CANCER PREDISPOSITION SYNDROMES IN CHILDREN

The term cancer predisposition syndrome (CPS)includes several familial cancers in which a clear mode of inheritance may be established, although a specific gene defect has not been described in all cases.Advance in genetics and the development of new imaging have led to better understanding and early detection of these syndromes and offer the diagnosis of any associated tumors. As a result, imaging has become an essential component to management of CPSs and the care ofchildren with neurofibromatosis type I, Beckwith- Wiedemann syndrome, Von Hippel-Lindau (VHL), multiple endocrine neoplasia (MEN), familial adenomatous polyp and other syndromes. A radiologist should be familiar with these syndromes, their common associated tumors and thenew imaging techniques that are available to optimize the assessment of affected children.Of course recent advance in genetics has led to better understanding and early recongnition of these diseases and proper genetic counseling helps these patients.