原发性肺动脉内膜肉瘤1例报告并国内20例回顾性分析

目的 探讨原发性肺动脉内膜肉瘤的临床及组织病理学特点.方法 对1例原发性肺动脉内膜肉瘤的临床及病理学特点进行分析,并结合国内公开报道的20例及国外文献进行回顾性分析.结果 原发性肺动脉内膜肉瘤临床多表现为进行性呼吸困难、胸闷、咳嗽、胸痛、晕厥、痰血、发热及乏力等症状,影像特征是肺动脉不均匀性扩张和管腔内软组织充盈缺损.组织病理学多表现为未分化或低分化的恶性间叶细胞肿瘤,具有成纤维细胞或肌纤维母细胞分化特征,免疫组化见波形蛋白(vimentin)、α-平滑肌动蛋白(SMA)阳性而结蛋白(desmin)阴性.结论 原发性肺动脉内膜肉瘤缺乏特异性临床及影像学表现,确诊有赖于组织病理学,预后差.

Primary intimal sarcoma of the pulmonary artery 1 case report and retrospective analysis of 20 cases published in China

Objective To explore the clinical and histopathological features of primary intimal sarco- ma of the pulmonary artery. Methods One case of primary intimal sarcoma of the pulmonary artery diag- nosed in our hospital and 20 cases published openly in China were analysed retrospectively. Results The clinical presentation of primary intimal sarcoma of the pulmonary artery was progressive dyspnea, cough, chest pain, syncope, bloody phlegm, fever and fatigue. From the radiological finding, its signs were of pulmonary artery dilation, reduced pulmonary vasculature and cardiomegaly, soft tissue mass near the right ventricle outflow tract. Histological examination of intimal sarcoma of the pulmonary artery revealed a poorly differentiated mesenchymal tumor of fibroblastic or myofibroblastic differentiation, consisting of mildly atypical spindle cells with atypia, mitosis, and nuclear polymorphism. Immunohistochemical analy- sis showed positive staining with antibodies against vimentin, alpha-smooth muscle actin, while negative for desmin. Conclusion Primary intimal sarcoma of the pulmonary artery has atypical clinical and radio- logical manifestation with poor prognosis, its definite diagnosis depends on histopathology.