Familiarize the radiologist with the clinical and imaging characteristics of pulmonary artery sarcomas,
in order to place the correct differential diagnosis with acute and chronic pulmonary embolism,
in which this rare malignancy is most commonly confused.
Pulmonary artery sarcomas (PAS) are rare malignant tumor,
which originate in most cases by the totipotent intimal stem cells of the pulmonary trunk,
and for this reason is referred to as "intimal sarcoma"; in the literature about 250 cases have been described [1,2].
The intimal PASs usually are malignant mesenchymal tumors,
with fibroblastic or miofibroblastic differentiation.
In some cases may present specific histological patterns and be classified in osteosarcomas,
rhabdomyosarcomas and angiosarcomas (most frequently).
The pulmonary artery leiomyosarcomas are instead...
Imaging findings OR Procedure details
Chest X-Ray is performed as the first level investigation,
but it result always nonspecific.
May be normal or more frequently can show an acute pulmonary embolism-like pattern,
with increase of pulmonary arteries diameter,
regional oligemia and parenchymal opacities suggestive of pulmonary infarcts (Figure 1a) .The main role of chest X-ray is to exclude other diseases that may present with similar symptoms and to make suggestions for the next work-up.
Scintigraphy is used in the diagnostic work-up of suspected pulmonary embolism,
it detects the consequences of...
PAS is a highly aggressive and rapidly evolving disease,
underdiagnosed and misunderstood.
Early diagnosis with the help of integrated imaging remains today the main road to pursue in order to obtain improvements in prognosis.
Angio-CT images in the presence of a filling defect with contrast enhancement in the pulmonary artery should put the suspicion of malignancy and the hypothesis should be confirmed by a high uptake on CT-PET with FDG or contrast MRI hyperintensity.
The presence of tumor extraluminal extension as well as distant metastases...
Reardon MJ (2010) Pulmonary artery sarcoma.
Methodist Debakey Cardiovasc J 6:38-43.
Gladish G et al (2005) Pulmonary artery angiosarcoma: a clinicopathologic and radiological correlation.
Ann Diagn Pathol 9:209-214.
Virmani R (1993) Sarcomas of the great vessels.
A clinicopathologic study.
Aquino SL et al (1997) Pulmonary artery sarcomas: a review of clinical and radiologic features.
J Comput Assist Tomogr 21:750-755.
Mendelson DS (2000) Pulmonary artery...
Domenico Attinà MD
Cardio-Thoracic Radiology Unit
Via Massarenti 9,