatr Congenit Heart Surg 2019; 10: 632-4. doi: 10.1177/2150135119846047
Files | ||
Issue | Vol 19 No S1 (2024): Supplementary 1 | |
Section | Case Report(s) | |
DOI | https://doi.org/10.18502/jthc.v19is1.18502 | |
Keywords | ||
Cardiac Ewing’s Sarcoma Heart Neoplasms Case report |
Rights and permissions | |
![]() |
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. |