Atrial myxoma

Adrian Marchidann MD (Dr. Marchidann of SUNY Health Science 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 February 21, 2017; expires February 21, 2020

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, and 60% of all tumors are found by echocardiogram. Its protean clinical manifestations make the clinical diagnosis difficult. Atrial myxomas explain a minority of all strokes, but many embolize systemically and may cause cardiac symptoms and even paraneoplastic syndromes. Its management requires surgical excision. In this update, Dr. Marchidann of State University of New York (SUNY) Health Science Center updates the data on clinical neurologic manifestations and treatment modalities of cardiac myxoma and ischemic stroke caused by large artery occlusion.

Key points

 

• 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, ephelides syndrome; lentigines, atrial myxoma, blue nevi syndrome; and Carney complex (a multiple neoplasia and lentiginosis syndrome) 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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