9540/1 Von Recklinghausen disease


Related terms

Neurofibromatosis, NOS

Definitions

Peripheral nerves and autonomic nervous system
ICD-O-3 topography code: C47

An autosomal dominant hereditary neoplastic syndrome. Two distinct clinicopathological entities are recognized:
Neurofibromatosis type 1 (NF1) is associated with the presence of cafe-au-lait cutaneous lesions, multiple neurofibromas, malignant peripheral nerve sheath tumors, optic nerve gliomas, and bone lesions.
Neurofibromatosis type 2 (NF2) is associated with the bilateral acustic schwannomas, meningiomas, and gliomas.



Tumours of the central nervous system
ICD-O-3 topography code: C70, C71 and C72

An autosomal dominant hereditary neoplastic syndrome. Two distinct clinicopathological entities are recognized:
Neurofibromatosis type 1 (NF1) is associated with the presence of cafe-au-lait cutaneous lesions, multiple neurofibromas, malignant peripheral nerve sheath tumors, optic nerve gliomas, and bone lesions.
Neurofibromatosis type 2 (NF2) is associated with the bilateral acustic schwannomas, meningiomas, and gliomas.