Primary aortic intimal sarcoma of the endothelial cell type with long-term survival

Primary tumors of the aorta are rare and are difficult to diagnose preoperatively. These tumors are malignant and ultimately fatal. A patient had initial evidence of aortoiliac obstructive disease. Aortic endarterectomy produced an excellent postoperative clinical result until the pathology report f...

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Bibliographic Details
Published in:Journal of vascular surgery 1998-03, Vol.27 (3), p.555-558
Main Authors: Majeski, James, Crawford, E.Stanley, Majeski, Elizabeth I., Duttenhaver, John R.
Format: Article
Language:English
Subjects:
Aged
Aorta, Abdominal
Arterial Occlusive Diseases - diagnostic imaging
Arterial Occlusive Diseases - etiology
Arterial Occlusive Diseases - surgery
Biological and medical sciences
Blood and lymphatic vessels
Cardiology. Vascular system
Diagnostic Errors
Diseases of the aorta
Endarterectomy
Hemangioendothelioma - complications
Hemangioendothelioma - pathology
Hemangioendothelioma - surgery
Humans
Male
Medical sciences
Radiography
Sarcoma
Survival Analysis
Tunica Intima - pathology
Vascular Neoplasms - complications
Vascular Neoplasms - pathology
Vascular Neoplasms - surgery
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Summary:Primary tumors of the aorta are rare and are difficult to diagnose preoperatively. These tumors are malignant and ultimately fatal. A patient had initial evidence of aortoiliac obstructive disease. Aortic endarterectomy produced an excellent postoperative clinical result until the pathology report from the endarterectomy specimen revealed aortic sarcoma. The patient refused a second operation for en bloc aortic resection. One year later with recurrent symptoms, the patient underwent en bloc resection of the aorta and common iliac vessels until clear margins were obtained. The tumor was found to be intimal aortic sarcoma of endothelial cell origin. The only metastasis was to bone, and it was controlled with radiation and chemotherapy. The patient lived a total of 8 years from the time of initial diagnosis. Abdominal aortic intimal sarcoma of the endothelial cell type is a rare variety of aortic sarcoma that can be managed successfully if diagnosed early. This specific type of tumor has a longer survival period and better prognosis than other types of aortic sarcoma when managed with aggressive surgical resection, radiation, and chemotherapy. (J Vasc Surg 1998;27:555-8.)
ISSN:0741-5214
1097-6809
DOI:10.1016/S0741-5214(98)70333-7