A myxoma is a rare
benign
Malignancy () is the tendency of a medical condition to become progressively worse; the term is most familiar as a characterization of cancer.
A ''malignant'' tumor contrasts with a non-cancerous benign tumor, ''benign'' tumor in that a malig ...
tumor
A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...
of the
heart
The heart is a muscular Organ (biology), organ found in humans and other animals. This organ pumps blood through the blood vessels. The heart and blood vessels together make the circulatory system. The pumped blood carries oxygen and nutrie ...
. Myxomata are the most common primary cardiac tumor in adults, and are most commonly found within the left atrium near the valve of the
fossa ovalis. Myxoma may also develop in the other heart chambers.
The tumor is derived from multipotent
mesenchymal
Mesenchyme () is a type of loosely organized animal embryonic connective tissue of undifferentiated cells that give rise to most tissues, such as skin, blood, or bone. The interactions between mesenchyme and epithelium help to form nearly ever ...
cells.
Cardiac myxoma can affect adults between 30 and 60 years of age.
Signs and symptoms
Symptoms may occur at any time, but most often they accompany a change of body position. Pedunculated myxomata can have a "wrecking ball effect", as they lead to stasis and may eventually embolize themselves. Symptoms may include:
*
Shortness of breath
Shortness of breath (SOB), known as dyspnea (in AmE) or dyspnoea (in BrE), is an uncomfortable feeling of not being able to breathe well enough. The American Thoracic Society defines it as "a subjective experience of breathing discomfort that con ...
with activity
*
Platypnea – Difficulty breathing in the upright position with relief in the supine position
*
Paroxysmal nocturnal dyspnea – Breathing difficulty when asleep
*
Dizziness
Dizziness is an imprecise term that can refer to a sense of disorientation in space, vertigo, or lightheadedness. It can also refer to Balance disorder, disequilibrium or a non-specific feeling, such as giddiness or foolishness.
Dizziness is a ...
*
Fainting
Syncope , commonly known as fainting or passing out, is a loss of consciousness and muscle strength characterized by a fast onset, short duration, and spontaneous recovery. It is caused by a decrease in blood flow to the brain, typically from ...
*
Palpitations – Sensation of feeling your heart beat
* Chest pain or tightness
* Sudden Death (In which case the disease is an autopsy finding)
The symptoms and signs of left atrial myxomata often mimic
mitral stenosis
Mitral stenosis is a valvular heart disease characterized by the Stenosis, narrowing of the opening of the mitral valve of the heart. It is almost always caused by Rheumatic Heart Disease, rheumatic valvular heart disease. Normally, the mitral va ...
.
General symptoms may also be present, such as:
*
Cough
A cough is a sudden expulsion of air through the large breathing passages which can help clear them of fluids, irritants, foreign particles and Microorganism, microbes. As a protective reflex, coughing can be repetitive with the cough reflex fol ...
*
Pulmonary edema
Pulmonary edema (British English: oedema), also known as pulmonary congestion, is excessive fluid accumulation in the tissue or air spaces (usually alveoli) of the lungs. This leads to impaired gas exchange, most often leading to shortness ...
– as blood backs up into the pulmonary artery, after increased pressures in the left atrium and atrial dilation
*
Hemoptysis
Hemoptysis or haemoptysis is the discharge of blood or blood-stained sputum, mucus through the mouth coming from the bronchi, larynx, vertebrate trachea, trachea, or lungs. It does not necessarily involve coughing. In other words, it is the airw ...
*
Fever
Fever or pyrexia in humans is a symptom of an anti-infection defense mechanism that appears with Human body temperature, body temperature exceeding the normal range caused by an increase in the body's temperature Human body temperature#Fever, s ...
*
Cachexia
Cachexia () is a syndrome that happens when people have certain illnesses, causing muscle loss that cannot be fully reversed with improved nutrition. It is most common in diseases like cancer, Heart failure, congestive heart failure, chronic o ...
– Involuntary weight loss
* General discomfort (
malaise
In medicine, malaise is a feeling of general discomfort, uneasiness or lack of wellbeing and often the first sign of an infection or other disease. It is considered a vague termdescribing the state of simply not feeling well. The word has exist ...
)
*
Joint pain
Arthralgia () literally means 'joint pain'. Specifically, arthralgia is a symptom of injury, infection, illness (in particular arthritis), or an allergic reaction to medication
Medication (also called medicament, medicine, pharmaceutic ...
* Blue discoloration of the skin, especially the fingers change color upon pressure, cold, or stress (
Raynaud's phenomenon
Raynaud syndrome, also known as Raynaud's phenomenon, is a medical condition in which the spasm of small arteries causes episodes of reduced blood flow to end arterioles. Typically the fingers, and, less commonly, the toes, are involved. Rare ...
)
*
Clubbing – Curvature of nails accompanied with soft tissue enlargement of the fingers
* Swelling – any part of the body
*
Presystolic heart murmur[Eric J. Topol]
The Topol Solution: Textbook of Cardiovascular Medicine
Third Edition with DVD, Plus Integrated Content Website, Volume 355. Lippincott Williams & Wilkins, Oct 19, 2006; page 223.
These general symptoms may also mimic those of
infective endocarditis
Infective endocarditis is an infection of the inner surface of the heart (endocardium), usually the heart valve, valves. Signs and symptoms may include fever, petechia, small areas of bleeding into the skin, heart murmur, feeling tired, and anem ...
.
Complications
*
Arrhythmias
Arrhythmias, also known as cardiac arrhythmias, are irregularities in the heartbeat, including when it is too fast or too slow. Essentially, this is anything but normal sinus rhythm. A resting heart rate that is too fast – above 100 beats ...
*
Pulmonary edema
Pulmonary edema (British English: oedema), also known as pulmonary congestion, is excessive fluid accumulation in the tissue or air spaces (usually alveoli) of the lungs. This leads to impaired gas exchange, most often leading to shortness ...
*
Peripheral emboli
* Spread (
metastasis
Metastasis is a pathogenic agent's spreading from an initial or primary site to a different or secondary site within the host's body; the term is typically used when referring to metastasis by a cancerous tumor. The newly pathological sites, ...
) of the tumor
* Blockage of the
mitral heart valve
*
Stroke
Stroke is a medical condition in which poor cerebral circulation, blood flow to a part of the brain causes cell death. There are two main types of stroke: brain ischemia, ischemic, due to lack of blood flow, and intracranial hemorrhage, hemor ...
*
Fusiform cerebral aneurysms
Causes
Myxomata are the most common type of adult primary heart tumor.
Most myxomata arise sporadically (90%), and only about 10% are thought to arise due to inheritance.
About 10% of myxomata are inherited, as in
Carney syndrome. Such tumors are called familial myxomata. They tend to occur in more than one part of the heart at a time, and often cause symptoms at a younger age than other myxomata. Other abnormalities are observed in people with Carney syndrome include skin myxomata, pigmentation, endocrine hyperactivity,
schwannomas and epithelioid blue
nevi.
Myxomata are more common in women than men.
Diagnosis
A doctor will listen to the heart with a
stethoscope
The stethoscope is a medicine, medical device for auscultation, or listening to internal sounds of an animal or human body. It typically has a small disc-shaped resonator that is placed against the skin, with either one or two tubes connected t ...
. A "tumor plop" (a sound related to movement of the tumor), abnormal heart sounds, or a murmur similar to the mid-diastolic rumble of
mitral stenosis
Mitral stenosis is a valvular heart disease characterized by the Stenosis, narrowing of the opening of the mitral valve of the heart. It is almost always caused by Rheumatic Heart Disease, rheumatic valvular heart disease. Normally, the mitral va ...
may be heard. These sounds may change when the patient changes position.
Right atrial myxomata rarely produce symptoms until they have grown to be at least 13 cm (about 5 inches) wide.
Tests may include:
*
Echocardiogram
Echocardiography, also known as cardiac ultrasound, is the use of ultrasound to examine the heart. It is a type of medical imaging, using standard ultrasound or Doppler ultrasound. The visual image formed using this technique is called an echo ...
and
Doppler study
* Chest
x-ray
An X-ray (also known in many languages as Röntgen radiation) is a form of high-energy electromagnetic radiation with a wavelength shorter than those of ultraviolet rays and longer than those of gamma rays. Roughly, X-rays have a wavelength ran ...
*
CT scan
A computed tomography scan (CT scan), formerly called computed axial tomography scan (CAT scan), is a medical imaging technique used to obtain detailed internal images of the body. The personnel that perform CT scans are called radiographers or ...
of chest
* Heart
MRI
Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to generate pictures of the anatomy and the physiological processes inside the body. MRI scanners use strong magnetic fields, magnetic field gradients, and rad ...
* Left heart
angiography
Angiography or arteriography is a medical imaging technique used to visualize the inside, or lumen, of blood vessels and organs of the body, with particular interest in the arteries, veins, and the heart chambers. Modern angiography is perfo ...
* Right heart angiography
*
ECG—may show
atrial fibrillation
Atrial fibrillation (AF, AFib or A-fib) is an Heart arrhythmia, abnormal heart rhythm (arrhythmia) characterized by fibrillation, rapid and irregular beating of the Atrium (heart), atrial chambers of the heart. It often begins as short periods ...
Blood tests:
* Blood tests: An
FBC may show anemia and increased WBCs (white blood cells). The
erythrocyte sedimentation rate
The erythrocyte sedimentation rate (ESR or sed rate) is the rate at which red blood cells in anticoagulated whole blood descend in a standardized tube over a period of one hour. It is a common hematology test, and is a non-specific measure of in ...
(ESR) is usually increased.
File:Atrial myxoma gsr.gif, Echocardiogram of atrial myxoma
File:UOTW 31 - Ultrasound of the Week 1.webm, Echocardiogram showing atrial myxoma
File:UOTW 31 - Ultrasound of the Week 2.jpg, Echocardiogram showing atrial myxoma[
]
Image:Atrial myxoma edge low mag.jpg, Atrial myxoma and myocardium. H&E stain.
Image:Atrial myxoma low mag.jpg, Atrial myxoma. H&E stain.
Image:Atrial myxoma intermed mag.jpg, Atrial myxoma. H&E stain.
Image:Atrial myxoma edge high mag.jpg, Atrial myxoma covered by endothelium
The endothelium (: endothelia) is a single layer of squamous endothelial cells that line the interior surface of blood vessels and lymphatic vessels. The endothelium forms an interface between circulating blood or lymph in the lumen and the r ...
. H&E stain.
Treatment
The surgery is treatment of choice, tumor must be surgically removed. Some patients will also need their
mitral valve
The mitral valve ( ), also known as the bicuspid valve or left atrioventricular valve, is one of the four heart valves. It has two Cusps of heart valves, cusps or flaps and lies between the atrium (heart), left atrium and the ventricle (heart), ...
replaced. This can be done during the same surgery. Usually, inadequate excision of the tumor, development from a secondary focus, or intracardiac implantation from the primary tumor are the attributable explanation for recurrence, and it is more likely to occur in the first 10 postoperative years, especially in younger patients.
Prognosis

Although a myxoma is not malignant with risk of metastasis,
complications are common. Untreated, a myxoma can lead to an
embolism
An embolism is the lodging of an embolus, a blockage-causing piece of material, inside a blood vessel. The embolus may be a blood clot (thrombus), a fat globule (fat embolism), a bubble of air or other gas (air embolism, gas embolism), amniotic ...
(tumor cells breaking off and traveling with the bloodstream). Myxoma fragments can move to the
brain
The brain is an organ (biology), organ that serves as the center of the nervous system in all vertebrate and most invertebrate animals. It consists of nervous tissue and is typically located in the head (cephalization), usually near organs for ...
,
eye
An eye is a sensory organ that allows an organism to perceive visual information. It detects light and converts it into electro-chemical impulses in neurons (neurones). It is part of an organism's visual system.
In higher organisms, the ey ...
, or
limbs.
If the tumor continues to enlarge inside the heart, it can block blood flow through the mitral valve and cause symptoms of
mitral stenosis
Mitral stenosis is a valvular heart disease characterized by the Stenosis, narrowing of the opening of the mitral valve of the heart. It is almost always caused by Rheumatic Heart Disease, rheumatic valvular heart disease. Normally, the mitral va ...
or
mitral regurgitation
Mitral regurgitation (MR), also known as mitral insufficiency or mitral incompetence, is a form of valvular heart disease in which the mitral valve is insufficient and does not close properly when the heart pumps out blood. Section: Valvular He ...
. This may require emergency surgery to prevent sudden death.
See also
*
Myxoma
*
Interleukin 6
Interleukin 6 (IL-6) is an interleukin that acts as both a pro-inflammatory cytokine and an anti-inflammatory myokine. In humans, it is encoded by the ''IL6'' gene.
In addition, osteoblasts secrete IL-6 to stimulate osteoclast formation. Smoo ...
*
Papillary fibroelastoma
*
Rhabdomyomas
*
Cardiac sarcomas
References
External links
{{DEFAULTSORT:Atrial Myxoma
Heart neoplasia