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PH-CV09

Tumours of the heart

Why metastases are by far the commonest heart tumours, how a benign left atrial myxoma obstructs, embolises and causes fever, and how fibroelastoma, rhabdomyoma and angiosarcoma differ, with tumour in the pericardium causing tamponade.

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  • 5 steps · 10 questions
  • 3D normal anatomy

By the end, you can

  • Explain why secondary tumours of the heart are far commoner than primary ones, and describe the routes by which they reach the pericardium and heart.
  • Describe the site, gross and microscopic appearance of a cardiac myxoma and relate it to obstruction, embolism and constitutional symptoms.
  • Compare papillary fibroelastoma, rhabdomyoma and fibroma, including the link between rhabdomyoma and tuberous sclerosis.
  • Explain how angiosarcoma and pericardial tumour cause a pericardial effusion and tamponade, and what grading and staging record for heart tumours.
  • Recognise the classic appearances, classification and imaging of heart tumours, and explain how excision, mTOR inhibitors, pericardial drainage and cancer treatment act on each.

What it is

Tumours of the heart are mostly secondary: deposits of cancers that began elsewhere, usually in the pericardium. Primary heart tumours are rare and most are benign (myxoma in adults, papillary fibroelastoma on valves, rhabdomyoma in children); the commonest primary malignancy in adults is angiosarcoma. Because of where they sit, even benign tumours can obstruct blood flow, embolise or disturb the rhythm.

5 types to tell apart
Metastases
Spread from lung, breast, melanoma, lymphoma and others; most often to the pericardium and epicardium.
Myxoma
The commonest primary tumour in adults; a gelatinous, stalked mass usually in the left atrium.
Papillary fibroelastoma
A small frond-like tumour on a valve surface; its main risk is embolism.
Rhabdomyoma
The commonest heart tumour of infants; a hamartoma of the ventricles, usually with tuberous sclerosis, that often regresses.
Angiosarcoma
The commonest primary malignant heart tumour in adults; a haemorrhagic, invasive tumour of the right atrium.

US spelling: tumours → tumors, centre → center, oesophagus → esophagus.

Normal function

Heart wall, chambers and pericardium

The heart wall has three layers: the endocardium lining the chambers and valves, the myocardium of contracting muscle cells, and the epicardium on the surface, which is the inner (visceral) layer of the serous pericardium. The encloses a thin film of fluid inside the tough, inelastic .

Adult cardiac muscle cells almost never divide, which is one reason primary heart tumours are so rare. The cells that can give rise to tumours are endothelial cells, fibroblasts, fat cells, the mesothelium of the pericardium and primitive mesenchymal cells beneath the endocardium. The receives blood from the lungs and empties through the mitral valve into the ; the two atria are separated by the , whose thin centre is the .

The heart has a rich blood supply and is drained by lymphatics that run to the mediastinal lymph nodes, beside the lungs and oesophagus. Both are routes by which cancers reach it.

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