ABSTRACT

Answer: (b) RMS may be associated with congenital anomalies including the nervous system and the genitourinary (GU) system. Neurofibromatosis is the most common syndrome in which RMS has been described. The incidence is 0.5% (Intergroup Rhabdomyosarcoma Study-IV study). The disease in these patients is very progressive and it is recommended that patients with RMS and neurofibromatosis be treated with intensive contemporary therapy protocols. Costello syndrome is associated with increased risk of solid tumors including RMS. Other congenital syndromes associated with RMS are Gorlin's basal cell nevus syndrome, RubinsteinTaybi syndrome, trisomy 21, BeckwithWiedemann syndrome, fetal alcohol syndrome, and Li-Fraumeni syndrome. Whereas Wilms' tumor is the most common malignancy associated with Beckwith-Wiedemann syndrome, in the first 7 years of life, RMS, adrenocortical carcinoma, and hepatoblastoma may be seen.