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| {{Infobox medical condition (new) | | {{Short description|A type of primary heart tumor}} |
| | name = Atrial myxoma | | {{Use dmy dates|date=October 2023}} |
| | image = Atrial myxoma high mag.jpg
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| | caption = [[Micrograph]] of an '''atrial myxoma'''. [[H&E stain]].
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| A '''myxoma''' is a rare [[Benign tumor|benign]] [[tumor]] of the [[human heart|heart]]. Myxomas are the most common primary cardiac tumor in adults, and are most commonly found within the left atrium. Myxomas may also develop in the other heart chambers.<ref name=":0">{{Cite journal|last=Hecht|first=Sisalee M.|date=2009-10-27|title=A Review of: "Current Diagnosis & Treatment: Cardiology. 3rd ed. Crawford, Michael H., ed."|journal=Medical Reference Services Quarterly|volume=28|issue=4|pages=401–402|doi=10.1080/02763860903249993|issn=0276-3869}}</ref> The tumor is derived from multipotent mesenchymal cells.<ref name=":0" />
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| ==Causes== | | ==Cardiac Myxoma== |
| Myxomas are the most common type of adult primary heart tumor.<ref name=":0" /><ref name="pmid18350919">{{Cite journal|last1=Vaideeswar|first1=P.|last2=Butany|first2=JW.|date=Feb 2008|title=Benign cardiac tumors of the pluripotent mesenchyme.|journal=Semin Diagn Pathol|volume=25|issue=1|pages=20–8|doi=10.1053/j.semdp.2007.10.005|pmid=18350919}}</ref> Most myxomas arise sporadically (90%), and only about 10% are thought to arise due to inheritance.<ref>{{Cite journal|last=Masters|first=Barry R.|date=2012-05-25|title=Harrisons's Principles of Internal Medicine, 18th Edition, two volumes and DVD. Eds: Dan L. Longo, Anthony S. Fauci, Dennis L. Kasper, Stephen L. Hauser, J. Larry Jameson and Joseph Loscalzo, ISBN 9780071748896 McGraw Hill|journal=Graefe's Archive for Clinical and Experimental Ophthalmology|volume=250|issue=9|pages=1407–1408|doi=10.1007/s00417-012-1940-9|issn=0721-832X}}</ref>
| | A '''cardiac myxoma''' is a type of [[primary heart tumor]] that is typically benign. It is the most common type of primary cardiac tumor in adults, accounting for approximately 50% of all such tumors. Myxomas are usually found in the [[left atrium]] of the heart, although they can occur in other chambers as well. |
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| About 10% of myxomas are inherited, as in [[Carney syndrome]]. Such tumors are called familial myxomas. 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 myxomas. Other abnormalities are observed in people with Carney syndrome include skin myxomas, pigmentation, endocrine hyperactivity, [[schwannoma]]s and epithelioid blue [[Nevus|nevi]].<ref name=":0" /> Myxomas are more common in women than men.<ref name=":0" /><ref name=":1">{{Cite journal|last=Aiello|first=Vera Demarchi|last2=Campos|first2=Fernando Peixoto Ferraz de|date=2016|title=Cardiac Myxoma|journal=Autopsy and Case Reports|volume=6|issue=2|pages=5–7|doi=10.4322/acr.2016.030|issn=2236-1960|pmc=4982778|pmid=27547737}}</ref>
| | ==Pathophysiology== |
| | Cardiac myxomas are gelatinous tumors that arise from the [[endocardium]], the inner lining of the heart. They are composed of a myxoid stroma, which is a loose connective tissue, and are often attached to the endocardium by a stalk. The tumors can vary in size and may be pedunculated or sessile. |
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| ==Signs and symptoms== | | ==Clinical Presentation== |
| Symptoms may occur at any time, but most often they accompany a change of body position. Pedunculated myxomas can have a "wrecking ball effect", as they lead to stasis and may eventually embolize themselves. Symptoms may include:
| | Patients with cardiac myxoma may present with a variety of symptoms, which can be grouped into three categories: obstructive, embolic, and constitutional. |
| * [[Dyspnea|Shortness of breath]] with activity
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| * [[Platypnea]] – Difficulty breathing in the upright position with relief in the supine position
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| * [[Cardiac asthma|Paroxysmal nocturnal dyspnea]] – Breathing difficulty when asleep
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| * [[Presyncope|Dizziness]]
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| * [[Syncope (medicine)|Fainting]]
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| * [[Palpitation]]s – Sensation of feeling your heart beat
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| * Chest pain or tightness
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| * Sudden Death (In which case the disease is an autopsy finding)
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| The symptoms and signs of left atrial myxomas often mimic [[mitral stenosis]].
| | ===Obstructive Symptoms=== |
| General symptoms may also be present, such as:
| | Obstructive symptoms occur when the myxoma interferes with normal blood flow through the heart. This can lead to symptoms such as [[dyspnea]], [[orthopnea]], and [[paroxysmal nocturnal dyspnea]]. In some cases, the tumor may cause [[syncope]] or even sudden cardiac death if it obstructs blood flow significantly. |
| * [[Cough]]
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| * [[Pulmonary edema]], as blood backs up into the pulmonary artery, after increased pressures in the left atrium and atrial dilation
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| * [[Hemoptysis]]
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| * [[Fever]]
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| * [[Cachexia]] – Involuntary weight loss
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| * General discomfort ([[malaise]])
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| * [[Arthralgia|Joint pain]]
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| * Blue discoloration of the skin, especially the fingers change color upon pressure, cold, or stress ([[Raynaud's phenomenon]])
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| * [[Nail clubbing|Clubbing]] – Curvature of nails accompanied with soft tissue enlargement of the fingers
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| * Swelling – any part of the body
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| * [[Presystolic murmur|Presystolic heart murmur]]<ref name=topol>Eric J. Topol. [https://books.google.com/books?id=35zSLWyEWbcC 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. {{ISBN|0781770122}}</ref>
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| These general symptoms may also mimic those of [[infective endocarditis]].
| | ===Embolic Symptoms=== |
| | Embolic symptoms occur when fragments of the tumor break off and travel through the bloodstream, potentially causing [[stroke]] or other [[embolism|embolic events]]. This is more common when the myxoma is located in the left atrium, as emboli can travel to the [[systemic circulation]]. |
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| ==Complications== | | ===Constitutional Symptoms=== |
| | | Constitutional symptoms are systemic symptoms that may include [[fever]], [[weight loss]], and [[malaise]]. These symptoms are thought to be due to the release of [[cytokines]] by the tumor. |
| * [[Cardiac dysrhythmia|Arrhythmias]]
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| * [[Pulmonary edema]]
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| * [[Peripheral emboli]]
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| * Spread ([[metastasis]]) of the tumor
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| * Blockage of the [[Mitral valve|mitral heart valve]]
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| * [[Stroke]]
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| * [[Fusiform cerebral aneurysms]]
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| ==Diagnosis== | | ==Diagnosis== |
| A doctor will listen to the heart with [[stethoscope]]. 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]] may be heard. These sounds may change when the patient changes position.
| | The diagnosis of cardiac myxoma is typically made using [[echocardiography]], which can visualize the tumor within the heart. [[Transesophageal echocardiography]] provides a more detailed view and is often used to confirm the diagnosis. Other imaging modalities, such as [[MRI]] or [[CT scan]], may also be used. |
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| Right atrial myxomas rarely produce symptoms until they have grown to be at least 13 cm (about 5 inches) wide.
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| Tests may include:
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| * [[Echocardiogram]] and [[Doppler echocardiography|Doppler study]]
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| * Chest [[x-ray]]
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| * [[CT scan]] of chest
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| * Heart [[MRI]]
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| * Left heart [[angiography]]
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| * Right heart angiography
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| * [[ECG]]—may show [[atrial fibrillation]]
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| Blood tests:
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| A [[complete blood count|FBC]] may show anemia and increased WBCs (white blood cells). The [[erythrocyte sedimentation rate]] (ESR) is usually increased.
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| <gallery>
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| File:Atrial myxoma gsr.gif|Echocardiogram of atrial myxoma
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| File:UOTW 31 - Ultrasound of the Week 1.webm|Echocardiogram showing atrial myxoma<ref name=UOTW31>{{cite web|title=UOTW #31 - Ultrasound of the Week|url=https://www.ultrasoundoftheweek.com/uotw-31/|website=Ultrasound of the Week|accessdate=27 May 2017|date=30 December 2014}}</ref>
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| File:UOTW 31 - Ultrasound of the Week 2.jpg|Echocardiogram showing atrial myxoma<ref name=UOTW31/>
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| </gallery>
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| <gallery>
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| Image:Atrial myxoma edge low mag.jpg|Atrial myxoma and myocardium. H&E stain.
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| Image:Atrial myxoma low mag.jpg|Atrial myxoma. H&E stain.
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| Image:Atrial myxoma intermed mag.jpg|Atrial myxoma. H&E stain.
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| Image:Atrial myxoma edge high mag.jpg|Atrial myxoma covered by [[endothelium]]. H&E stain.
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| </gallery>
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| ==Treatment== | | ==Treatment== |
| The tumor must be surgically removed. Some patients will also need their [[mitral valve]] replaced. This can be done during the same surgery. | | The primary treatment for cardiac myxoma is surgical resection. This involves removing the tumor and a portion of the surrounding endocardium to prevent recurrence. Surgery is usually curative, and the prognosis after resection is generally excellent. |
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| Myxomas may come back if surgery did not remove all of the tumor cells.
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| ==Prognosis== | | ==Prognosis== |
| [[File:Atrial myxoma embolus.jpg|thumb|An embolized fragment of an atrial myxoma in the iliac bifurcation.]]
| | The prognosis for patients with cardiac myxoma is generally good following surgical removal. Recurrence is rare but can occur, particularly if the tumor is not completely excised. Regular follow-up with echocardiography is recommended to monitor for recurrence. |
| Although a myxoma is not malignant with risk of metastasis,<ref name=":1" /> complications are common. Untreated, a myxoma can lead to an [[embolism]] (tumor cells breaking off and traveling with the bloodstream). Myxoma fragments can move to the [[human brain|brain]], [[human eye|eye]], or [[Limb (anatomy)|limbs]].
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| If the tumor continues to enlarge inside the heart, it can block blood flow through the mitral valve and cause symptoms of [[mitral stenosis]] or [[Mitral insufficiency|mitral regurgitation]]. This may require emergency surgery to prevent sudden death.<ref>{{Cite web | url=http://www.jcecho.org/article.asp?issn=2211-4122;year=2018;volume=28;issue=1;spage=59;epage=60;aulast=Barik |title = A Biatrial Myxoma with Triple Ripples}}</ref>
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| ==See also==
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| * [[Myxoma]]
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| * [[Interleukin 6]]
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| * [[Papillary fibroelastoma]]
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| * [[Rhabdomyoma]]s
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| * [[Cardiac sarcoma]]s
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| ==References==
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| {{Reflist}}
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| == External links == | | ==Related Pages== |
| {{Medical resources
| | * [[Heart disease]] |
| | DiseasesDB = 1075
| | * [[Cardiac tumor]] |
| | ICD10 =
| | * [[Echocardiography]] |
| | ICD9 =
| | * [[Surgical oncology]] |
| | ICDO =
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| | OMIM =
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| | MedlinePlus = 007273
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| }}
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| {{Heart and thymus neoplasia}}
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| {{DEFAULTSORT:Atrial Myxoma}}
| | [[Category:Cardiac tumors]] |
| [[Category:Heart neoplasia]] | | [[Category:Heart diseases]] |
| {{dictionary-stub1}}
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A type of primary heart tumor
Cardiac Myxoma
A cardiac myxoma is a type of primary heart tumor that is typically benign. It is the most common type of primary cardiac tumor in adults, accounting for approximately 50% of all such tumors. Myxomas are usually found in the left atrium of the heart, although they can occur in other chambers as well.
Pathophysiology
Cardiac myxomas are gelatinous tumors that arise from the endocardium, the inner lining of the heart. They are composed of a myxoid stroma, which is a loose connective tissue, and are often attached to the endocardium by a stalk. The tumors can vary in size and may be pedunculated or sessile.
Clinical Presentation
Patients with cardiac myxoma may present with a variety of symptoms, which can be grouped into three categories: obstructive, embolic, and constitutional.
Obstructive Symptoms
Obstructive symptoms occur when the myxoma interferes with normal blood flow through the heart. This can lead to symptoms such as dyspnea, orthopnea, and paroxysmal nocturnal dyspnea. In some cases, the tumor may cause syncope or even sudden cardiac death if it obstructs blood flow significantly.
Embolic Symptoms
Embolic symptoms occur when fragments of the tumor break off and travel through the bloodstream, potentially causing stroke or other embolic events. This is more common when the myxoma is located in the left atrium, as emboli can travel to the systemic circulation.
Constitutional Symptoms
Constitutional symptoms are systemic symptoms that may include fever, weight loss, and malaise. These symptoms are thought to be due to the release of cytokines by the tumor.
Diagnosis
The diagnosis of cardiac myxoma is typically made using echocardiography, which can visualize the tumor within the heart. Transesophageal echocardiography provides a more detailed view and is often used to confirm the diagnosis. Other imaging modalities, such as MRI or CT scan, may also be used.
Treatment
The primary treatment for cardiac myxoma is surgical resection. This involves removing the tumor and a portion of the surrounding endocardium to prevent recurrence. Surgery is usually curative, and the prognosis after resection is generally excellent.
Prognosis
The prognosis for patients with cardiac myxoma is generally good following surgical removal. Recurrence is rare but can occur, particularly if the tumor is not completely excised. Regular follow-up with echocardiography is recommended to monitor for recurrence.
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