Neurofibromatosis update.

VM Riccardi - Neurofibromatosis, 1989 - europepmc.org
VM Riccardi
Neurofibromatosis, 1989europepmc.org
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 …
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.
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