neurofibrosarcoma


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neurofibrosarcoma

/neu·ro·fi·bro·sar·co·ma/ (-fi″bro-sahr-ko´mah) a malignant type of schwannoma superficially resembling a fibrosarcoma, sometimes occurring in association with neurofibromatosis that is undergoing malignant transformation.

malignant peripheral nerve sheath tumour

A poorly understood malignancy, presumably of Schwann cell origin, which is thought to arise from the neural crest. It is the most common malignancy of peripheral nerves, and represents 5 to 10% of all soft-tissue tumours. It occurs in the lower extremities in relatively young patients (age 20 to 50). Half of cases occur in patients with neurofibromatosis type 1, in which the male:female ratio is 4:1; 2/3 of MPNSTs arise from neurofibromas, 1/3 arise de novo. Origin from pre-existing schwannomas, ganglioneuromas or phaeochromocytomas are rare; 10% arise in a background of therapeutic radiation.
 
Management
Wide local excision; radiation and chemotherapy have been used with varying degrees of failure.

neurofibrosarcoma

See NEUROSARCOMA.

neurofibrosarcoma

a malignant tumor originating from Schwann cells of the peripheral nervous system and seen most commonly in cattle.
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12) Cases of multicentric neurofibrosarcoma have been previously described in Canada geese (Branta canadensis).
MRI diagnosis was inconclusive, but the differential included, schwannoma, malignant histiocytoma, neurofibrosarcoma, or soft tissue malignancy.
Malignant peripheral nerve sheath tumor (MPNST) was previouslyknown as malignant schwannoma, neurogenic sarcoma, malignant neurilemmoma, and neurofibrosarcoma.
Multiple endocrine neoplasia 2B syndrome includes ganglioneuromas, and patients with neurofibromatosis, or von Recklinghausen's disease, carry a 7% to 10% lifetime risk of developing malignant neurofibrosarcoma.
Chromosomes 17 and 11 involved in marker formation in neurofibrosarcoma in von Recklinghausen disease: a cytogenetic and in situ hybridization study.
In such patients, the incidence of malignant transformation to neurofibrosarcoma is increased.
The tumour, known as a Neurofibrosarcoma, makes up just one per cent of all cancers, and it grew quickly, resulting in Paul having a 4lb tumour removed from his abdominal area.
Differential diagnosis of Castleman's disease Mediastinum Head and neck Hypervascular metastasis Carcinoma of the thyroid Leiomyosarcoma Cat scratch fever Lymphoma Epstein-Barr virus Mediastinitis Hodgkin's disease Medullary carcinoma Human immunodeficiency of the thyroid virus infection Neurofibrosarcoma Infectious lymphadenitis Parathyroid adenoma Lymphoma Substernal goiter Metastatic disease Thymoma Paraganglioma Parathyroid adenoma Schwannoma
The type of tumour, known as a neurofibrosarcoma, makes up just 1per cent of all cancers.