Wu Song, Chen Qiulin, Yang Yi, Liu Jialiang, Li Yuankun, Cheng Shengjun, Wu Yutian
Department of Cardiothoracic Surgery, Department of Traditional Chinese Medicine, Chengdu Fifth People's Hospital, Chengdu, China.
Front Cardiovasc Med. 2025 Jun 10;12:1572673. doi: 10.3389/fcvm.2025.1572673. eCollection 2025.
Leiomyosarcoma is frequently found in the retroperitoneum, mesentery, omentum, uterus, or subcutaneous tissue. However, primary cardiac leiomyosarcoma is rare and even more uncommon is its coexistence with a benign tumor. We report a case involving a 61-year-old female with a right ventricular outflow tract leiomyosarcoma in conjunction with a benign mass located in the main pulmonary artery. Echocardiography revealed a 2.5 × 2.2 cm isoechoic mass in the right ventricular outflow tract and a 3.8 × 1.8 cm irregular isoechoic mass in the main pulmonary artery. Computed tomography angiography (CTA) indicated a patchy filling defect in a similar location. Initially, pulmonary embolism was considered, however, the possibility of tumors could not be excluded. Given the high risk of mass embolization, we proceeded with emergency surgery. A large, irregular, solid mass was found attached to the wall of the main pulmonary artery, fortunately without involvement of the pulmonary valve. Exploration of the right ventricular outflow tract uncovered an additional solid, smooth, well-encapsulated mass. Immunohistochemical analysis of the right ventricular outflow tract mass confirmed the presence of tumor cells that were positive for Desmin and smooth muscle actin (SMA), while negative for S-100 and myoglobin, leading to a diagnosis of leiomyosarcoma. For the pulmonary mass, Immunohistochemistry revealed the proliferation of fibrous tissue, mucus degeneration, and calcification within the focal area. The imaging characteristics of cardiac leiomyosarcoma combined with benign pulmonary artery tumors may be misinterpreted as thrombosis, however, surgical resection remains a viable treatment option.
平滑肌肉瘤常见于腹膜后、肠系膜、大网膜、子宫或皮下组织。然而,原发性心脏平滑肌肉瘤罕见,其与良性肿瘤并存则更为少见。我们报告一例61岁女性病例,其右心室流出道存在平滑肌肉瘤,同时主肺动脉内有一个良性肿块。超声心动图显示右心室流出道有一个2.5×2.2厘米的等回声团块,主肺动脉内有一个3.8×1.8厘米的不规则等回声团块。计算机断层血管造影(CTA)显示在类似位置有斑片状充盈缺损。最初考虑为肺栓塞,但不能排除肿瘤的可能性。鉴于肿块栓塞风险高,我们进行了急诊手术。发现一个大的、不规则的实性肿块附着在主肺动脉壁上,幸运的是未累及肺动脉瓣。对右心室流出道进行探查时发现另一个实性、表面光滑、包膜完整的肿块。对右心室流出道肿块进行免疫组织化学分析,证实肿瘤细胞结蛋白和平滑肌肌动蛋白(SMA)呈阳性,而S-100和肌红蛋白呈阴性,诊断为平滑肌肉瘤。对于肺部肿块,免疫组织化学显示局部区域有纤维组织增生、黏液变性和钙化。心脏平滑肌肉瘤合并良性肺动脉肿瘤的影像学特征可能被误诊为血栓形成,然而,手术切除仍是一种可行的治疗选择。