Unveiling Aortic Angiosarcoma: A Rare and Aggressive Vascular Malignancy in Vascular Oncology - A Case Report

Abstract

Introduction: Primary aortic malignancy is a very rare pathology, with only 190 cases of aortic tumors reported in the literature to date. Usually, symptoms are insidious and nonspecific, so the disease is diagnosed at an advanced stage. Objective: Here, we present a case report of a primary malignant tumor of the aorta. In our case, the diagnosis was established using computed tomography angiography (CTA) and biopsy of indeterminate masses obtained during aortography. Methods: The patient underwent surgical resection of the affected aortic segment along with a tumor. Histopathological examination revealed the diagnosis of a primary malignant tumor of the aorta - angiosarcoma. Postoperatively, patient received adjuvant chemotherapy according to the standard treatment regimen for sarcoma. Results: One month later, postoperative CT of a chest, abdomen and pelvis was performed, revealing no evidence of metastases or pathological lymph nodes in the examined areas. Conclusion: Combined surgical and systemic therapies may improve overall survival.

Keywords: angiosarcoma; hemangiosarcoma; open aortic surgery; primary aortic malignancy.

Figure 1.

(A) and (B) CT angiography scan of the chest, abdomen and pelvis showing thrombus masses of unknown origin and atypical appearance in the abdominal aorta and left renal artery.

Figure 2.

Aortography revealing a tumor mass, accompained by a biopsy sample for histopathological analysis.

Figure 3.

Intraoperative picture showing the interposition of a bifurcated graft at the level of the abdominal aorta, after resection of the angiosarcoma with a bypass to the left renal artery.

Figure 4.

(A) Immunohistochemical reaction with antibody against CD31 (40x); (B) immunohistochemical reaction with antibody against FLI1 (40x).

Figure 5.

Control CT angiography and reconstruction of the chest, abdomen, and pelvis, which shows the vascular prosthesis. The proximal prosthesis end is sutured below the right renal artery, and both distal ends of the bifurcation to common iliac arteries (white arrows).