primary pulmonary angiosarcoma ( ppa ) is a rare neoplasm originating from endothelial cells of small vessels of the lung . the highest incidence of the disease occurs in the fifth and sixth decade of life . chest computed tomography ( ct ) demonstrates solitary or multifocal lesions occasionally accompanied by ground - glass opacities or pleural effusion [ 24 ] . diffuse alveolar haemorrhage with concomitant haemothorax is an extremely rare manifestation of primary angiosarcoma of the lung . the diagnosis is based on histological findings complemented by immunohistochemical stainings typical for endothelial markers . we present a case of rapid progression of ppa manifested as bilateral pulmonary opacities and haemothorax . a 65-year - old smoking woman presented a four - month history of haemoptysis , cough , and dyspnoea . the patient 's medical history was significant for chronic obstructive pulmonary disease ( copd ) , permanent atrial fibrillation ( af ) under continuous anticoagulation treatment , hypertension , and diabetes . due to severe haemoptysis , she reduced the dose of anticoagulant by 50% without medical consultation . chest ct performed one month prior to hospitalisation revealed bilateral interstitial opacities with air bronchogram and ground - glass attenuations . the radiological findings suggested interstitial lung disease , and the patient was referred to our department . on admission physical examination showed obesity ( bmi 47.5 ) , tachypnoea ( 30 per minute ) , tachycardia ( 105 per minute ) , and oedema of the ankles . on auscultation , the respiratory sounds over the lower right lobe were diminished , and crackles over the middle right lobe and the whole left lung were detected . the patient had respiratory insufficiency ( pao2  50 mmhg , paco2  33 mmhg , ph 7.48 ) . laboratory tests revealed an elevated level of c - reactive protein ( 26.5 mg / dl ) , d - dimer ( 3991 ng / ml ) and anaemia ( haemoglobin 9 g / dl , haematocrit 29.7% ) . chest x - ray demonstrated right - sided opacity corresponding to pleural fluid , scattered pulmonary lesions , and moderately enlarged hila ( fig . 1 ) . computed tomography scans of the chest showed progression of bilateral ground - glass opacities of different shape and size with peripheral predilection , small nodules ( including one peripheral left lung nodule with slight cavitation ) , right - sided pleural effusion , and enlargement of the heart ( fig . 2 and 3 ] . there were no radiological signs of pulmonary embolism in large branches of pulmonary arteries , but a suggestion that small vessels could have been filled with thrombotic material was made . echocardiography showed enlargement of the right ventricle and both atria , and elevated pulmonary artery pressure at 42 mmhg . the values of tumour markers ( cancer antigen ca 15 - 3 , ca 19 - 9 , ca 125 , carcinoembryonic antigen ) , anti - neutrophil cytoplasmic antibody ( anca ) , and anti - nuclear antibody ( ana ) were in normal range . bronchoscopy revealed a bronchi field with a significant amount of fresh blood without any visible point of origin . the haematocrit of the effusion was 19.8% and it was higher than 50% of the haematocrit of peripheral blood , which met the criteria of true haemothorax . on suspicion of immunologically - induced intra - alveolar haemorrhage , 80 mg of prednisone was administrated . after a brief stabilisation , intra - operative biopsy for frozen section examination did not reveal any neoplasmatic cells . during the next two days after the surgery chest radiograph shows right - sided opacity corresponding to pleural fluid ; diffuse pulmonary lesions focally confluent , forming bigger opacities within left lung ; hila moderately enlarged computed tomography scan shows a large right pleural effusion high - resolution computed tomography ( hrct ) scans : multiple ground - glass opacities , predominantly subpleural ; some small nodules bilaterally microscopic examination of pleural samples revealed that the areas of slit spaces were filled with erythrocytes and surrounded by epithelioid cells . immunohistochemical reactions showed diffuse reactivity with anti - cd31 ( lc70 , cell marque ) antibody ( fig . 4 ) and focal anti - cd34 ( qbend/10 , cell marque ) staining . reactions with cytokeratins ( ae1ae3 , roche ) and calretinin ( sp65 , roche ) were negative . in the lung specimen , cohesive and the lesions were composed of groups of loosely packed epithelioid cells mixed with fibrin and erythrocytes . adjacent alveolar spaces were filled with red blood cells and haemosiderin - laden macrophages ( fig . epithelioid cells revealed diffuse reactivity with anti - cd31 antibody ( cd31 , magnification 200 ) 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 ) typically , angiosarcomas occur as skin and subcutaneous tissue neoplasms of the head , neck , and lower and upper extremities , or involve the heart and extrathoracic organs [ 3 , 4 ] some reports suggest a correlation between radiotherapy , chemotherapy , past surgical interventions , environmental carcinogens , or chronic tuberculosis pyothorax [ 1 , 3 , 6 , 7 ] . primary pulmonary angiosarcoma patients complain of haemoptysis , dyspnoea , cough , weight loss , fatigue , pleuritic chest pain , and sometimes fever [ 3 , 8 ] . nevertheless , due to nonspecific complaints , continuous anticoagulant therapy , history of heart disease , misleading radiological features , and rapid progression , the diagnosis was made in a very advanced and metastatic stadium . in the presented case the haematocrit of the fluid was higher than 50% of the haematocrit of peripheral blood , which met the criteria of true haemothorax . there are no differences between ppa and metastatic angiosarcoma of the lung in radiological manifestation . in both cases ct demonstrates solitary or multifocal lesions , sometimes associated with ground - glass opacities or pleural effusion . usually at the time of diagnosis , the neoplasm presents extensive local and metastatic invasion [ 14 ] . images obtained on admission revealed bilateral pulmonary opacities with ground - glass attenuations , a small number of nodules , and pleural effusion . the histological examination of epithelioid angiosarcomas reveals single or multifocal tumours composed of sheets of atypical epithelioid cells , focal vasoformative areas , and necrosis . haematoxylin - eosin stained sections show irregularly anastomosing vessels , cells with nuclear atypia , and often a high mitotic count [ 4 , 6 ] . factor viii , cd 31 , cd34 , fli-1 , ulex europaeus agglutinin 1 , and vimentin are typical endothelial markers [ 4 , 7 , 8 ] . the biopsy specimen of our patient showed positive reaction for cd 31 and cd 34 . the other stains , including cytokeratin ( ae1/ae3 ) for epithelial and calretinin for mesothelial proliferations , were negative . it is suggested that at least two markers should be present in order to confirm the endothelial origin . there have been reports summarising poor prognostic factors for angiosarcomas , such as age ( > 70 years ) , tumour size ( > 5 cm ) , metastases at presentation , grade , and mitotic count , but they are still under debate [ 7 , 10 ] . the presented case is a rare manifestation of primary pulmonary angiosarcoma with diffuse pulmonary haemorrhage and haemothorax . non - distinctive clinical and radiological symptoms and a rapidly progressive course make this type of neoplasm a considerable challenge for clinicians . our case is a reminder of the necessity to include ppa in differential diagnosis of diffuse alveolar haemorrhage and haemothorax .
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 .