Khalili Ahmadali, Negargar Sohrab, Vaez Gharamaleki Jalil, Faravan Amir
Cardiovascular Research Center, Madani Heart Center, Tabriz University of Medical Sciences, Tabriz, Iran.
Department of Anesthesia, Faculty of Medicine, Tabriz University of Medical Sciences, Tabriz, Iran.
J Tehran Heart Cent. 2024;19(Suppl 1):56-59. doi: 10.18502/jthc.v19is1.18502.
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.
尤因肉瘤是一种起源不明的高度侵袭性圆形细胞肿瘤，很少作为原发性心脏肿瘤出现。在这个有趣的病例中，我们描述了一名成年患者右心室原发性尤因肉瘤的病例。患者为一名27岁男性，出现上腹部疼痛、发热、心动过速、恶心、呕吐、呼吸急促和干咳等症状。包括计算机断层扫描和超声心动图在内的影像学检查显示，前纵隔有一肿块，起源于右心室游离壁并延伸至心包。肿块对右心室流出道和肺动脉施加压力。根据临床表现和辅助检查结果，认为有必要进行手术干预。小心地从心脏组织中分离出肿块，并切除右心室流出道、肺动脉瓣和主肺动脉。这些结构用复合涤纶移植物替换，吻合到主肺动脉分叉处。此外，小心切除上腔静脉和肺静脉区域附近的肿块。患者在稳定状态下成功脱离体外循环泵，并转入重症监护病房。组织病理学分析证实为小圆细胞肉瘤，具体为尤因肉瘤。康复后，患者出院并安排定期随访。出院后，患者被转诊至放疗中心并接受放疗。1年后的随访检查中，未观察到疾病复发或进展的迹象，前景乐观。该病例强调了心脏尤因肉瘤及时转诊和治疗的重要性，这可能显著改善预后。
