[Durgical](https://durgical.com/en/) › [DurgiPedia](https://durgical.com/en/durgipedia/) › [Conditions](https://durgical.com/en/conditions/) › Cardiac tumours (atrial myxoma)

[Conditions](https://durgical.com/en/conditions/) · [Cardiology](https://durgical.com/en/conditions/)

# Cardiac tumours (atrial myxoma)

A benign, jelly-like tumour inside the heart, usually in the [left atrium](https://durgical.com/en/anatomy/left-atrium/). It can block blood flow or shed clots and is removed by surgery.

Updated 2026-11-01

## Structures involved

Tap one to see what it is and open it in 3D.

- [Left atrium](https://durgical.com/en/anatomy/left-atrium/)
- [Interatrial septum](https://durgical.com/en/anatomy/interatrial-septum/)
- [Mitral anterior leaflet](https://durgical.com/en/anatomy/anterior-leaflet-of-left-atrioventricular-valve/)
- [Mitral posterior leaflet](https://durgical.com/en/anatomy/posterior-leaflet-of-left-atrioventricular-valve/)
- [Right atrium](https://durgical.com/en/anatomy/right-atrium/)
- [Left ventricle](https://durgical.com/en/anatomy/left-ventricle/)
- [Pulmonary veins](https://durgical.com/en/anatomy/pulmonary-veins/)

## Why it happens

- [Benign vs malignant tumours: the comparison table](https://durgical.com/en/general-pathology/benign-vs-malignant-tumours-the-comparison-table/)
- [Systemic (arterial) thromboembolism](https://durgical.com/en/general-pathology/systemic-thromboembolism/)
- [Murmurs and the hemodynamics of valve disease](https://durgical.com/en/physiology/murmurs-and-the-hemodynamics-of-valve-disease/)

## What it is

Primary cardiac tumours are rare; metastases to the heart are far more common. Myxoma is the most common primary cardiac tumour in adults: a soft, gelatinous, usually pedunculated mass, most often in the left atrium attached by a stalk to the interatrial septum near the fossa ovalis. Though benign, it can obstruct the mitral valve, embolise and cause systemic inflammatory symptoms.

## Who gets it

Primary cardiac tumours are found in well under 0.1% of autopsies, and about half of benign primary tumours in adults are myxomas. Myxomas are most common in women between 30 and 60 years. About 7–10% are familial, most often as part of Carney complex. In children the most common primary cardiac tumour is rhabdomyoma, linked to tuberous sclerosis.

## Causes

- Sporadic myxoma: arises from multipotent mesenchymal cells in the endocardium, usually near the fossa ovalis
- Familial myxoma in Carney complex: autosomal dominant, usually variants in PRKAR1A
- Other primary tumours: papillary fibroelastoma (valves), rhabdomyoma and fibroma (children), lipoma, angiosarcoma (most common primary malignant tumour)
- Secondary tumours (most common overall): metastases from lung, breast, melanoma, lymphoma and leukaemia

## Risk factors

- Female sex and middle age (sporadic myxoma)
- Family history of myxoma or Carney complex
- Tuberous sclerosis (rhabdomyoma in infants)
- Advanced cancer (cardiac metastases)

## Pathophysiology

Myxomas cause disease by three mechanisms. Obstruction: a mobile left atrial tumour prolapses into the mitral orifice in diastole, mimicking mitral stenosis and raising left atrial and pulmonary pressures; symptoms may change with posture. Embolism: friable fragments or overlying thrombus travel to the brain, limbs, [kidneys](https://durgical.com/en/anatomy/kidney/) or coronary arteries (right-sided tumours embolise to the lungs). Constitutional effects: the tumour secretes interleukin-6, causing fever, weight loss, arthralgia, anaemia and raised inflammatory markers.

## Classification

- Primary benign: myxoma, papillary fibroelastoma, rhabdomyoma, fibroma, lipoma
- Primary malignant: sarcomas (angiosarcoma most common), primary cardiac lymphoma
- Secondary: metastases by direct, lymphatic or haematogenous spread
- Myxoma by location: left atrium (about 75%), [right atrium](https://durgical.com/en/anatomy/right-atrium/), ventricles (more often in Carney complex)

## Symptoms and signs

- Dyspnoea, orthopnoea and pulmonary oedema (mitral obstruction)
- Dizziness or syncope, sometimes positional
- Sudden neurological deficit or limb ischaemia (embolism)
- Fever, weight loss, fatigue and arthralgia
- May be asymptomatic and found incidentally on echocardiography

## Red flags

- Stroke or transient ischaemic attack in a young person without vascular risk factors
- Syncope or sudden pulmonary oedema with a changing murmur
- Embolic limb ischaemia
- Mobile intracardiac mass on echocardiography (risk of embolism and sudden death)

## Diagnosis

**Clinical**
Signs resembling mitral stenosis that vary with position; an early diastolic «tumour plop» after S2; constitutional symptoms. In Carney complex look for spotty [skin](https://durgical.com/en/anatomy/skin/) pigmentation (lentigines), cutaneous myxomas and endocrine overactivity (Cushing syndrome, acromegaly).

**Tests**
Raised erythrocyte sedimentation rate and C-reactive protein, anaemia, hypergammaglobulinaemia. ECG is nonspecific. Histology after excision confirms the diagnosis; percutaneous biopsy is avoided because of embolic risk. Genetic testing and family screening when Carney complex is suspected.

**Imaging**
Transthoracic echocardiography is the first test; transoesophageal echocardiography defines the attachment site and size. Cardiac MRI characterises tissue and separates tumour from thrombus; CT helps plan surgery and look for metastatic disease.

## Differential diagnosis

- Left atrial thrombus (usually in the appendage, with atrial fibrillation or mitral stenosis)
- Rheumatic mitral stenosis
- Infective endocarditis with large vegetations (fever, emboli)
- Other cardiac tumours, including metastases
- Vasculitis or connective tissue disease (constitutional symptoms)

## Goal of treatment

Remove the tumour promptly to prevent embolism, obstruction and sudden death, then follow for recurrence.

## Treatment

**Surgical**
Prompt surgical excision on cardiopulmonary bypass, removing the stalk with a margin of the [interatrial septum](https://durgical.com/en/anatomy/interatrial-septum/) and repairing the defect with a patch; the valve is repaired if damaged.

**Acute**
Emergency management of complications while surgery is arranged: stroke or limb ischaemia pathways, treatment of pulmonary oedema; surgery is not delayed once the diagnosis is made.

**Follow-up**
Echocardiographic follow-up for recurrence (more frequent in familial and multiple tumours); screening of first-degree relatives in Carney complex. Nursing after surgery: rhythm monitoring for atrial arrhythmias, neurological checks and wound care.

## Complications

- Ischaemic stroke and other systemic emboli
- Sudden death from complete obstruction of the mitral orifice
- Heart failure and pulmonary hypertension
- Arrhythmias
- Recurrence after excision, mainly in Carney complex

## Prognosis

After complete excision of a sporadic myxoma the prognosis is excellent and recurrence is uncommon. Familial myxomas recur more often and need lifelong surveillance. Malignant primary tumours and metastases have a poor prognosis.

## Prevention

- No primary prevention; family screening and surveillance in Carney complex
- Early echocardiography in young patients with unexplained stroke or emboli

## Test yourself

Think of the answer, then tap the question to see it.

## Where is the classic atrial myxoma located?

In the left atrium, attached by a stalk to the interatrial septum near the fossa ovalis.

## What auscultatory sound is described with a mobile myxoma?

An early diastolic «tumour plop».

## Which cytokine produced by myxomas explains fever and raised inflammatory markers?

Interleukin-6.

## What is the most common cardiac tumour overall?

Metastasis to the heart.

## What is the most common primary cardiac tumour in children, and with which syndrome?

Rhabdomyoma, associated with tuberous sclerosis.

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## Related conditions

- [Mitral stenosis](https://durgical.com/en/conditions/mitral-stenosis/)
- [Ischaemic stroke](https://durgical.com/en/conditions/ischaemic-stroke/)
- [Transient ischaemic attack](https://durgical.com/en/conditions/transient-ischaemic-attack/)
- [Syncope (vasovagal, orthostatic hypotension, cardiac)](https://durgical.com/en/conditions/syncope/)
- [Heart failure](https://durgical.com/en/conditions/heart-failure/)
- [Atrial fibrillation](https://durgical.com/en/conditions/atrial-fibrillation/)
- [Infective endocarditis](https://durgical.com/en/conditions/infective-endocarditis/)
- [Pulmonary embolism](https://durgical.com/en/conditions/pulmonary-embolism/)
- [Acute limb ischaemia](https://durgical.com/en/conditions/acute-limb-ischaemia/)
- [Cushing syndrome](https://durgical.com/en/conditions/cushing-syndrome/)
- [Pulmonary hypertension and cor pulmonale](https://durgical.com/en/conditions/pulmonary-hypertension-and-cor-pulmonale/)

Educational information, not medical advice. For any symptom, see a health professional.

Sources: [Atrial myxoma](https://medlineplus.gov/ency/article/007273.htm) (MedlinePlus, US National Library of Medicine, 2026); [Carney complex](https://medlineplus.gov/genetics/condition/carney-complex/) (MedlinePlus Genetics, US National Library of Medicine, 2026).

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Source: https://durgical.com/en/conditions/cardiac-tumours/
License: CC BY 4.0 (https://creativecommons.org/licenses/by/4.0/). Cite as: DurgiPedia (durgical.com), https://durgical.com/en/conditions/cardiac-tumours/
