Myxoma

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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]

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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.

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).

Diagnosis

It is most seen on echocardiography, as a pedunculated mass that is heterogeneous in appearance.

The differential diagnosis include other cardiac tumors such as lipomas and rhabdomyomas. These other tumors of the heart are typically not pedunculated, however, and are more likely to infiltrate the muscle of the heart. Cardiac Magnetic resonance Imaging can help non-invasively diagnose cardiac tumors.

Symptoms

Symptoms associated with cardiac myxomas are typically due to the effect of the mass of the tumor obstructing the normal flow of blood within the chambers of the heart.

Some symptoms of myxoma may be associated with the release of interleukin 6 (IL-6) by the myxoma. High levels of IL-6 may be associated with a higher risk of embolism of the myxoma.

Symptoms of a cardiac myxoma include:

• Dyspnea on exertion
• Fever
• Weight loss
• Lightheadedness or syncope (Loss of consciousness)
• Hemoptysis
• Sudden death

Diagnosis

Chest x-ray

• 
  Lateral chest radiograph from a 16-year-old girl with syncope and bacterial endocarditis. The radiograph demonstrates two areas of dense calcification (arrowheads) overlying the posterior aspect of heart. The posterior-anterior (PA) view confirmed location in the heart (not shown). At surgery a calcified myxoma of the right atrium was removed. Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

Echocardiography

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  Left Atrial Myxoma (M Mode echo)
• 
  Transthoracic echocardiogram (short axis, parasternal view) through the level of the atria shows a round, echogenic mass (arrowhead) within the left atrium attached to the atrial septum. The patient was a 44-year-old woman with chest pain and left atrial myxoma. AO = aorta; RA = right atrium; RV = right ventricle. Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

Cardiac CT

• 
  CT demonstrate a cardiac myxoma in the left atrium Image courtesy of RadsWiki and copylefted
• 
  CT demonstrate a cardiac myxoma in the left atrium Image courtesy of RadsWiki and copylefted
• 
  CT demonstrate a cardiac myxoma in the left atrium Image courtesy of RadsWiki and copylefted

Cardiac MRI

• 
  Axial T1-weighted MRI shows a soft tissue mass within the left atrium isointense to skeletal muscle (arrowheads). A friable myxoma was removed from the left atrium of a 53-year-old man with cerebrovascular accidents.

Gross Pathological Findings

Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

• 
  A gelatinous tumor is attached by a narrow pedicle to the atrial septum. The myxoma has an irregular surface and nearly fills the left atrium.
• 
  Left Atrial Myxoma

• 
  Myxoma Embolus to Iliac Bifurcation: An embolized fragment of the tumor
• 
  Atrial Myxoma: A calcified right atrial mass on the X ray of a 47-year-old man. Resection demonstrated a smooth-surfaced tumor. The gritty material seen microscopically on cut section was calcified and ossified myxoma.

• 
  Myxoma: Gross natural color (outstanding) photo of intact large left atrial myxoma with atrium and left ventricle opened in usual way

Histological Findings

Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

• 
  Cardiac myxoma with ossification: Note central area of bone surrounded by myxoma.
• 
  Cardiac Myxoma: Gamna Bodies: A peculiar form of fibrosis with deposition of iron pigment, identical to that seen in the spleens of patients with sickle cell anemia, is not uncommon in myxoma.

• 
  Cardiac Myxoma Common features at the interface with the atrial septum include lymphoid aggregates, smooth muscle bundles, and thick walled vessels which angiographically may look like neovascularization.
• 
  Cardiac Myxoma The extramedullary hematopoiesis seen here is present in about 7 percent of cardiac myxomas.

• 
  Cardiac Myxoma Glandular structures are seen in less than 5 percent of cases. In this example, they were limited to the base of the myxoma
• 
  Cardiac Myxoma In this myxoma, glandular structures compose the majority of the lesion. Typical myxoma nests merge imperceptibly with glands. The differential diagnosis includes metastatic carcinoma; however, atypia and mitoses are absent.

Videos

Right atrial myxoma

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Treatment

Myxomas are usually removed surgically. The surgeon removes the myxoma, along with at least 5 surrounding milimeters of atrial septum. He or she will then repair the septum using material from the pericardium.

See also

• Carney complex
• Myxomatosis
• Primary tumors of the heart

External links

• Template:GPnotebook
• Template:MerckHome

Acknowledgements

The content on this page was first contributed by: C. Michael Gibson M.S., M.D. Template:SIB de:Myxom sv:Myxom