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.
EnzingerFM. Angiomatoid malignant fibrous histiocytoma: a distinct fibrohistiocytic tumor of children and young adults simulating a vascular neoplasm. Cancer1979;44:2147–2157.
2.
CostaMJWeissSW. Angiomatoid malignant fibrous histiocytoma. A follow-up study of 108 cases with evaluation of possible histologic predictors of outcome. Am J Surg Pathol1990;14:1126–1132.
3.
Fanburg-SmithJCMiettinenM. Angiomatoid “malignant” fibrous histiocytoma: a clinicopathologic study of 158 cases and further exploration of the myoid phenotype. Hum Pathol1999;30:1336–1343.
4.
FletcherCDMUnniKMertensK. WHO Classification of Tumours: Pathology and Genetics of Soft Tissue and Bone. Lyon, France: IARC Press, 2002.
5.
SmithMECostaMJWeissSW. Evaluation of CD68 and other histiocytic antigens in angiomatoid malignant fibrous histiocytoma. Am J Surg Pathol1991;15:757–763.
6.
RossiSSzuhaiKIjszengaM. EWSR1-CREB1 and EWSR1-ATF1 fusion genes in angiomatoid fibrous histiocytoma. Clin Cancer Res2007;13:7322–7328.
7.
WatersBLPanagopoulosIAllenEF. Genetic characterization of angiomatoid fibrous histiocytoma identifies fusion of the FUS and ATF-1 genes induced by a chromosomal translocation involving bands 12q13 and 16p11. Cancer Genet Cytogenet2000;121:109–116.
8.
HallorKHMertensFJinY. Fusion of the EWSR1 and ATF1 genes without expression of the MITF-M transcript in angiomatoid fibrous histiocytoma. Genes Chrom Cancer2005;44:97–102.
9.
AntonescuCRDal CinPNafaK. EWSR1-CREB1 is the predominant gene fusion in angiomatoid fibrous histiocytoma. Genes Chrom Cancer2007;46:1051–1060.
10.
SomersGRVieroSNathanPCTeshimaIPereiraCZielenskaM. Association of the t(12;22)(q13;q12) EWS/ATF1 rearrangement with polyphenotypic round cell sarcoma of bone: a case report. Am J Surg Pathol2005;29:1673–1679.
11.
RaddaouiEDonnerLRPanagopoulosI. Fusion of the FUS and ATF1 genes in a large, deep-seated angiomatoid fibrous histiocytoma. Diagn Mol Pathol2002;11:157–162.
12.
HallorKHMicciFMeis-KindblomJM. Fusion genes in angiomatoid fibrous histiocytoma. Cancer Lett2007;18; 251:158–163.
13.
DunhamCHussongJSeiffMPfeiferJPerryA. Primary intracerebral angiomatoid fibrous histiocytoma: report of a case with a t(12;22)(q13;q12) causing type 1 fusion of the EWS and ATF-1 genes. Am J Surg Pathol2008;32:478–484.
14.
ThwayK. Angiomatoid fibrous histiocytoma: a review with recent genetic findings. Arch Pathol Lab Med2008;132:273–277.
15.
AntonescuCRNafaKSegalNHDal CinPLadanyiM. EWS-CREB1: a recurrent variant fusion in clear cell sarcoma—association with gastrointestinal location and absence of melanocytic differentiation. Clin Cancer Res2006;12:5356–5362.
16.
HisaokaMIshidaTKuoTT. Clear cell sarcoma of soft tissue: a clinicopathologic, immunohistochemical, and molecular analysis of 33 cases. Am J Surg Pathol2008;32:452–460.
17.
SandbergAABridgeJA. Updates on the cytogenetics and molecular genetics of bone and soft tissue tumors: clear cell sarcoma (malignant melanoma of soft parts). Cancer Genet Cytogenet2001;130:1–7.
18.
SandbergAA. Updates on the cytogenetics and molecular genetics of bone and soft tissue tumors: liposarcoma. Cancer Genet Cytogenet2004;155:1–24.
