Lu Ping, Yin Bei-Bei
Department of Cardiac Surgery, The First Affiliated Hospital of Shandong First Medical University, Jinan 250014, Shandong Province, China.
Department of Oncology, The First Affiliated Hospital of Shandong First Medical University, Jinan 250014, Shandong Province, China.
World J Clin Cases. 2020 Mar 6;8(5):986-994. doi: 10.12998/wjcc.v8.i5.986.
Primary intimal sarcoma of the pulmonary artery is a rare malignant tumor originating from the pulmonary artery, which has a low incidence rate and is easily misdiagnosed as pulmonary embolism. There is no standard protocol for the treatment of primary intimal sarcoma of the pulmonary artery.
This study reports a patient with primary intimal sarcoma of the pulmonary artery who was admitted to our hospital in 2017. The clinical characteristics, diagnosis, treatment and outcome of the patient were retrospectively analyzed. The patient was a Chinese Han male aged 44 years. He had three consecutive episodes of syncope, and was thus admitted to a local hospital. Computed tomography pulmonary angiography showed multiple lesions with abnormal densities in the pulmonary trunk, left pulmonary artery, mediastinum and pericardium, which were consistent with recurrence after tumor resection. He underwent surgery, and was pathologically diagnosed with intimal sarcoma of the pulmonary artery. He relapsed 3 mo after surgery, and apatinib was administered. His condition was stable after 4 mo, with tolerable and controllable adverse reactions. He subsequently died 19 mo after surgery.
Primary intimal sarcoma of the pulmonary artery has no specific clinical or imaging manifestations. The diagnosis of this disease depends on histopathology and immunohistochemistry, and has a poor clinical prognosis. Surgical treatment is currently a favorable option for primary intimal sarcoma of the pulmonary artery, and targeted therapy may provide new insights for the development of effective treatment methods.
肺动脉原发性内膜肉瘤是一种起源于肺动脉的罕见恶性肿瘤，发病率低，易被误诊为肺栓塞。目前尚无肺动脉原发性内膜肉瘤的标准治疗方案。
本研究报告了1例2017年入住我院的肺动脉原发性内膜肉瘤患者。对该患者的临床特征、诊断、治疗及转归进行回顾性分析。患者为44岁中国汉族男性。因连续3次晕厥入住当地医院。胸部CT血管造影显示肺动脉主干、左肺动脉、纵隔及心包多发密度异常病灶，符合肿瘤切除术后复发表现。患者接受手术治疗，术后病理诊断为肺动脉内膜肉瘤。术后3个月复发，给予阿帕替尼治疗。4个月后病情稳定，不良反应可耐受且可控。患者术后19个月死亡。
肺动脉原发性内膜肉瘤无特异性临床及影像学表现。该病的诊断依赖于组织病理学及免疫组化，临床预后较差。手术治疗目前是肺动脉原发性内膜肉瘤的首选治疗方式，靶向治疗可能为有效治疗方法的开发提供新的思路。
