Aortic mural leiomyosarcoma with spinal involvement

Abstract Objectives There are few reports of aortic leiomyosarcoma arising from the mural region with extraluminal expansion. We experienced three cases of mural leiomyosarcoma, two originating from the thoracic aorta and one from the abdominal aorta, causing back pain with spinal involvement. The objectives of this study were to describe the clinical presentation and course of these three cases. Methods We retrospectively reviewed three consecutive patients with primary tumors of the aorta who underwent tumor resection and were anatomopathologically diagnosed with mural leiomyosarcoma at our institution between July 2012 and June 2015. Results Three male patients were aged from 47 to 61 years. For all patients, the chief complaint was back pain attributed to spinal involvement with direct invasion of the tumor. The extraluminal expansion was shown around the aorta, and pathological vertebral fractures and spinal canal stenosis were observed in all cases. For one patient, tumor resection and vertebral reconstruction were performed following aortic replacement through endovascular stenting. Another patient presented with progressive paraplegia, and cytoreductive surgery was performed. The remaining patient was diagnosed with a ruptured abdominal aortic aneurysm resulting in open surgery and the implantation of an aortic prosthesis. All patients were resistant to any adjuvant therapy and died between 4 and 19 months after surgery. Conclusions In all cases, spinal involvement was shown. Back pain is a considerable initial presentation of aortic mural leiomyosarcoma with extraluminal expansion. To our knowledge, this is the first report to demonstrate in minute detail the clinical course of mural leiomyosarcoma of the aorta with spinal involvement.