Cerebral Myxomatous Aneurysms: Case Series and Systematic Review of Literature with Adequate Follow-Up Periods and Aneurysmal Wall Biopsy Results

Abstract

Background: Cerebral myxomatous aneurysms (CMA) are intracranial aneurysms caused by cardiac myxoma. The exact mechanism underlying their development has not yet been elucidated. And an optimal treatment method has not yet been established because of rarity of the disease. In addition, most existing reports have had a short follow-up period or lack of follow-up imaging test results. The aim of this study was to provide better insights into the course and treatment options of CMAs.

Methods: We describe 4 CMA patients treated in our hospital and literature search was performed using PubMed and Embase databases. Keywords used were as follows: "cerebral myxomatous aneurysm", "myxomatous aneurysm", "cardiac myxoma", and "intracranial aneurysm". Only publications in English and related to this disorder with adequate follow-up periods and aneurysmal wall biopsy results were included. Clinical, radiological, pathological, and treatment characteristics were analyzed.

Results: A total of 149 CMA cases were managed conservatively that included 9 enlargements, 6 regressions, and 134 stable aneurysms, which were identified for a total of 453.33 aneurysm years (1.91% per aneurysm year, 1.27% per aneurysm year, and 28.76% per aneurysm year, respectively). Poor outcome rate was high in cases with hemorrhage (either parenchymal or subarachnoidal hemorrhage) due to rupture of the CMA (46.67%, 7 out of 15). Thirteen cases had aneurysm biopsy results of which 11 showed tumor invasion on the aneurysm wall.

Conclusions: Even though the natural course of unruptured, benign-looking CMAs appears to be favorable, the presence or absence of viable tumor cells in the aneurysm may promote an unfavorable disease course. In the case of large, symptomatic, and enlarging CMAs, surgical, endovascular, or combined treatment should be considered as the mortality and morbidity due to rupture might be high.

Keywords: Cardiac myxoma; Cerebral myxomatous aneurysm; Fusiform aneurysm; Intracranial aneurysm; Myxomatous aneurysm; Subarachnoid hemorrhage.