Case of the Month
Section Editor: Nicholas Stence, MD
Children's Hospital Colorado, Aurora, CO
May 2022
Next Case of the Month Coming June 7...
CNS Manifestations of Cardiac Myxoma
- Clinical Course:
- This case represents an example of a rare cause of cardioembolic ischemic infarcts and distant metastases from recurrent cardiac myxoma, as a demonstration of multiple embolic manifestations of cardiac myxomas.
- A 60-year-old woman presented with left-sided facial droop and weakness. CT and MRI confirmed a right MCA territory infarct with hemorrhage. Additional punctate infarcts in the cerebellum raised concern for cardioembolic infarcts. Echocardiogram was performed to evaluate for a cardiac source of infarct and demonstrated a large, irregular mass in the left atrium, confirmed by cardiac CT.
- Resection of the mass with cardiothoracic surgery performed 2 days later confirmed cardiac myxoma.
- One year later, the patient presented with bilateral blurry vision and ataxia. CT showed hemorrhage in the right frontal and left occipital lobes. MRI of the brain showed them to be enhancing lesions without evidence of stroke, compatible with metastatic lesions. In view of a history of cardiac myxoma, cardiac MRI was performed and showed a new, small, right ventricular enhancing mass.
- Due to the risk of intracranial hemorrhage, the right ventricular mass was not resected. The right frontal brain lesion was, however, resected and revealed myxoid contents, indicating metastases from cardiac myxoma. Subsequent brain MRI follow-up showed interval increase in size of the left occipital mass, indicating tumor progression.
- Background:
- Cardiac myxomas are the most common primary cardiac tumors in adults, although they are a rare cause of embolic stroke.
- Most cardiac myxomas occur within the left atrium, and cardiovascular sequelae of mitral valve obstruction are the most frequent presenting symptoms. However, myxomas within the left atrium are also the most likely to embolize. Systemic embolization occurs in up to 50% of patients with cardiac myxomas, frequently to the cerebral or renal vasculature.
- Cardiac myxoma embolization to the brain can result in ischemic infarcts, neoplastic intracranial aneurysms, or parenchymal metastases.
- Diagnosis:
- Cardiac myxomas are frequently identified during echocardiography to evaluate for a source of suspected cardioembolic infarcts, which would demonstrate a mobile, echogenic mass.
- Cardiac CT or MRI can be obtained to further characterize the lesion or differentiate it from an intracardiac thrombus.
- Treatment:
- Surgical resection is the mainstay of treatment to avoid further embolic or cardiovascular events and is performed after a period of anticoagulation. Prognosis following surgical resection is good, with recurrent disease occurring in approximately 3% with sporadic disease.
- The etiology of myxoma recurrence in sporadic cases is unclear but may be related to incomplete tumor excision, growth from a second focus, or intracardiac implantation from the original tumor.
- In familial cases, including Carney complex, the rate of recurrence is much higher and may be upwards of 20%.
- Radical excision therapy with cardiac transplantation has been reported for those with recurrent disease.
- In patients who are unable to tolerate surgical resection, chemotherapy is usually ineffective, and therapy is aimed at excision or radiation of embolic lesions.
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