You are asked to make a diagnosis in a 56-year-old man who presents with the sudden onset of left-sided weakness and numbness involving his face, upper extremity and, to a lesser extent, his leg. This event was preceded by a 6 month history of intermittent fever, anorexia, malaise, and a weight loss of 6 kg. He had intermittent episodes of shortness of breath, relieved by lying down. Basic lab studies are notable for the presence of a microcytic anemia, polyclonal hypergammaglobulinemia, and an elevated erythrocyte sedimentation rate (ESR). His chest film and an apical recording of his heart are found below. What is your diagnosis, what test(s) would you order to confirm your suspicions, and what treatment, if any, would you recommend?
Click image to hear "tumor plop"
DIAGNOSIS: Left atrial myxoma with systemic emboli and an audible "tumor plop". Myxomas are the most common primary cardiac tumor, accounting for one-third to one-half of cases. Approximately seven percent of myxomas are familial or part of the NAME syndrome (nevi, atrial myxoma, myxoid neurofibroma and ephelides) or the LAMB syndrome (lentigines, atrial myxoma, and blue nevi). Arising from the interatrial septum in the vicinity of the fossa ovalis, they are more commonly located in the left atrium where they can (as in this patient) fragment, causing systemic embolization and an inflammatory response with elevated ESRs, CRP,s and immunoglobulins. The most common clinical presentation resembles mitral valve disease as the tumor can obstruct the mitral valve causing a mitral stenosis-like murmur and platypnea. Damage to the mitral valve can result in the murmur of mitral insufficiency. As in the presented patient, the tumor can move in an out of the left ventricle (see angiogram above), causing a "tumor plop" as the myxoma hits against the ventricular wall. The treatment is surgical (see the pathology specimen above). Note that the chest film is compatible with left ventricular enlargement, and that the left atrial appendage is prominent. You can click this image to listen to the tumor plop.