9540/0 Neurofibroma, NOS


Definitions

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

An intraneural or extraneural neoplasm arising from nerve tissues and neural sheaths. It is composed of perineurial-like fibroblasts and Schwann cells. It usually presents as a localized cutaneous lesion and less often as a circumscribed peripheral nerve mass. Neurofibromas which arise from major nerves and plexiform neurofibromas are precursor lesions to malignant peripheral nerve sheath tumors.
Multiple neurofibromas are a hallmark of the neurofibromatosis type 1 (NF1) which is inherited by an autosomal dominant trait.



Breast
ICD-O-3 topography code: C50

An intraneural or extraneural neoplasm arising from nerve tissues and neural sheaths. It is composed of perineurial-like fibroblasts and Schwann cells.

Neurofibroma and schwannoma of the breast are discussed together under Benign peripheral nerve-sheath tumours.





Nasal cavity and Sinuses
ICD-O-3 topography code: C30-C31

An intraneural or extraneural neoplasm arising from nerve tissues and neural sheaths. It is composed of perineurial-like fibroblasts and Schwann cells. It usually presents as a localized cutaneous lesion and less often as a circumscribed peripheral nerve mass. Neurofibromas which arise from major nerves and plexiform neurofibromas are precursor lesions to malignant peripheral nerve sheath tumors.
Multiple neurofibromas are a hallmark of the neurofibromatosis type 1 (NF1) which is inherited by an autosomal dominant trait.



Hypopharynx, Larynx and Trachea
ICD-O-3 topography code: C13, C32, C33

An intraneural or extraneural neoplasm arising from nerve tissues and neural sheaths. It is composed of perineurial-like fibroblasts and Schwann cells. It usually presents as a localized cutaneous lesion and less often as a circumscribed peripheral nerve mass. Neurofibromas which arise from major nerves and plexiform neurofibromas are precursor lesions to malignant peripheral nerve sheath tumors.
Multiple neurofibromas are a hallmark of the neurofibromatosis type 1 (NF1) which is inherited by an autosomal dominant trait.



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

An intraneural or extraneural neoplasm arising from nerve tissues and neural sheaths. It is composed of perineurial-like fibroblasts and Schwann cells. It usually presents as a localized cutaneous lesion and less often as a circumscribed peripheral nerve mass3
 
Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (Eds.)
WHO Classification of Tumours of the Central Nervous System.
4th Edition
International Agency for Research on Cancer: Lyon 2007



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Neurofibromas which arise from major nerves and plexiform neurofibromas are precursor lesions to malignant peripheral nerve sheath tumors. Multiple neurofibromas are a hallmark of neurofibromatosis type 1 (NF1) which is inherited by an autosomal dominant trait.



Connective, subcutaneous and other soft tissues
ICD-O-3 topography code: C49

An intraneural or extraneural neoplasm arising from nerve tissues and neural sheaths. It is composed of perineurial-like fibroblasts and Schwann cells. It usually presents as a localized cutaneous lesion and less often as a circumscribed peripheral nerve mass. Neurofibromas which arise from major nerves and plexiform neurofibromas are precursor lesions to malignant peripheral nerve sheath tumors.
Multiple neurofibromas are a hallmark of the neurofibromatosis type 1 (NF1) which is inherited by an autosomal dominant trait.