hereditary preneoplasia

hereditary preneoplasia

A general term for a neoplastic substrate related to a syndrome Examples Phakomatoses–eg, Cowden's disease, multiple exostoses, Peutz-Jegher syndrome, von Hippel-Lindau syndrome, neurofibromatosis, tuberous sclerosis, genodermatoses–eg, albinism, dyskeratosis congenita, epidermodysplasia verruciformis, polydysplastic epidermolysis bullosa, Werner syndrome, xeroderma pigmentosa, chromosome instability syndromes and immune deficiencies–eg, ataxia-telangiectasia, CVID, Wiskott-Aldrich syndrome, X-linked agammaglobulinemia and X-linked lymphoproliferative syndrome. See Cancer families, Common variable immunodeficiency. Cf One-hit, two-hit model.