title: Neurofibromatosis update.
abstract: This paper presents a review dealing with three major aspects of a modern understanding of the disorders known collectively as the neurofibromatoses (NF). In one section, the focus is on NF heterogeneity, clarifying the distinction between NF-1, NF-2 and atypical forms of NF. In another section, the focus is on NF-1 neural-crest tumors, with particular emphasis on the four types of neurofibromas (cutaneous, subcutaneous, nodular plexiform and diffuse plexiform), schwannomas, astrocytomas and neurofibrosarcomas. In the final section, focus is on NF-1 nontumor features, both as a clinical guide and as a reminder of the breadth of concerns that a pathogenetic schema will have to consider.
authors.affiliation: Neurofibromatosis Program, Baylor College of Medicine, Houston, Tex.
authors.forename: V M
authors.initials: VM
authors.lastname: Riccardi
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000473:pathology; D004806:Ependymoma / Q000473:pathology; D006801:Humans; D007859:Learning Disabilities / Q000473:pathology; D008579:Meningioma / Q000473:pathology; D009432:Neural Crest / Q000473:pathology; D009456:Neurofibromatosis 1 / Q000145:classification / Q000175:diagnosis / Q000235:genetics / Q000473:pathology; D010673:Pheochromocytoma / Q000473:pathology; D012583:Schwann Cells / Q000473:pathology; D013964:Thyroid Neoplasms / Q000473:pathology