Piechuta Aleksandra, Przybyłowski Tadeusz, Szołkowska Małgorzata, Krenke Rafał
Department of Internal Medicine, Pneumonology and Allergology, Medical University of Warsaw, Poland.
Pneumonol Alergol Pol. 2016;84(5):283-9. doi: 10.5603/PiAP.2016.0036.
Primary pulmonary angiosarcoma (PPA) is a rare tumour arising from arterial or venous pulmonary vessels of various size. It is characterized by aggressive course and poor prognosis. The early diagnosis is difficult due to diverse clinical and radiological manifestations. We present a case report of 70 year-old man, active cigarette-smoker, with a 2-month history of non-massive hemoptysis. The thorax CT revealed several solid pulmonary nodules surrounded by areas of ground glass opacity. As bronchoscopy failed to deliver adequate tissue samples, video assisted thoracic surgery (VATS) with pleura and lung biopsy was necessary. Histopathological findings were consistent with pulmonary angiosarcoma. Since no extrapulmonary lesions were demonstrated, the final diagnosis of primary pulmonary angiosarcoma was made. The patient died three months after the onset of symptoms. Our case report highlights that differential diagnosis in patients with hemoptysis and pulmonary nodules should include primary pulmonary sarcoma.
原发性肺血管肉瘤(PPA)是一种罕见的肿瘤,起源于不同大小的肺动静脉血管。其特点是病程进展迅速,预后较差。由于临床表现和影像学表现多样,早期诊断困难。我们报告一例70岁男性病例,该患者有长期吸烟史,有2个月的非大量咯血病史。胸部CT显示多个实性肺结节,周围有磨玻璃影。由于支气管镜检查未能获取足够的组织样本,因此需要进行电视辅助胸腔镜手术(VATS)并取胸膜和肺组织活检。组织病理学结果与肺血管肉瘤相符。由于未发现肺外病变,最终诊断为原发性肺血管肉瘤。患者在症状出现三个月后死亡。我们的病例报告强调,咯血和肺结节患者的鉴别诊断应包括原发性肺肉瘤。