Myxoma overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2]Maria Fernanda Villarreal, M.D. [3]Cafer Zorkun, M.D., Ph.D. [4]Ahmad Al Maradni, M.D. [5]
Overview
A myxoma (Myxo- = Latin for mucus) is the most common primary tumor of the heart. Cardiac myxoma was first described in 1845. In 1951, Prichard described a kind of microscopic endocardial structure of the atrial septum, which was suggested to be associated with cardiac myxoma. Clarence Crafoord, a Swedish cardiovascular surgeon successfully removed a cardiac myxoma for the first time in 1954. Before 1951, cardiac myxoma diagnosis was made only at post-mortem examination. In 1959, the first M-mode echocardiogram of a left atrial myxoma was reported. Cardiac myxomas are classified by the WHO histological classification of tumors of the heart "Benign tumors and tumor-like lesions" and categorized into a type of pluripotent mesenchymal tumor. Cardiac myxoma arises from remnants of subendocardial vasoformative reserve cells, which are primitive mesenchymal cells that are normally involved in the supportive structure of the endocardium. Myxomas are usually located in the fossa ovalis and endocardium of the atrial septum. Some symptoms of myxoma may be associated with the release of interleukin 6 (IL-6). On gross pathology, a gelatinous, irregular surface that fills the left atrium is the characteristic finding of myxoma. A common histopathological finding is the Gamna-Gandy Bodies that consist of fibrosis and deposition of iron pigments. The main cause of cardiac myxoma remains unknown. However, in some cases like inherited myxomatosis, there is a strong relation with genetic mutations of PRKAR1A gene. Cardiac myxoma should be differentiated from other benign and malignant primary heart tumors including papillary fibroelastoma, lipoma, rhabdomyoma, and cardiac metastasis. Cardiac myxomas are the most common primary cardiac tumor in adults, with a reported prevalence of 0.03% in general population. The incidence of cardiac myxoma is about 1/ 100,000 per year. The majority of patients with cardiac myxoma are diagnosed between 30 to 60 years; children are rarely affected. Females are more commonly affected with cardiac myxoma than men. The female-to-male ratio is approximately 1.8 to 1. Cardiac myxomas represent 78% of heart tumors. Common risk factors in the development of myxoma are female gender and genetic predisposition. In some cases, right atrial myxoma has been associated with tricuspid stenosis and atrial fibrillation. If left untreated, cardiac myxoma progression occurs slowly. The overlap of various phenomena such as thrombosis, hemorrhage, or fragmentation may influence tumor growth, detachment, and consequently embolism. Constitutional symptoms, such as: weight loss, fatigue, weakness are often the initial clinical onset of cardiac myxoma, and may resemble those from endocarditis. Metastases are uncommon in cardiac myxoma. Since the majority of the cardiac myxomas are left-sided, it may progress to develop mitral valve obstruction or systemic embolic events, such as stroke. Whereas, right atrial myxomas may obstruct the tricuspid valve and can present as right sided heart failure. Approximately 20% of patients with cardiac myxoma are asymptomatic. Overall, clinical features of cardiac myxoma are associated with the size of the tumor, location, size, and mobility. There is no single diagnostic study of choice for myxoma. Myxoma may be diagnosed based on clinical presentation and detecting the cardiac mass. Symptoms associated with cardiac myxomas are typically due to the effect of the mass of the tumor obstructing the normal blood flow within the heart chambers. Left atrial myxoma symptoms may mimic mitral stenosis, while right atrial myxomas rarely produce symptoms until they have grown to be at least 13 cm wide. General symptoms may also mimic those of infective endocarditis. Cardiac 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. There are no specific physical findings for cardiac myxoma. The auscultatory presence of a "tumor plop" (which is caused by the obstruction of the mitral valve orifice by the tumor) on physical examination is highly suggestive of cardiac myxoma. Common physical examination findings of cardiac myxoma include systolic or diastolic murmurs (depending on size, mobility, and location of the tumor). Laboratory findings consistent with cardiac myxoma are generally non-specific, results often demonstrate anemia, leukocytosis and elevated erythrocyte sedimentation rate. On chest x-ray, cardiac myxoma is characterized by normal results and in some cases a calcification overlying the heart. On CT scan, cardiac myxoma is characterized by low attenuation and areas of dystrophic calcification in cardiac chambers. CT scan may be helpful in the diagnosis of cardiac myxoma because it provides better soft-tissue contrast than echocardiography, and it can also differentiate calcification and fat, and may allow tissue diagnosis of some masses such as lipomas. On Magnetic Resonance Imaging (MRI) or Cardiac Magnetic Resonance (CMR), cardiac myxoma is characterized by a soft tissue mass within the cardiac chambers isointense to skeletal muscle. This imaging modality plays an important role in the evaluation of cardiac masses and is of great value when echocardiographic findings are suboptimal or when the lesion has an atypical location or appearance. The echocardiogram is the initial modality and most useful diagnostic imaging study in cardiac myxoma. On cardiac ultrasound, cardiac myxoma is characterized by the presence of a heterogeneous pedunculated mass that is commonly located in the left atrium. Echocardiography allows for evaluation assessment of tumor mobility, as it often protrudes through valve flaps. As a testing modality, two-dimensional echocardiography is often coupled with other modalities (such as, Doppler echocardiography) to detect vascular abnormalities that frequently occur in cardiac myxomas. Another diagnostic study for cardiac myxoma is cardiac angiography, which often demonstrates contrast media-enhanced tumor vasculature. There is no known medical therapy for cardiac myxomas. Surgery is the mainstay of treatment for cardiac myxoma. The feasibility of surgery depends on the patient hemodynamic stability at diagnosis. Cardiac myxoma surgery has operative mortality around 0 to 3%, depending on risk factors or mechanical damage to a heart valve, as well as adhesion of the tumor to valve leaflets. The short and long-term prognosis is generally regarded as excellent.
Historical Perspective
Cardiac myxoma was first described in 1845. In 1951, Prichard described a kind of microscopic endocardial structure of the atrial septum, which was suggested to be associated with cardiac myxoma. Clarence Crafoord, a Swedish cardiovascular surgeon successfully removed a cardiac myxoma for the first time in 1954. Before 1951, cardiac myxoma diagnosis was made only at post-mortem examination. In 1959, the first M-mode echocardiogram of a left atrial myxoma was reported.
Classification
Cardiac myxomas are classified by the WHO histological classification of tumors of the heart "Benign tumors and tumor-like lesions" and categorized into a type of pluripotent mesenchymal tumor.
Pathophysiology
Cardiac myxoma arises from remnants of subendocardial vasoformative reserve cells, which are primitive mesenchymal cells that are normally involved in the supportive structure of the endocardium. Myxomas are usually located in the fossa ovalis and endocardium of the atrial septum
Some symptoms of myxoma may be associated with the release of interleukin 6 (IL-6). On gross pathology, a gelatinous, irregular surface that fills the left atrium is the characteristic finding of myxoma. A common histopathological finding is the Gamna-Gandy Bodies that consist of fibrosis and deposition of iron pigments.
Causes
The main cause of cardiac myxoma remains unknown. However, in some cases like inherited myxomatosis, there is a strong relation with genetic mutations of PRKAR1A gene.
Differentiating Myxoma from other Diseases
Cardiac myxoma should be differentiated from other benign and malignant primary heart tumors including papillary fibroelastoma, lipoma, rhabdomyoma, and cardiac metastasis.
Epidemiology and Demographics
Cardiac myxomas are the most common primary cardiac tumor in adults, with a reported prevalence of 0.03% in general population. The incidence of cardiac myxoma is about 1/ 100,000 per year. The majority of patients with cardiac myxoma are diagnosed between 30 to 60 years; children are rarely affected. Females are more commonly affected with cardiac myxoma than men. The female-to-male ratio is approximately 1.8 to 1. Cardiac myxomas represent 78% of heart tumors.
Risk Factors
Common risk factors in the development of myxoma are female gender and genetic predisposition. In some cases, right atrial myxoma has been associated with tricuspid stenosis and atrial fibrillation.
Natural History, Complications and Prognosis
If left untreated, cardiac myxoma progression occurs slowly. The overlap of various phenomena such as thrombosis, hemorrhage, or fragmentation may influence tumor growth, detachment, and consequently embolism. Constitutional symptoms, such as: weight loss, fatigue, weakness are often the initial clinical onset of cardiac myxoma, and may resemble those from endocarditis. Metastases are uncommon in cardiac myxoma.
Since the majority of the cardiac myxomas are left-sided, it may progress to develop mitral valve obstruction or systemic embolic events, such as stroke. Whereas, right atrial myxomas may obstruct the tricuspid valve and can present as right sided heart failure. Approximately 20% of patients with cardiac myxoma are asymptomatic. Overall, clinical features of cardiac myxoma are associated with the size of the tumor, location, size, and mobility.
Diagnosis
Diagnostic Study of Choice
There is no single diagnostic study of choice for myxoma. Myxoma may be diagnosed based on clinical presentation and detecting the cardiac mass.
History and Symptoms
Symptoms associated with cardiac myxomas are typically due to the effect of the mass of the tumor obstructing the normal blood flow within the heart chambers. Left atrial myxoma symptoms may mimic mitral stenosis, while right atrial myxomas rarely produce symptoms until they have grown to be at least 13 cm wide. General symptoms may also mimic those of infective endocarditis. Cardiac 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
There are no specific physical findings for cardiac myxoma. The auscultatory presence of a "tumor plop" (which is caused by the obstruction of the mitral valve orifice by the tumor) on physical examination is highly suggestive of cardiac myxoma. Common physical examination findings of cardiac myxoma include systolic or diastolic murmurs (depending on size, mobility, and location of the tumor).
Laboratory Findings
Laboratory findings consistent with cardiac myxoma are generally non-specific, results often demonstrate anemia, leukocytosis and elevated erythrocyte sedimentation rate.
Chest X-ray
On chest x-ray, cardiac myxoma is characterized by normal results and in some cases a calcification overlying the heart.
CT Scan
On CT scan, cardiac myxoma is characterized by low attenuation and areas of dystrophic calcification in cardiac chambers. CT scan may be helpful in the diagnosis of cardiac myxoma because it provides better soft-tissue contrast than echocardiography, and it can also differentiate calcification and fat, and may allow tissue diagnosis of some masses such as lipomas.
MRI
On Magnetic Resonance Imaging (MRI) or Cardiac Magnetic Resonance (CMR), cardiac myxoma is characterized by a soft tissue mass within the cardiac chambers isointense to skeletal muscle. This imaging modality plays an important role in the evaluation of cardiac masses and is of great value when echocardiographic findings are suboptimal or when the lesion has an atypical location or appearance.
Echocardiography
The echocardiogram is the initial modality and most useful diagnostic imaging study in cardiac myxoma. On cardiac ultrasound, cardiac myxoma is characterized by the presence of a heterogeneous pedunculated mass that is commonly located in the left atrium. Echocardiography allows for evaluation assessment of tumor mobility, as it often protrudes through valve flaps. As a testing modality, two-dimensional echocardiography is often coupled with other modalities (such as, Doppler echocardiography) to detect vascular abnormalities that frequently occur in cardiac myxomas.
Other Diagnostic Studies
Another diagnostic study for cardiac myxoma is cardiac angiography, which often demonstrates contrast media-enhanced tumor vasculature.
Treatment
Medical Therapy
There is no known medical therapy for cardiac myxomas.
Surgery
Surgery is the mainstay of treatment for cardiac myxoma. The feasibility of surgery depends on the patient hemodynamic stability at diagnosis. Cardiac myxoma surgery has operative mortality around 0 to 3%, depending on risk factors or mechanical damage to a heart valve, as well as adhesion of the tumor to valve leaflets. The short and long-term prognosis is generally regarded as excellent.