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] Ahmad Al Maradni, M.D. [3]
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). Common physical examination findings of myxoma include murmur and abnormal heart sounds that change when the patient changes positions. Surgery is the mainstay of treatment for myxomas.
Historical Perspective
Pathophysiology
On gross pathology, a gelatinous, irregular surface that fills the left atrium is characteristic finding of myxoma. On microscopic histopathological analysis, Gamna Bodies consisting of fibrosis and deposition of iron pigments are characteristic findings of myxoma. Complications that can develop as a result of myxoma are: arrhythmias, pulmonary edema, peripheral emboli, metastasis, blockage of the mitral heart valve.
Causes
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. Myxoma is a rare disease that tends to affect infants and children, females are more commonly affected with myxoma than males.[2]
Risk Factors
Common risk factors in the development of myxoma are female gender and genetic predisposition.
Natural History, Complications and Prognosis
Complications that can develop as a result of myxoma are: arrhythmias, pulmonary edema, peripheral emboli, metastasis, blockage of the mitral heart valve.
Diagnosis
Staging
There is no established system for the staging of myxoma.
History and Symptoms
Atrial myxoma symptoms may occur at any time, but most often they tend to occur with changes in body position. Common symptoms include: chest pain, palpitation, dizziness, syncope and dyspnea on exertion.
Physical Examination
Common physical examination findings of myxoma include murmur and abnormal heart sounds that change when the patient changes positions.
Laboratory Findings
Laboratory findings consistent with Myxoma show anemia, leukocytosis and elevated erythrocyte sedimentation rate.
Chest X-ray
On chest x-ray, Myxomas are characterised by calcification overlying the posterior aspect of heart.
CT Scan
On myxoma CT scan is characterised by low attenuation and areas of dystrophic calcification in cardiac chambers.
MRI
on magnetic resonance imaging cardiac myxoma characterised by a soft tissue mass within the cardiac chambers isointense to skeletal muscle.
Echocardiography
On cardiac ultrasound, myxoma is characterised by the presence of a heterogeneous pedunculated mass that is commonly located in the left atrium.
Other Diagnostic Studies
cardiac ventriculography is also used in the diagnosis of myxoma
Treatment
Medical Therapy
Surgery
Surgery is the mainstay of treatment for myxomas.
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)
- ↑ National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/publications/pdp