What is cardiac sarcoma?

Updated: Jan 17, 2019
  • Author: John H Raaf, MD, PhD; Chief Editor: Eric H Yang, MD  more...
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Primary cardiac neoplasms are rare entities, [1, 2, 3] with an autopsy prevalence of 0.001-0.28%. The most common primary malignant tumor of the heart and pericardium is sarcoma. With the publication of the 4th edition of the WHO Classification of Tumours of the Lung, Pleura, Thymus and Heart, cardiac sarcomas are now classified as follows [4] :

  • Angiosarcoma
  • Undifferentiated pleomorphic sarcoma
  • Osteosarcoma
  • Myxofibrosarcoma
  • Leiomyosarcoma
  • Rhabdomyosarcoma
  • Synovial sarcoma
  • Miscellaneous sarcomas

Among the changes from the 3rd edition, cardiac sarcomas with a predilection for the left atrium (the most common site in the heart), have been simplified. This group of sarcomas previously included low-grade lesions (fibrosarcoma, myxofibrosarcoma, myxosarcoma, fibromyxosarcoma), and intermediate- to high-grade lesions (myxoid malignant fibrous histiocytoma, malignant fibrous histiocytoma). High-grade sarcomas are now classified as undifferentiated pleomorphic sarcoma to conform with the WHO classification of soft-tissue tumors. The older designations of the lower-grade sarcomas have also been combined under the classification of myxofibrosarcoma. [4]

Another change in classification of cardiac sarcomas is that of tumors with bone matrix formation. In the third edition, sarcomas with osteosarcoma or chondrosarcoma were classified as a subtype of undifferentiated pleomorphic sarcoma. Partly to conform to the WHO classification of soft-tissue tumors, the current edition places these tumors in a separate category of osteosarcoma analogous to extraskeletal osteosarcomas of the soft tissue. [4]

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