Review Article

Pediatric Sclerosing Rhabdomyosarcomas: A Review

Table 2

Histopathological, immunohistochemical, and genetic features of 21 cases of pediatric sclerosing rhabdomyosarcomas.

YearAuthorAge/
Sex
Vimentin DesminMyogenin (myf4)MyoD1 SMACytokeratinS100CD99Amount of fibrosisRhabdo-myoblastsMitosesPAX3/FXHR by RT-PCRKaryotype

2002Folpe et al. [8]18/M NAFocally (+)Focally (+)Strongly (+) 3+ Neg. Neg. Neg.Abundant (−)>25/20 HPF NA NA

2004Vadgama et al. [9]3/FStrongly (+)Strongly (+)Focally (+)Strongly (+)Focally (+) Neg. Neg.Focally (+) Dense (−)32/100 HPF Neg. NA

2004Chiles et al. [7] 6/M NA Pos. Pos. Pos. NA NA NA NA >50% (−) NA NA NA
11/M NA NA NA NA NA NA NA NA 10–50% (−) NA Neg. CK
7/F NAFocally (+)Focally (+)Strongly (+) NA NA NA NA >50% (−) NA NA NA
10/F NAStrongly (+)Focally (+)Strongly (+) NA NA NA NA >50% (−) NA Neg. NA
3/M NAStrongly (+)Focally (+)Strongly (+) NA NA NA NA >50% (−) NA Pos. NA
8/F NAStrongly (+)Focally (+)Strongly (+) NA NA NA NA >50% (−) NA NA NA
16/F NAFocally (+)Focally (+)Strongly (+) NA NA NA NA >50% (−) NA Neg. NA
4/F NAStrongly (+)Strongly (+)Strongly (+) NA NA NA NA 10–50% (−) NA NA NA
12/M NAStrongly (+)Focally (+)Strongly (+) NA NA NA NA >50% (−) NA Neg. NA
9/M NAFocally (+)Strongly (+)Strongly (+) NA NA NA NA 10–50% (−) NA NA CK
4/M NAStrongly (+)Strongly (+)Strongly (+) NA NA NA NA 10–50% (−) NA NA NA
0.3/M NAFocally (+)Strongly (+)Strongly (+) NA NA NA NA 10–50% (−) NA NA NA
5/M NAStrongly (+)Focally (+)Focally (+) NA NA NA NA >50% (+) NA Neg. NA

2006 Zambrano et al. [11]8/F NAStrongly (+)Focal (+)Focally (+)Focally (+) NA Neg. Neg.Abundant (−)Numerous Neg. NK
18/M NAStrongly (+)Weakly (+)Strongly (+)Strongly (+) NA Neg.Weakly (+)Abundant (−)Numerous Neg. CK
17/F NAFocally (+)Focally (+)Focally (+)Focally (+) NA Neg.Weakly (+)Abundant (+)Numerous Neg.CK

2008Wang et al. [10]12/FStrongly (+)Focally (+)Focally (+)Strongly (+)Focally (+) NA Pos. NAAbundant (−)5/10 HPF NA NA

2009Bouron-Dal Soglio et al. [6]7/M NAStrongly (+)Focally (+) NA NA NA NA NAAbundant (−) NA Neg. CK

2013Present case11/MStrongly (+)Strongly (+)Focal (+)Not done Neg. Neg.Neg. Neg.Abundant (−) NANot doneNot done

NA: not available; Neg.: negative; Pos.: positive; HPF: high power field; CK: complex karyotype; NK: normal karyotype.