Pérez del Río M J, Molina Suárez R, Fresno Forcelledo M F, Veiga González M, Madrigal Rubiales B, González González M, Herrero Zapatero A
Servicio de Anatomía Patológica, Hospital Nuestra Señora de Covadonga, Universidad de Oviedo.
Rev Esp Cardiol. 1998 Oct;51(10):850-2. doi: 10.1016/s0300-8932(98)74831-6.
Pulmonary artery intimal sarcomas tend to be presented with symptoms of pulmonary thromboembolism and grow regionally, with little capacity to metastasize. They probably originate from subendothelial cells, that become myofibroblasts. Knowledge of it is important to establish a presurgery diagnosis, with the possibility of a total resection, the only useful treatment until now. We report a case of a pulmonary artery primary sarcoma, in a 73 year old woman, admitted with hemoptysis and pleuritic chest pain, who died ten days after. Autopsy revealed an intraluminal mass at the pulmonary artery trunk, without regional nor distance involvement. Microscopic study showed a pleomorphic tumor with spindle and epithelioid cells, positive for actin, desmin and vimentin. All these data support the diagnosis of primary intimal sarcoma of the pulmonary artery. We want to emphasize the myogenic differentiation of the tumor, uncommon in previously reported cases.
肺动脉内膜肉瘤往往表现为肺血栓栓塞症状，呈局部生长，转移能力较弱。它们可能起源于成为肌成纤维细胞的内皮下细胞。了解这一点对于术前诊断很重要，因为有可能进行根治性切除，这是目前唯一有效的治疗方法。我们报告一例73岁女性的肺动脉原发性肉瘤病例，该患者因咯血和胸膜炎性胸痛入院，十天后死亡。尸检显示肺动脉主干腔内有肿块，无局部及远处受累。显微镜检查显示肿瘤具有多形性，含梭形细胞和上皮样细胞，肌动蛋白、结蛋白和波形蛋白呈阳性。所有这些数据支持肺动脉原发性内膜肉瘤的诊断。我们想强调该肿瘤的肌源性分化在既往报道病例中并不常见。
