ABSTRACT
Children with von Recklinghausen's neurofibromatosis have an increased risk of rhabdomyosarcoma. Li–Fraumeni's syndrome is an autosomal dominant cancer syndrome associated with a germ line mutation of p53. The alveolar variant of the rhabdomyosarcoma accounts for 20% and occurs commonly in the extremities, the trunk or the perineum. They have an unfavourable prognosis. Genitourinary rhabdomyosarcoma accounts for 26%. Bladder tumours produce haematuria and urinary obstruction. The incidence of neuroblastoma is 1 in 7500–10,000. Wilms's tumour incidence is 1 in 10,000 in children younger than 15 years. Bazex's syndrome is associated with psoriasiform lesions on the hands, foot, ears and nose. It is a rare paraneoplastic syndrome affecting the skin. In 70% of the cases reported in the literature, the cutaneous syndrome precedes the discovery of the neoplasm, which generally involves the upper air ways or the gastrointestinal tract and the lungs.
