9540/3 Malignant peripheral nerve sheath tumor


Synonyms

MPNST, NOS
Neurofibrosarcoma
Neurogenic sarcoma
Neurosarcoma


Definitions

Tumours of the central nervous system
ICD-O-3 topography code: C70, C71 and C72
ICD10: C70, C71 and C72
           

An uncommon, highly aggressive malignant tumor, arising from the peripheral nerves and affecting mostly adults in their third to sixth decades of life. It usually occurs in medium-sized and large nerves of the buttock, thigh, upper arm, or the paraspinal region.

Histological variants:
> Epithelioid MPNST
> MPNST with divergent mesenchymal/epithelial differentiation
> Melanotic MPNST

MPNST may be associated with neurofibromatosis type 1 (NF1).



Prostate gland
ICD-O-3 topography code: C61
ICD10: C61
           

An uncommon, highly aggressive malignant tumor, arising from the peripheral nerves and affecting mostly adults in their third to sixth decades of life. It usually occurs in medium-sized and large nerves of the buttock, thigh, upper arm, or the paraspinal region.
MPNST may be associated with neurofibromatosis type 1 (NF1).



Nasal cavity and Sinuses
ICD-O-3 topography code: C30-C31
ICD10: C30-C31
           

An uncommon, highly aggressive malignant tumor, arising from the peripheral nerves and affecting mostly adults in their third to sixth decades of life. It usually occurs in medium-sized and large nerves of the buttock, thigh, upper arm, or the paraspinal region.
MPNST may be associated with neurofibromatosis type 1 (NF1).



Cervix uteri
ICD-O-3 topography code: C53
ICD10: C53
           

Malignant peripheral nerve sheath tumour (MPNST) is an uncommon, highly aggressive malignant tumour, arising from the peripheral nerves and affecting mostly adults in their third to sixth decades of life. It usually occurs in medium-sized and large nerves of the buttock, thigh, upper arm, or the paraspinal region. MPNST may be associated with neurofibromatosis type 1 (NF1).

Cervical MPNST is extremely rare. Fascicles of atypical spindle cells invade the cervical stroma and the surrounding endocervical glands. Myxoid paucicellular areas and densely cellular areas are characteristically intermixed. Patients typically present with vaginal bleeding or discharge. The prognosis is poor1
 
Tavassoli FA, Devilee P (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Breast and Female Genital Organs.
3rd Edition
IARC Press: Lyon 2003



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Peripheral nerves and autonomic nervous system
ICD-O-3 topography code: C47
ICD10: C47
           

Malignant peripheral nerve sheath tumor (MPNST) is an uncommon, highly aggressive malignant tumor, arising from the peripheral nerves and affecting mostly adults in their third to sixth decades of life. It usually occurs in medium-sized and large nerves of the buttock, thigh, upper arm, or the paraspinal region.
MPNST may be associated with neurofibromatosis type 1 (NF1).

The following subtypes are distinguished:
Epithelioid MPNST
MPNSTwith divergent mesenchymal and/or epithelial differentiation
Melanotic MPNST



Connective, subcutaneous and other soft tissues
ICD-O-3 topography code: C49
ICD10: C49
           

An uncommon, highly aggressive malignant tumor, arising from the peripheral nerves and affecting mostly adults in their third to sixth decades of life. It usually occurs in medium-sized and large nerves of the buttock, thigh, upper arm, or the paraspinal region.
MPNST may be associated with neurofibromatosis type 1 (NF1).