THE EXPRESSION PATTERN OF CONTRACTILE AND INTERMEDIATE FILAMENT PROTEINS IN DEVELOPING SKELETAL-MUSCLE AND RHABDOMYOSARCOMA OF CHILDHOOD - DIAGNOSTIC AND PROGNOSTIC UTILITY
Lcd. Wijnaendts et al., THE EXPRESSION PATTERN OF CONTRACTILE AND INTERMEDIATE FILAMENT PROTEINS IN DEVELOPING SKELETAL-MUSCLE AND RHABDOMYOSARCOMA OF CHILDHOOD - DIAGNOSTIC AND PROGNOSTIC UTILITY, Journal of pathology, 174(4), 1994, pp. 283-292
In order to investigate whether rhabdomyosarcoma (RMS) can be related
to equivalent stages of skeletal muscle development, muscle tissue of
21 human foetuses and 112 primary RMSs were characterized immunohistoc
hemically using antibodies directed against vimentin, desmin, muscle-s
pecific actin (HHF35), sarcomeric actin (sr-actin), smooth muscle acti
n (sm-actin), and troponin-T. During fetal skeletal muscle development
, all myotubes/fibres of the first and second generations expressed de
smin, HHF35, and sr-actin. Vimentin was almost exclusively present in
immature primary and secondary myotubes/fibres. Troponin-T was express
ed in immature myotubes/fibres of the first and second generations as
well as mature fibres of the second generation. Sm-actin was never exp
ressed. Vimentin was expressed in 96 per cent of primary and 98 per ce
nt of relapsed RMS; HHF35 in 96 and 98 per cent, respectively; desmin
in 95 and 100 per cent; troponin-T in 82 and 75 per cent; sr-actin in
71 and 86 per cent; and sm-actin in 13 and 17 per cent. The proportion
of RMS cells reacting with vimentin, HHF35, and desmin was consistent
ly higher than those expressing sr-actin and troponin-T. Neither the s
hape nor size of neoplastic RMS cells nor the histopathological types
were related to the expression pattern of the investigated markers. RM
S with aberrant expression of two or more markers predicted a worse pr
ognosis than RMS in which at most one marker was aberrantly expressed
(25 per cent and 54 per cent 10-year survival, P=0.01). These results
demonstrate that HHF35, desmin, sr-actin, and troponin-T have the pote
ntial to confirm the commitment of the tumours to the myogenic pathway
which supports the diagnosis of RMS. However, it was impossible to re
late RMS to equivalent stages of skeletal muscle development. Aberrant
marker expression by RMS cells correlated significantly with patients
' survival.