What is the pathophysiology of myxofibrosarcoma and undifferentiated pleomorphic sarcoma?

Updated: Jan 17, 2019
  • Author: John H Raaf, MD, PhD; Chief Editor: Eric H Yang, MD  more...
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Answer

Together, this group of tumors that occur primarily in the left atrium account for at least 50% of cardiac sarcomas. A small proportion of undifferentiated pleomorphic sarcomas occur in other chambers, most frequently the left ventricle and right atrium.

Histologically, these tumors are heterogeneous, ranging from bland collagen-rich areas that may in small samples appear benign to low-grade sarcomas with myxoid background to areas of high pleomorphism and mitotic activity (undifferentiated pleomorphic sarcoma). [6]  

These whitish lesions have a firm texture and exhibit infiltrative growth patterns. Myxofibrosarcoma may have a better prognosis than does undifferentiated pleomorphic sarcoma.


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