TY - JOUR T1 - Demographical Profile and Spectrum of Multiple Malignancies in Children and Adults with Neurocutaneous Disorders JF - Anticancer Research JO - Anticancer Res SP - 5453 LP - 5457 DO - 10.21873/anticanres.12877 VL - 38 IS - 9 AU - AGATA MARJANSKA AU - AGNIESZKA JATCZAK-GACA AU - AGNIESZKA WOJTKIEWICZ AU - MARIUSZ WYSOCKI AU - JAN STYCZYNSKI Y1 - 2018/09/01 UR - http://ar.iiarjournals.org/content/38/9/5453.abstract N2 - Background/Aim: Neurocutaneous disorders, also referred as phacomatoses, are congenital disorders manifesting at different ages with central nervous system and cutaneous abnormalities. Analysis of the demographic and clinical profile of patients with phacomatoses in the context of the incidence and spectrum of malignancy. Materials and Methods: This is a retrospective analysis of 20 years of data in a single-center study in Poland. Results: Phacomatoses were diagnosed in 45.6% (913/2,003) of referred patients, including 61.4% children. The distribution of phacomatoses included: neurofibromatosis type 1 (NF1) in 92.4%, tuberous sclerosis complex (TSC) 3.9%, neurofibromatosis type 2 (NF2) 2.0%, Klippel-Trenaunay syndrome 0.5%, Von Hippel-Lindau syndrome 0.5%, and other sporadic diseases 0.7%. Non-phacomatosis patients were diagnosed mainly for cafe-au-lait-macules (42.8%). The frequency of malignancy was 9.4% (86/913), including 9.1% in patients with NF1; 27.8% in NF2; and 8.3% in TSC. Multiple malignancies were diagnosed in 0.7% and 7% of all phacomatosis and malignancy-diagnosed patients, respectively. Conclusion: The risk of malignancy in patients with phacomatoses was 21.3-fold higher than in the general population. The risk of secondary malignancy was 7%. ER -