Atrial myxoma

Adrian Marchidann MD (

Dr. Marchidann of SUNY Downstate Medical Center has no relevant financial relationships to disclose.

)
Steven R Levine MD, editor. (

Dr. Levine of the SUNY Health Science Center at Brooklyn has received honorariums from Genentech for service on a scientific advisory committee and a research grant from Genentech as a principal investigator.

)
Originally released June 11, 2001; last updated March 12, 2018; expires March 12, 2021

This article includes discussion of atrial myxoma and cardiac myxoma. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Atrial myxoma is the most common intracardiac tumor in adults. Atrial myxoma may impair the cardiac function and cause stroke by embolization. Additionally, myxoma may mimic infective, immunologic, and malignant diseases. Several cutaneous syndromes have been described that may facilitate timely recognition. Its protean clinical manifestations make the clinical diagnosis difficult. Approximately 60% of all tumors are found by echocardiogram. The definitive treatment is surgical excision. In this update, the author updates the data on clinical neurologic manifestations and treatment modalities of cardiac myxoma and ischemic stroke caused by large artery occlusion.

Key points

 

• Atrial myxoma is the most common intracardiac tumor. Although benign by histopathologic criteria, its central location may result in a fatal outcome if not treated in a timely manner.

 

• Myxomas have a wide variety of cardiac, embolic, and systemic manifestations, including paraneoplastic syndromes, mediated by a multitude of humoral factors.

 

• No guidelines for acute ischemic stroke caused by atrial myxoma embolism treatment have been published.

 

• Intravenous thrombolysis within a 3-hour window was used successfully in some patients with acute stroke caused by myxoma.

 

• The risk of hemorrhagic transformation may be higher than in other patients due to prior asymptomatic infarcts or aneurysms.

 

• Intra-arterial recanalization is more effective than the usual treatments, including intravenous tPA, in patients with demonstrated large artery occlusion.

Historical note and terminology

Left atrial myxoma was first described over 150 years ago (King 1845). The first antemortem diagnosis, by angiocardiography, was in 1952 (Knepper et al 1988), and the first surgical excision documented 3 years later (Crafoord 1955).

The first documented association between nonembolic dermatologic manifestations and atrial myxomas was made in 1973. The nevus, atrial myxoma, myxoid neurofibromata, and ephelides or endocrine hyperactivity (NAME syndrome); lentigines, atrial myxoma, and blue nevi (LAMB syndrome); and Carney complex (an atrial myxoma associated with multiple neoplasia lentigines) were first reported in 1980, 1984, and 1985, respectively (Greeson et al 1998). Sneddon syndrome, which commonly presents with a livedo-type rash and diffuse cerebrovascular disease, has also been associated with atrial myxoma (Schellong et al 1997).

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