Angiomatoid Fibrous Histiocytoma with t(2;22)(q33;q12.2) and EWSR1 Gene Rearrangement

Lei Shao; Singh, Vivekanand; Cooley, Linda
March 2009
Pediatric & Developmental Pathology;Mar/Apr2009, Vol. 12 Issue 2, p143
Academic Journal
Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor of low malignant potential. The tumor is mostly seen in the deep dermis and subcutis of the lower extremities in children and young adults. Histologically, the tumor forms lobulated sheets of plump round to spindle cells surrounded by a fibrous pseudocapsule and lymphoid cuff. The cytogenetic and molecular hallmarks of AFH are not well defined. Only 4 of 30 reported cases of AFH have had karyotypic information. We present a case of AFH in the inguinal region of a 12-year-old girl. The tumor showed characteristic histological features, t(2;22)(q33;q12.2), and EWSR gene rearrangement by fluorescence in situ hybridization.


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