Xu Rui, Zhao Yixuan, Xu Xiaosen, Liu Shuang, Hu Chenyu, Lv Dongmei, Wu Huiying
Department of Ultrasonography, Second Hospital of Jilin University.
Department of Ultrasonography, Changchun Traditional Chinese, Medicine Hospital, Changchun, Jilin, China.
Medicine (Baltimore). 2020 Jan;99(3):e18813. doi: 10.1097/MD.0000000000018813.
Pulmonary artery intimal sarcoma (PAIS) is a rare and highly aggressive tumor, and approximately 80% of pulmonary cases occur in the pulmonary trunk. We report herein a case of retrograde extension of the sarcoma to the pulmonary valve and right ventricle, which is an uncommon manifestation of this lethal tumor.
A 41-year-old woman was initially diagnosed with pulmonary thromboembolism (PTE) and transferred to our hospital.
Computed tomographic pulmonary angiography (CTPA) showed that there are low-density filling defects in both pulmonary arteries, and the patient was diagnosed with PTE. However, the ultrasonographers considered that the lesion is a space-occupying type that involves the right ventricular outflow tract and pulmonary valve instead of PTE. Postoperative pathology confirmed the diagnosis of PAIS.
The patient underwent resection of pulmonary artery sarcoma and endarterectomy.
During the follow-up via telephone 1 month after discharge, the patient reported to have been feeling well.
Owing to the rarity of the disease and its non-specific clinical manifestations, approximately half of the PAIS cases are misdiagnosed or have a delayed diagnosis. Thus, improving our understanding of the disease and facilitating its early diagnosis are essential.
肺动脉内膜肉瘤（PAIS）是一种罕见且侵袭性很强的肿瘤，约80%的肺部病例发生在肺动脉主干。我们在此报告一例肉瘤逆行延伸至肺动脉瓣和右心室的病例，这是这种致命肿瘤的一种罕见表现。
一名41岁女性最初被诊断为肺血栓栓塞症（PTE）并转入我院。
计算机断层扫描肺动脉造影（CTPA）显示双肺动脉有低密度充盈缺损，患者被诊断为PTE。然而，超声检查人员认为该病变是一种占位性病变，累及右心室流出道和肺动脉瓣，而非PTE。术后病理证实为PAIS。
患者接受了肺动脉肉瘤切除术和动脉内膜切除术。
出院后1个月电话随访时，患者报告感觉良好。
由于该疾病罕见且临床表现不具特异性，约一半的PAIS病例被误诊或诊断延迟。因此，提高对该疾病的认识并促进其早期诊断至关重要。
