Neurofibrosarcoma as a complication of von Recklinghausen neurofibromatosis.
Neurofibrosarcoma (NFS), which is rare in the general population, occurs with excessive frequency among patients with von Recklinghausen neurofibromatosis (NF-1). Among 693 patients with NF-1 enrolled in the Baylor NF Program, 24 patients (3.5%) developed NFS or one of its variant forms. Nine of the twenty-four patients developed NFS within 3-94 months after enrolling in the Program for other reasons. Focus on these 9 patients alone suggests that the relative risk for patients with NF-1 developing NFS is 10-10,000 times greater than for the general population. Also, in NFS cohorts, patients with NF-1 are almost twice as likely to be under 30 years of age.
Riccardi
V M
VM
Department of Medicine, Baylor College of Medicine, Houston, Tex.
Powell
P P
PP
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D009455:Neurofibroma / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000453:epidemiology / Q000503:physiopathology; D012307:Risk Factors