V. Karpuz et al., Malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation and glandular component, ANN PATHOL, 20(1), 2000, pp. 62-65
An exceptional case of malignant peripheral nerve sheath tumor with striate
d muscle differentiation and glandular component is reported, in a 52-year
old man. This tumor measured 8 cm in diameter, and was localized in the che
st wall, infiltrating the skeletal muscle. The mesenchymal portion of the t
umor was composed mostly of spindle cells arranged in interlacing fascicles
. Between these fascicles, there were large cells with abundant eosinophili
c cytoplasm and clear elongated nucleus. Immunohistochemical study demonstr
ated cytokeratin, EMA and CEA expression in the glandular component and S10
0 protein expression in the major portion of the mesenchymal component. The
large cells identified as rhabdomyoblasts, expressed desmin, myoglobin, al
pha-SR actin and alpha-SM actin. The tumor recurred 5 years after its resec
tion. Histological and immunohistochemical features were identical. We thin
k that positivity of neoplastic striated muscle cells with alpha-SM actin r
eflects an early differentiation phase of these cells.