CASE 528 Published on 24.10.2000
0

Sarcoma of the pulmonary artery

Section

Chest imaging

Case Type

Clinical Cases

Authors

M. Micallef, A.J.Collins, C.S.Elliott

Connected authors
Patient

38 years, female

Categories
No Area of Interest ; Imaging Technique CT, MR
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Clinical History
Recurrent right chest pain and dyspnea.
Imaging Findings
First admitted with cough and right sided chest pain worse on inspiration. Chest radiograph and perfusion scan are shown. Spiral CT showed a filling defect in the lower and middle lobe vessels. The clinical condition did not improve. Thrombolysis was attempted with no success. Six months from the first admission the patient was readmitted with dyspnea, chest pain and hemoptysis. On examination there was a right pleural effusion and a pulmonary outflow murmur. Chest radiograph, lung perfusion scan and CT angiogram (shown) demonstrated a mass filling the lumen of the right main pulamonary artery and no perfusion of the right lung. Embolectomy was peformed in a specialist institution.
Discussion
Primary pulmonary angiosarcoma is very rare with only some 200 cases reported. However angiosarcomas remain the most common primary tumours of the heart and great vessels. The majority arise from the right atrium or pulmonary vessels. The associated clinical symptoms are varied and non-specific. Infiltration of the pulmonary arteries can mimic pulmonary embolism as in our case. Early diagnosis is therefore difficult but improves survival. Approximately 80% of the tumours behave as aggressive intra-luminal neoplasms arising from the intimal cells . They are characterised histologically by staining with factor VIII related antigen. Despite the increasing availability of echocardiography , spiral CT and MR studies distinction from chronic pulmonary embolism remains difficult with 50% of diagnoses made at thoracotomy/cytology. Recent interest has focused on dynamic contrast enhanced MR to obtain 2D multiplanar and ultrafast 3D sequences with some success.
Differential Diagnosis List
Pulmonary angiosarcoma
Final Diagnosis
Pulmonary angiosarcoma
Case information
URL: https://www.eurorad.org/case/528
DOI: 10.1594/EURORAD/CASE.528
ISSN: 1563-4086
Figure 1

Chest Radiograph

Chest Radiograph
On first admission, chest radiograph showed blunting of the right costophrenic angle and reduced vascular markings of the right base (not well seen on digital image).
Chest Radiograph
On the second admission there was a raised right hemidiaphragm and a riht pleural effusion.
Figure 2

Perfusion lung scan

Perfusion lung scan
On first admission there was reduced perfusion in the lower and middle lobes.
Perfusion lung scan
On second admission there was absent perfusion of the entire right lung.
Figure 3

Spiral CT scan of the chest on second admission.

Spiral CT scan of the chest on second admission.
Iv contrast. Section at the aorto-pulmonary window.
Spiral CT scan of the chest on second admission.
Iv contrast.Large filling defect in the pulmonary outflow tract.
Spiral CT scan of the chest on second admission.
Iv contrast.Large filling defect in the pulmonary outflow tract.
Spiral CT scan of the chest on second admission.
Iv contrast.Large filling defect in the pulmonary outflow tract.
Spiral CT scan of the chest on second admission.
Iv contrast.Large filling defect in the pulmonary outflow tract.
Spiral CT scan of the chest on second admission.
Iv contrast.Section just caudal to the filling defect in the pulmonary outflow tract.
Spiral CT scan of the chest on second admission.
Iv contrast.Note the infarct in the right base which followed the obstruction of the pulmonary outflow tract.
Spiral CT scan of the chest on second admission.