Recurrent Embolic Stroke From Aortic Intimal Sarcoma Mimicking Atherosclerotic Disease

Intimal sarcoma of the aorta is an extremely rare malignancy frequently misdiagnosed as atherosclerotic disease. 1,2Herein, we present a case of recurrent embolic strokes initially attributed to aortic arch atherosclerosis, ultimately diagnosed as intimal sarcoma after surgery.A 67-year-old female with hypertension, dyslipidemia, and prediabetes presented with acute left leg weakness.Brain MRI demonstrated multiple small cortical embolic infarctions in the right hemisphere (Fig. 1A).No significant atherosclerotic stenosis was identified in the intracranial or extracranial arteries.Echocardiography, Holter monitoring, and transcranial Doppler ruled out cardiogenic and paradoxical embolism.Given the absence of an identifiable cardioembolic source and clinical concern for cancer-related stroke, chest CT angiography was subsequently performed to evaluate for occult malignancy and aortic pathology as alternative embolic sources.Chest CT angiography demonstrated severe stenosis at the origin of the right brachiocephalic trunk and an irregular, plaque-like intraluminal lesion along the aortic arch, initially interpreted as atherosclerotic plaque (Fig. 1B andC).The patient was started on dual antiplatelet and high-intensity statin therapy.Despite full adherence to prescribed medications and well-controlled cardiovascular risk factors, approximately 50 days later, she presented with new-onset right leg weakness.Repeat brain MRI revealed new acute infarctions in the left hemisphere (Fig. 1D).Given recurrent stroke despite optimal medical therapy and irregular and ulcerative morphology with severe luminal stenosis prompted the decision for surgical intervention.She was referred for aortic arch intervention.Total aortic arch replacement was performed.The tumor demonstrated irregular margins and a shaggy appearance, extending from the distal ascending aorta to the left common carotid artery (Fig. 1E).A friable mural thrombus was removed.Histopathological examination unexpectedly revealed high-grade intimal sarcoma.Immunohistochemistry showed focal positivity for actin and GATA3, with negative staining for CD34 and desmin.Following surgery, dual antiplatelet therapy (aspirin and clopidogrel) was maintained for 3 months and subsequently de-escalated to aspirin monotherapy.Adjuvant chemotherapy has been ongoing since the pathological diagnosis.Over 7 months of follow-up, no recurrence of cerebral infarction has been observed.Our case shares similarities with previously reported cases. 344]56 With respect to infarct patterns, Mo et al. 3 reported multiple embolic infarctions involving the cerebellum and cortex, while Shimizu et al. 4 described multiple infarcts in the left frontal cortex.Natsume et al. 5 documented multiple cortical embolic infarctions, and Kim et al. 6 reported a large infarction in the left middle cerebral artery territory.Taken together, including the present case, aortic intimal sarcoma appears to predominantly manifest as either multiple cortical embolic infarctions or large territorial infarction, consistent with an embolic mechanism originating from the aortic lesion.As noted by Ropp et al., 2 intimal sarcoma is often misdiagnosed as atheromatous plaque on imaging.Unlike previous reports of younger patients, 6 our elderly pa-