Aortic mural leiomyosarcoma with spinal involvement

Abstract

There are few reports of aortic leiomyosarcoma arising from the mural region with extraluminal expansion. We experienced 3 cases of mural leiomyosarcoma, 2 originating from the thoracic aorta and 1 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 3 cases. We retrospectively reviewed 3 consecutive patients with primary tumors of the aorta who underwent tumor resection and were anatomopathologically diagnosed with mural leiomyosarcoma at Niigata University Medical and Dental Hospital between July 2012 and June 2015. Three male patients were aged from 47 to 61years. 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 after 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 19months after surgery. 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 show in minute detail the clinical course of mural leiomyosarcoma of the aorta with spinal involvement.