COSTELLO-SYNDROME - 2 CASES WITH EMBRYONAL RHABDOMYOSARCOMA

Costello syndrome is a well delineated mental retardation syndrome of unknown aetiology in which the occurrence of benign tumours, especiall y papillomata, is recognised. We report two children in whom the diagn osis of Costello syndrome was made in the first months of life, who bo th developed a retroperitoneal embryonal rhabdomyosarcoma. Although no t previously reported, the occurrence of this relatively uncommon chil dhood tumour in two girls with Costello syndrome suggests that an incr eased risk of malignancy may be part of this condition. The genetic ba sis of this susceptibility requires further clarification.