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A woman in her 20s presented to the emergency department with dysarthria and mild ataxia. Her medical history was significant for Raynaud’s phenomenon, hypertension, anxiety, depression and cigarette smoking. Due to concerns for cerebral vasculitis, digital subtraction angiography (DSA) was obtained. No abnormality was detected (figure 1). Echocardiogram identified a 2.3 cm×4.8 cm left atrial mass, which was surgically excised. Histopathology of the lesion confirmed atrial myxoma.
Seven years later, she represented to the emergency department after a fall. Cranial CT showed multiple fusiform cerebral aneurysms, confirmed on DSA (figure 2). With few treatment options available, she remains on conservative management at our institution via yearly MR angiogram, with no significant change after 4 years.
Myxomas are the most common benign primary cardiac tumour, with over 75% arising in the left atrium.1 Rarely, myxomas may result in the formation of multiple cerebral aneurysms, typically fusiform in nature,2 as in this case, which is unusual for having comparison normal cerebral DSA prior to the lesion excision. Affected patients are predominantly middle aged and female. A recent systematic review noted 55 cases described in the literature to date, of which 35 patients were female and 86% of patients were younger than 60 years of age at time of diagnosis.2
The pathogenesis of cerebral aneurysm formation in the context of atrial myxoma is unclear. Penetration of cerebral vessel-wall endothelium by embolic myxoma cells is felt to be the cause of aneurysm formation. These cells subsequently proliferate in the subintimal layer, destroying the arterial wall.2 This theory is supported by histopathological studies which demonstrate myxoma cells in the lumen of resected aneurysm tissue.3–5 Although resection of atrial myxoma minimises the risk of subsequent embolic phenomena, it does not decrease the risk of delayed cerebral aneurysm formation.2 There are similarities with intracranial mycotic aneurysm formation secondary to septic emboli, with aneurysms developing in peripheral intracranial branches after vascular wall damage.6 Other causes of multiple cerebral aneurysm formation include polycystic kidney disease and hypertension.7 8
Atrial myxomas are a rare benign primary cardiac tumour, tending to arise in the left atrium.
Patients with left-sided atrial myxoma may rarely form multiple fusiform cerebral aneurysms.
Resection of myxoma tissue minimises risk of subsequent embolic phenomena, but does not decrease the risk of cerebral aneurysm formation.
Patient consent for publication
Contributors AA was involved in conceptualisation, patient consent, preparation of the manuscript and revision of the manuscript. AC was involved in conceptualisation, patient care, preparation of the manuscript and revision of the manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.