Table 1

Summary of clinicopathological and molecular characteristics of angiomatoid fibrous histiocytomas

CaseAge(y)/sexSiteSize (cm)Classical featuresStromal changeUnusual patternMitosis (10HPF)Nuclear pleomorphismImmunohistochemistryMolecular studiesTxFollow up
PsAPsCLyCDesminEMACD99
111/MBack1.5++Fibromyxoid7Striking+++++EWSR1-CREB1*SENED, 33 m
212/FScalp5.5++OsC1Moderate+++++EWSR1-CREB1*SENED, 24 m
313/FThigh2+++OsC2Moderate+++++EWSR1-CREB1SENED, 4 m
433/MScalp2.6+++Sclerosing2Striking++++EWSR1-CREB1SENED, 1 m
521/FLeg4+++Sclerosing5ModerateNDNDNDEWSR1-CREB1SENED, 18 m
617/MThigh3+++66Moderate†++EWSR1-CREB1SENA
718/MBack1.2++Fibromyxoid1Moderate++NDEWSR1-CREB1‡SENA
84/FBack1.5+++13Absent+++++EWSR1-CREB1SENA
959/FElbow1.6++MyxoidReticular0Absent++++++++EWSR1-ATF1‡*SENED, 8 m
1063/MElbow1.5+++SclerosingHyV, palisading1Moderate+++++EWSR1-CREB1‡SENA
119/FThigh2.2+++Nested2Absent++++Non-informativeWENED, 108 m
125/MScalp3.2+++0Absent++++++SENED, 43 m
135/MAbdominal wall0.6++Fibromyxoid0Moderate+Non-informativeSENED, 61 m
  • *Confirmed by direct sequencing

  • †Concomitant with areas of small blue round cells.

  • EWSR1 rearrangement by fluorescence in situ hybridisation (FISH).

  • HyV, hyalinised vessels; LyC, lymphoplasmacytic cuffing; NA, not available; NED, no evidence of disease; ND, not done; OsC, osteoclast-like cells; PsA, pseudoangiomatoid spaces; PsC, pseudocapsule; SE, simple excision; Tx, treatment; WE, wide excision.