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肺血管肉瘤

Angiosarcoma in the lung.

作者信息

Patel A M, Ryu J H

机构信息

Mayo Clinic, Division of Thoracic Diseases and Internal Medicine, Rochester, Minn 55905.

出版信息

Chest. 1993 May;103(5):1531-5. doi: 10.1378/chest.103.5.1531.

Abstract

Angiosarcoma involving the lung is a rare disorder and its clinical features are not well known. We conducted a retrospective analysis of 15 patients seen at our institution from 1950 to 1990 in an attempt to better characterize the spectrum of clinical and radiographic findings of angiosarcoma in the lung. No documented case of primary angiosarcoma of the lung was seen. The diagnosis of metastatic angiosarcoma to the lung was made antemortem in 12 of 15 cases, either by lung biopsy specimen (5 patients), biopsy evidence of metastatic disease elsewhere with abnormal chest radiograph (4 patients), or a compatible clinical picture in a patient with previously documented angiosarcoma arising in an extrapulmonary site (3 patients). The median age at the time of diagnosis was 45 years with the most common presenting symptom being hemoptysis (7 of 15 patients). Other presenting complaints included weight loss (6 of 15), cough (4 of 15), and chest pain (4 of 15) occurring 6 weeks to 1 year prior to diagnosis. Chest radiographs frequently disclosed multiple pulmonary nodules (11 of 15). Primary origins of the angiosarcoma most commonly included the heart and breast. Metastatic sites other than the lung included the pericardium, liver, spleen, kidney, adrenal gland, bone, and brain. The prognosis is generally poor, with our study population surviving an average of 9 months after diagnosis.

摘要

累及肺部的血管肉瘤是一种罕见疾病,其临床特征尚不为人熟知。我们对1950年至1990年间在我院就诊的15例患者进行了回顾性分析,旨在更好地描述肺部血管肉瘤的临床和影像学表现范围。未发现有记录的原发性肺血管肉瘤病例。15例中有12例在生前诊断为肺转移性血管肉瘤,其中5例通过肺活检标本确诊,4例通过其他部位转移性疾病的活检证据及胸部X线异常确诊,3例通过有肺外部位先前记录的血管肉瘤且临床表现相符确诊。诊断时的中位年龄为45岁,最常见的症状是咯血(15例中有7例)。其他就诊主诉包括体重减轻(15例中有6例)、咳嗽(15例中有4例)和胸痛(15例中有4例),这些症状在诊断前6周至1年出现。胸部X线片常显示多个肺结节(15例中有11例)。血管肉瘤的原发部位最常见的包括心脏和乳腺。除肺部外的转移部位包括心包、肝脏、脾脏、肾脏、肾上腺、骨骼和大脑。预后通常较差,我们研究中的患者诊断后平均存活9个月。

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