Intimal aortic sarcoma mimicking ruptured thoracoabdominal type IV aneurysm. a rare case report and review of the literature

Abstract

Primary intimal aortic sarcoma represents a very rare and highly lethal medical entity. Diagnosis is made either by embolic events caused by the tumor or by surrounding tissue symptoms such as pain. Herein we report an extremely rare case of a 51-year-old man previously operated for ascending aortic aneurysm, who presented with clinical and radiological findings suggestive of a ruptured thoracoabdominal type IV aneurysm. The patient underwent radical resection of the aorta and surrounding tissue with placement of a composite 4-branched graft. The diagnosis was made by frozen section and regular histopathologic examination of the specimen and the patient received adjuvant chemotherapy. Nine months after surgery the patient is still alive and has no signs of recurrence. We review the literature and discuss the option of postoperative chemotherapy.

Aortic Rupture due to Co-localization of Aortic Intimal Myofibroblastic Sarcoma and Urothelial Carcinoma: A Unique Case Report

“…Although Thalheimer et al ( 3 ) categorized intimal sarcomas as intimal angiosarcomas and IMFSs, as described above, it seems that the concept of IMFS has not been widely recognized, as no intimal sarcoma case has been reported under the name of IMFS, while a relatively large number of cases of intimal angiosarcoma have been reported. However, we found at least two reported cases ( 5 , 6 ) that correspond to IMFS in terms of the immunohistochemical findings of positivity for α-SMA and negativity for CD31 and CD34, as seen in the present case. These immunohistochemical findings may be able to be used to differentiate IMFS from aortic intimal angiosarcoma.…”

“…Aortic sarcomas sometimes present as acute aortic rupture or dissection ( 5 , 6 , 9 , 10 ). In addition, there have been a few aortic rupture cases due to the invasion of tumors besides aortic sarcomas ( 7 , 8 ).…”

Aortic Intimal Sarcoma Contributes to Atherosclerotic Renovascular Hypertension: An Autopsy Case Report and Review of the Literature

“…An acute or sub-acute embolus and obstruction or stenosis of any vessel are indicative of AIS. On the other hand, the mural type usually extends extramurally to the para-aortic tissues and lymph nodes (11).…”

“…Primary aortic sarcomas are classified into the intimal type, which originates from the endothelium, and the mural type, which originates from the media or adventitia. This classification depends on both the location and immunohistochemical pattern of the tumors (11). AIS progresses insidiously and tends to spread widely along the lumen, obstructing all branches of the aorta (12); the most common location is the abdominal aorta (13).…”

Primary Sarcoma of Descending Aorta

“…1 To date, the role of neoadjuvant chemotherapy is debated and surgery still remains the mainstay option when the tumor is resectable. 2 We report the case of a surgically treated primary sarcoma of the aorta.…”