Cardiac Myxoma Presenting as Cerebral Aneurysm with Intracerebral Hemorrhage
Daniel Leon-Ariza1, Sofia Matta1, Jose Posas1, Joseph Tarsia1
1Ochsner Clinic Foundation
Objective:

To demonstrate an atypical presentation of cardiac myxoma as an intracerebral aneurysm with hemorrhage with initial negative echocardiogram. 

Background:

Myxomas represent about 83% of all primary cardiac tumors. Mostly occur on the left atrium (83-88%). Systemic embolization presents in 16% of the cases with embolic cerebral infarct. Atrial myxomas cause up to 0.5% of ischemic strokes. Brain metastasis are found months after diagnosis of cardiac myxoma. Myxomatous aneurysms have causes of aneurysm formation such as myxomatous emboli causing vascular occlusion and perivascular damage with pseudoaneurysm formation; vasa vasorum infiltration by myxoma cells; and neoplastic subendothelial infiltration with myxomatous cells with fibroblastic proliferation and neovascularization. 

Design/Methods:
NA.
Results:

44-year-old female with history of migraines. Initially presented with sudden onset headaches and bilateral vision loss. On admission, a right occipital hemorrhage was treated as a P3 fusiform aneurysm rupture of unknown origin. TTE was negative. Patient was discharged and lost to follow-up with Vascular Neurology. Months later, patient developed severe headaches with seizure-like activity. Vasogenic edema and hemorrhage on CT head. CT abdomen and pelvis suggested metastatic disease. Cardiac MRI with left atrial mass suggested cardiac tumor. TEE demonstrated a left atrial multilobular mass. Biopsy of mass consistent with cardiac myxoma with glandular elements. Glandular elements were positive for cytokeratins (AE1/AE3, CK7), CEA and epithelial membrane antigen. 

Conclusions:

This is a rare presentation of tumor emboli as the first sign of cardiac myxoma. An elusive negative TTE in the setting of an ICH was done until substantial vasogenic edema and increased hemorrhage appeared several months later. Cytokeratin-positive cardiac myxoma with glandular elements seen on biopsy. CK7 has been associated with metastatic cancers. Caspase-cleaved cytokeratins levels are related with disruption of cerebral architecture leading to increased intracerebral hemorrhage. 

10.1212/WNL.0000000000205388