Department of Cardiovascular Surgery, The Second Affiliated Hospital of Zhejiang University School of Medicine, No. 88 Jiefang Road, Hangzhou, China.
J Cardiothorac Surg. 2021 Mar 19;16(1):27. doi: 10.1186/s13019-021-01404-2.
Spindle cell sarcomas of the right atrium are extremely rare primary cardiac tumours, with very few cases reported in the medical literature. Pseudoaneurysms caused by cardiac spindle cell sarcoma have never been reported worldwide.
A 32-year-old woman was referred to our hospital for recurrent pericardial haemorrhagic effusion and pleural effusion. Three-dimensional transthoracic echocardiogram, contrast chest CT, and contrast MRI revealed a pseudoaneurysm on the right side of the right atrium with a thrombus. There was a defect between the pseudoaneurysm and the right atrium. PET-CT suggested that FDG metabolism inhomogeneity increased in the mass in the right atrium. Exfoliative cytology detection of massive pericardial effusion and pleural effusion revealed no tumour cells. Spindle cell sarcoma of the right atrium was not confirmed until the patient underwent right thoracic exploration and biopsy. Before a confirmed diagnosis, symptomatic treatment, such as chest effusion and pericardium effusion drainage, and transfusion of red blood cells were mainly used to relieve the patient's symptoms. Unfortunately, the patient was lost to optimal treatments and passed away 20 days after the pathological diagnosis was made.
The prognosis of spindle cell sarcomas remains poor due to delays in diagnosis, early metastasis and few available therapeutic options. Recurrent pericardial effusion and pleural effusion, especially in the nature of haemorrhagic effusion, and/or right atrial pseudoaneurysm shown on the transthoracic echocardiogram must be considered and highly suspected as malignancy by patients and physicians. If the diagnosis cannot be confirmed, histopathology should be performed as soon as possible to avoid losing the best treatment opportunity.
右心房梭形细胞肉瘤是极为罕见的原发性心脏肿瘤，在医学文献中仅有少数病例报道。由心脏梭形细胞肉瘤引起的假性动脉瘤在全球范围内从未有过报道。
一名 32 岁女性因反复发作性心包积血和胸腔积液被转至我院。三维经胸超声心动图、对比胸部 CT 和对比 MRI 显示右心房右侧有一个假性动脉瘤伴血栓形成。假性动脉瘤与右心房之间存在一个缺损。PET-CT 提示右心房内肿块 FDG 代谢不均匀增加。大量心包积液和胸腔积液的脱落细胞学检测未发现肿瘤细胞。直到患者接受右胸探查和活检，才确诊为右心房梭形细胞肉瘤。在明确诊断之前，主要采用对症治疗，如胸腔积液和心包积血引流以及输血，以缓解患者的症状。不幸的是，由于诊断延误、早期转移和治疗选择有限，该患者失去了最佳治疗机会，在病理诊断后 20 天去世。
由于诊断延误、早期转移和治疗选择有限，梭形细胞肉瘤的预后仍然较差。反复发作的心包积液和胸腔积液，尤其是血性积液的性质，以及（或）经胸超声心动图显示的右心房假性动脉瘤，必须引起患者和医生的重视和高度怀疑为恶性肿瘤。如果无法确诊，应尽快进行组织病理学检查，以免错失最佳治疗机会。
