Diffuse pulmonary haemorrhage accompanied by haemothorax as a rare presentation of primary lung angiosarcoma

Abstract

Primary pulmonary angiosarcoma is an extremely rare disease. Chest computed tomography demonstrates solitary or multifocal lesions, sometimes associated with ground-glass opacities or pleural effusion. Diagnosis is based on histological examination that reveals spindle-shaped epithelioid cells with positive staining for endothelial markers (factor VIII, CD 31, CD34, Fli-1, Ulex europaeus agglutinin 1, vimentin). The prognosis is poor and effective treatment is still being researched. This is a report of a 65-year-old patient with a four-month history of haemoptysis, cough, and dyspnoea. The primary radiological findings suggested interstitial lung disease. After one month the clinical presentation evolved into diffuse pulmonary haemorrhage with concomitant haemothorax. The diagnosis of primary lung angiosarcoma was based on histological and immunohistochemical examination of the lung and pleural biopsy obtained by videothoracoscopy.

Keywords: angiosarcoma; diffuse pulmonary haemorrhage; haemothorax.

Fig. 1

Chest radiograph shows right-sided opacity corresponding to pleural fluid; diffuse pulmonary lesions focally confluent, forming bigger opacities within left lung; hila moderately enlarged

Fig. 2

Computed tomography scan shows a large right pleural effusion

Fig. 3

High-resolution computed tomography (HRCT) scans: multiple ground-glass opacities, predominantly subpleural; some small nodules bilaterally

Fig. 4

Epithelioid angiosarcoma, pleural lesion. Epithelioid cells revealed diffuse reactivity with anti-CD31 antibody (CD31, magnification 200×)

Fig. 5

Epithelioid angiosarcoma. Low- and medium-power magnification of a pulmonary lesion composed of epithelioid cells, haemorrhages, and fibrin deposits surrounded by hyperaemic lung parenchyma with aggregations of haemosiderin-laden macrophages (HE staining, magnification 200×)