Primary Mural Sarcoma of the Abdominal Aorta Presenting as Disabling Claudication
Eric W Murphy1, Thomas C Matthews2, William D Jordan3
1University of South Carolina School of Medicine, Columbia, SC;2Northside Vascular Surgery, Atlanta, GA;3Emory University School of Medicine, Atlanta, GA
INTRODUCTION:Primary malignant tumors of the aorta are exceptionally rare. Pre-operative diagnosis can be rather challenging as the initial presentation may present as aortitis, aneurysm or occlusive disease. Most confirmed cases are either diagnosed at autopsy or by histologic evaluation of thromboemboli. We report a case of mural sarcoma of the infrarenal abdominal aorta that presented with disabling claudication that was diagnosed after pathologic evaluation of the aortic specimen.
CASE REPORT:A 58 year old man presented to vascular surgery with bilateral lower extremity disabling claudication. He had a history of bilateral iliac stents and follow up iliac angioplasty performed at outside facilities. Other medical history was pertinent for testicular cancer treated with radiation 22 years earlier He presented to us with recurrent, disabling claudication at approximately 50 yards. He had an ankle brachial index of 0.5 bilaterally. CT angiography imaging revealed severe in-stent stenosis of the iliac stents and subtotal aortic occlusion. We performed an aortobifemoral bypass with a 16x8mm bifurcated dacron graft - constructed in an end-to-end fashion proximally and end-to-side fashion distally.
The procedure and post-operative course were well tolerated without complication. A small segment of the infrarenal aorta was resected to facilitate aortic reconstruction as part of the routine surgical technique. This aortic segment appeared normal with severe atherosclerotic disease. Generally, our standard practice is to send this segment for gross pathology only. However, in this particular case - the patient’s rheumatologist requested microscopic analysis of his aorta to evaluate for vasculitis. The histomorphology and immunoprofile of the specimen identified primary mural sarcoma of the aorta, which was confirmed after further review from 3 separate institutions’ pathology departments.
The patient’s claudication symptoms resolved completely post procedure. Postoperative ABIs were greater than 1 bilaterally. Metastatic disease of the bone was later diagnosed via a follow up PET scan and was confirmed via biopsy. Considering this patient's presentation as occlusive disease, the resected specimen and clinical concern provided an early diagnosis, that was only found to be metastatic after staging PET CT scan. He was successfully treated with proton therapy and immunotherapy. He is currently in PET negative remission over two years after his diagnosis.
DISCUSSION:
Primary aortic sarcoma is a highly unusual tumor with less than 200 reported cases since its initial discovery in 1873. With its presentation being similar to occlusive arterial disease, it is very difficult to detect prior to tissue diagnosis. This is a rare case of early detection after an aortobifemoral reconstruction for disabling claudication.
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