Rhabdomyosarcoma arising in a congenital giant nevus associated with neurocutaneous melanosis in a neonate.

The case of a newborn with a pedunculated embryonal rhabdomyosarcoma arising from a pigmented giant congenital nevus located on the posterior part of the neck and upper trunk is presented. The nevus was associated with melanosis of the leptomeninges and central nervous system. The pedunculated tumor was removed and the infant was given subsequent chemotherapy. In spite of well-tolerated chemotherapy, he died on the 30th day of life due to a staphylococcal pneumonia.