Angiomatoid fibrous histiocytoma: a clinicopathological analysis of ten cases

ZHANG Rong-jun; HUANG Hai-jian; LIU Qiu-yue; ZHENG Zhi-yong

doi:10.3969/j.issn.1007-8096.2018.01.007

Abstract：Objective To investigate the clinicpathological and molecular features of angiomatoid fibrous histiocytoma (AFH).Methods The clinical,histopathological,molecular findings,immunophenotype,treatment and prognosis in 10 cases of AFH were evaluated with review of the relevant literatures.Results 5 cases were male,and 5 were female,aged from 6-76 years (mean 33.7 years).Clinical manifestations were subcutaneous soft tissue mass,cough,or discoverd by physical examination.The tumor size was 1 to 7.5 cm (mean 4.14 cm).Microscopically,the histopathological findings were similar among soft tissue and other sites.The tumors had pseudocapsule with "lymphocyte sheath".Some cases were classic type (6/10),which had solid region and pseudovascular lacunae areas.In solid region,the tumor cells were spindle,round,oval,with beam,sheet or irregular arrangement,and mild atypia.Mitotic fiqures were rare (about ＜ 2/10 HPF),with local visible multinucleated giant cells.At pseudovascular lacunae areas,it can be similar to hemangiomatous and without vascular endothelial cells lined.Some cases were the solid types (4/10),characterized with the solid distribution,no hemangioma-like areas.Immunephenotyping,showed that tumor cells were positive for Vimentin (10/10),a-SMA (3/10),Desmin (8/10),CD68 (5/10),EMA (9/10),CD99 (8/10),and CD34,S100,CD56,CDll7,INI1,ALK1,HMB45,and STAT6 were negative.EWSR1 gene isolation signal was detected in 10 cases by fluorescence in situ hybridization (10/10).Conclusions AFH is a rare aggressive soft tissue tumor and its correct diagnosis relies on histopathologic features,immunohistochemical staining,and gene detection.The primary treatment is completely surgical resection and postoperative long-term follow-up is reserved.