We report the case of a 19 month old boy referred to our institution becaus
e of a pelvic tumor initially identified as an embryonal rhabdomyosarcoma a
nd treated with surgery and chemotherapy. Eight years after the initial sur
gery, a local tumor recurrence with bone metastasis was found, Histological
examination and immunohistochemistry showed a double differentiation with
both muscular and neuronal cells, This double differentiation was retrospec
tively found in the initial tumor, then allowing the diagnosis of malignant
ectomesenchymoma also called gangliorhabdomyosarcoma. This rare tumor, occ
urring mainly during childhood, is composed of neuroblasts and/or ganglion
cells and of malignant mesenchymal cells (usually rhabdomyosurcomatous cell
s).