Angiomatoid fibrous histiocytoma explained

Angiomatoid fibrous histiocytoma

Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue cancer that affects children and young adults.

Pathology

It is characterized by cystic blood-filled spaces and composed of histiocyte-like cells. A lymphocytic cuff is common. It often simulates a vascular lesion, and was initially described as doing this.[1]

AFH typically has a chromosomal translocation involving the ATF1 gene -- t(12;16) FUS/ATF1 or t(12;22) EWS/ATF1.[2]

See also

Notes and References

  1. Enzinger . FM. . Angiomatoid malignant fibrous histiocytoma: a distinct fibrohistiocytic tumor of children and young adults simulating a vascular neoplasm. . Cancer . 44 . 6 . 2147–57 . Dec 1979 . 10.1002/1097-0142(197912)44:6<2147::aid-cncr2820440627>3.0.co;2-8. 228836 .
  2. Thway. Khin. Fisher. Cyril. 2015-05-01. Angiomatoid Fibrous Histiocytoma: The Current Status of Pathology and Genetics. Archives of Pathology & Laboratory Medicine. en. 139. 5. 674–682. 10.5858/arpa.2014-0234-RA. 0003-9985. 25927151. Further to the characterization of the recurrent chromosomal rearrangements that result in the EWSR1-CREB1, t(12;22)(q13;q12) EWSR1-ATF1, and t(12;16)(q13;p11) FUS-ATF1 gene fusions, AFH is of course now established as a translocation-associated neoplasm.. free.