Intimal sarcoma of the aortic valve and ascending aorta: a case report

Intimal sarcomas are rare tumors that typically affect the major vessels, such as the pulmonary artery and aorta, and are associated with a particularly poor prognosis. Intimal sarcomas found in the aorta are most commonly located in the abdominal section between the celiac artery and the iliac bifurcation. The descending aorta is involved in 30% of cases, while involvement of the ascending aorta is rare. Additionally, the symptoms of intimal sarcomas are usually nonspecific, making preoperative diagnosis difficult. The majority of neoplasms can only be found by histological analysis of the surgical specimen.

A 69-year-old female patient was diagnosed with intimal sarcoma of the ascending aorta and aortic valve, which was initially thought to be nonbacterial thrombotic endocarditis. The patient complained of chest discomfort accompanied by reduced activity tolerance and had elevated serum levels of interleukin 6, lactic dehydrogenase, high-sensitivity troponin I, and brain natriuretic peptide, along with mild anemia. Echocardiography and computed tomography with contrast-enhanced angiography revealed a vegetation measuring 9.8 mm × 6.6 mm between the root of the aorta and aortic valve, suggesting an initial diagnosis of nonbacterial thrombotic endocarditis. However, an aortic intimal sarcoma was detected by immunohistochemistry and postoperative histology. Additionally, ¹⁸F-fluorodeoxyglucose positron emission tomography detected metastasis in the right adrenal gland and left humeral head.

Intimal sarcomas are an uncommon and extremely aggressive type of primary malignant aortic tumor. Diagnosis might be challenging and requires a high level of suspicion. Standard surgical treatment may include resection of the affected segment followed by interposition graft replacement. Given the poor prognosis even after complete resection, it is essential to rule out metastasis.