Background: Marfan syndrome (MS) most often shows as thoracic aortic aneurysm (TAA) or aortic dissection, but it may also involve other vascular territories. This study aimed to identify those extrathoracic vascular manifestations most frequently associated with MS.
Methods: A systematic review of the literature with Preferred Reporting Items for Systematic Reviews and Meta-Analyses criteria was carried out.
Neoplasias affecting the aorta are usually due to a variety of thoracic and abdominal tumors, which are more common than primary tumors of the aortic wall. Those tumors that can invade the abdominal aorta are usually sarcomas, which are able to mimic, both clinically and radiologically, an aortic disease such as an aneurysm or a dissection. There are few clinical scenarios where surgical resection and aortic repair needs to be performed, and indications have not still been clearly established in the literature.
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