Rhabdomyosarcoma in childhood. An immunohistological analysis with myoglobin, desmin and vimentin

Abstract

A retrospective immunohistological analysis of 64 rhabdomyosarcomas in children was performed using antibodies against desmin and in 35 cases against myoglobin. In addition a group of 12 undifferentiated tumours in which the differential diagnosis included rhabdomyosarcomas was studied. Rhabdomyosarcomas were desmin positive in 57 cases (89%), 28 cases of which showed positivity of undifferentiated small cells (44%). Myoglobin was positive in 23 cases (66%), but only one case showed positivity of undifferentiated small cells. The results show the limited use of myoglobin in the diagnosis of rhabdomyosarcoma, especially of cases with a low degree of differentiation. Three out of 12 undifferentiated tumours were desmin positive and were reclassified as rhabdomyosarcomas. In 49 rhabdomyosarcomas the investigation was complemented by the demonstration of vimentin. Vimentin was shown to be present in 27 cases in tumour cells (55%). Undifferentiated cells were positive in 26 tumours (53%) and rhabdomyoblasts reacted in 9 cases (18%). Coexpression of vimentin and desmin in some cases reflects a situation in rhabdomyosarcomas that aberrantly mimics skeletal muscle embryogenesis. In three cases desmin and vimentin positive globular inclusions were observed. It is suggested that their formation is related to dystrophic changes of contractile and cytoskeletal filaments. From the diagnostic point of view a high percentage of desmin positive cases makes desmin a successful marker for rhabdomyoblastic tumours. It is pointed out, however, that even immunohistochemistry may not contribute to solving the problem of undifferentiated tumours and that each case must be evaluated comprehensively.