Primary cardiac tumors

Primary cardiac tumors are uncommon and comprise only a small minority of all tumors that involve the heart: most are mediastinal or lung tumors that extend through the pericardium and into the heart, or metastases ¹.

Epidemiology

Primary cardiac tumors have an estimated autopsy prevalence of 0.001-0.03% ². 

Pathology

Primary cardiac tumors can then be divided into:

• benign cardiac tumors: 60-75% ^1,4
  • cardiac myxoma: most common in adults
  • cardiac lipoma (≈10% ⁴, second most common in adults ⁶)
  • cardiac rhabdomyoma: most common in children
  • papillary fibroelastoma
  • cardiac fibroma
  • cardiac hemangioma
  • cardiac paraganglioma
  • pericardial teratoma (can rapidly grow despite being benign) ^7,8
    • fetal pericardial teratoma
• malignant cardiac tumors
  • sarcomas account for 25% of all cardiac tumors ^2,6
    • cardiac angiosarcoma: most common malignant primary cardiac tumor ^3,9
    • undifferentiated sarcoma of the heart
    • cardiac leiomyosarcoma
    • cardiac spindle cell sarcoma
    • cardiac fibrosarcoma
    • cardiac liposarcoma
    • primary cardiac osteosarcoma: 3-9% of primary cardiac malignant tumors ⁹
  • malignant fibrous histiocytoma of heart
  • cardiac hemangiopericytoma
  • primary cardiac lymphoma
  • pericardial mesothelioma