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D017253Level 7

Neurofibromatoses

**Definition:** A group of disorders characterized by an autosomal dominant pattern of inheritance with high rates of spontaneous mutation and multiple neurofibromas or neurilemmomas. NEUROFIBROMATOSIS 1 (generalized neurofibromatosis) accounts for approximately 95% of cases, although multiple additional subtypes (e.g., NEUROFIBROMATOSIS 2, neurofibromatosis 3, etc.) have been described. (From Neurochirurgie 1998 Nov;44(4):267-72)

**Tree numbers:** - C04.557.580.600.580.590 - C04.700.631 - C10.562.600 - C10.574.500.549 - C16.320.400.560 - C16.320.700.633

**Synonyms:** - Multiple Neurofibromas - Neurofibromatosis - Neurofibromatosis Syndrome

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