Incidence, types, and management of cancer in patients with neurofibromatosis.
Neurofibromatosis Type 1 (NF1) is a common genetic disease with an incidence of 1 in 3,000 to 4,000. Clinical manifestations may include cafe-au-lait macules, axillary freckling, neurofibromas, Lisch nodules, skeletal dysplasias, learning disabilities, and a variety of neoplasms. In persons with NF1, some malignancies occur at an increased incidence compared to the general population. These include central nervous system astrocytomas, especially involving the visual pathways; ependymomas; meningiomas; neurofibrosarcomas; rhabdomyosarcomas; nonlymphocytic leukemias; and pheochromocytomas. The authors discuss the dysplastic and malignant features associated with NF and the concept of NF as a "neurocristopathy."
Cohen
B H
BH
Department of Pediatrics and Neurology, Cleveland Clinic Foundation.
Rothner
A D
AD
D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009378:Neoplasms, Multiple Primary / Q000453:epidemiology* / Q000401:mortality / Q000473:pathology / Q000628:therapy; D009456:Neurofibromatosis 1 / Q000209:etiology / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D015996:Survival Rate