Neurofibromatosis are several genetically inherited conditions that are clinically and genetically different and carry a high possibility of tumor formation. This disorder is divided into Neurofibromatosis type 1, Neurofibromatosis type 2, and Schwannomatosis.
Pathophysiology
It is an autosomal dominant disorder, whcih means only 1 copy of the affected gene is needed for the disorder to develop. Therefore, if only 1 parent has neurofibromatosis, his or her children have a 50% chance of developing the condition as well
The affected child could have mild NF1 even though inherited from a parent with a severe form of the disorder
Classification
Neurofibromatosis type 1, in which the nerve tissue grows tumors (neurofibromas) that may be benign, and may cause serious damage by compressing nerves and other tissues
Neurofibromatosis type 2, in which bilateral acoustic neuromas (tumors of the vestibulococchlear nerve or CN8 aka schwannoma) develop, often leading to hearing loss
Schwannomatosis, in which painful schwannomas develop on spinal and peripheral nerves
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