Myxoma overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]
Overview
A myxoma (Myxo- = Latin for mucus) is the most common primary tumor of the heart. Myxomas are usually located in either the left or right atrium of the heart; about 86 percent occur in the left atrium.[1] Myxomas are typically pedunculated, with a stalk that is attached to the interatrial septum. The most common location for attachment of the stalk is the fossa ovalis region of the interatrial septum. The phrase "myxomatous degeneration" refers to the process in which connective tissue becomes filled with mucus.
About 71% of myxomas occur in the heart, 41% on the skin, and 7% in the oral cavity (usually on the palate).
Historical Perspective
Pathophysiology
On gross pathology, a gelatinous, irregular surface that fills the left atrium is characteristic findings of Myxoma.
On microscopic histopathological analysis, Gamna Bodies consisting of fibrosis and deposition of iron pigments are characteristic findings of Myxoma.
Causes
Genetic factors are thought to be involved in causes of Myxoma as it tends to occur more frequently in families.
Differentiating Myxoma from other Diseases
Myxoma should be differentiated from other cardiac tumor including lipoma, rhabdomyoma, rhabdomyosarcoma, thrombosis and metastasis.
Epidemiology and Demographics
The incidence of benign cardiac tumor including Myxoma is about 250 per 100.000 persons, the majority are infants and children with females being more commonly affected.
Risk Factors
Common risk factors in the development of Myxoma are female gender and genetic predisposition.
Natural History, Complications and Prognosis
Myxoma is usually a benign tumor, but complications such as: Arrhythmias, Pulmonary edema, Peripheral emboli, metastasis, Blockage of the mitral heart valve can occur.
Diagnosis
Staging
Myxoma is a benign tumor with no established system for the staging.
History and Symptoms
Atrial myxoma Symptoms may occur at any time, but most often they tend occur with change in body position, left atrial myxoma symptoms may mimic mitral stenosis, right atrial myxomas rarely produce symptoms until they have grown to be at least 13 cm (about 5 inches) wide, general symptoms may also mimic those of infective endocarditis.
Physical Examination
Common physical examination findings of Myxoma include abnormal heart sounds, that change when the patient changes postioion, or murmur.
Laboratory Findings
Complete blood count in patient with Myxoma may show anemia and elevated white blood cells. The erythrocyte sedimentation rate may be increased.
Chest X-ray
On chest x-ray, Myxomas are characterised by calcification.
CT Scan
CT scan show low attenuation and areas of dystrophic calcification in Myxoma.
MRI
Cardiac Magnetic resonance Imaging can help non-invasively in the diagnosis Myxoma.
Echocardiography
On cardiac ultrasound Myxoma appears as a heterogeneous pedunculated mass commonly in left atrium.
Other Diagnostic Studies
Doppler study, cardiac MRI and cardiac ventriculography are also used in the diagnosis of Myxoma.
Treatment
=Medical Therapy
Surgery
Surgery is the mainstay of treatment for Myxomas. The surgeon removes the myxoma, along with at least 5 surrounding millimeters of atrial septum, then repair the septum using material from the pericardium.
References
- ↑ Knepper LE, Biller J, Adams HP Jr, Bruno A. Neurologic manifestations of atrial myxoma. A 12-year experience and review. Stroke. 1988 Nov;19(11):1435-40. (Medline abstract)