neurofibrosarcoma

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.

Collins Dictionary of Medicine © Robert M. Youngson 2004, 2005

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Case 1 Case 2 Sex Male Female Breed Mixed Mixed Age 12 years 10 years- Neoplasms of Skin tumors in the Inguinal near the vulva the location pelvic member, knee region Neoplasia type Neurofibrosarcoma Solid mammary carcinoma Type of surgery Subdermal tube near the Subdermal tube near the flank region.

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Malignant peripheral nerve sheath tumor (MPNST) was previouslyknown as malignant schwannoma, neurogenic sarcoma, malignant neurilemmoma, and neurofibrosarcoma. MPNST is a rare neoplasm that originates in nerve sheath cells, including Schwann cells and neural and perineural fibroblasts.

Malignant peripheral nerve sheath tumor of the infratemporal fossa with intracranial extension

This information made it possible to narrow the histological differential diagnosis of her lesions to neurofibrosarcoma, neurofibromatosis, or schwannoma.

Neurofibromatosis type I: a case report and review of the literature

Soft-tissue sarcomas are tumors arising from extraskeletal connective tissues and from Schwann cells and include fibrosarcoma, liposarcoma, hemangiopericytoma, rhabdomyosarcoma, lymphangiosarcoma, malignant fibrous histiocytoma, leiomyosarcoma, myxosarcoma, myxofibrosarcoma, mesenchymoma, spindle cell tumors, and malignant peripheral nerve sheath tumors (neurofibrosarcoma, Schwannoma).

Pelvic limb amputation for the treatment of a soft-tissue sarcoma of the tibiotarso-tarsometatarsal joint in a blue-and-gold macaw (Ara ararauna)

For reasons not yet understood, schwannomas are mostly benign and less than 1% becomes malignant, degenerating into a form of cancer known as neurofibrosarcoma. Schwannomas can arise from a genetic disorder called neurofibromatosis.

Schwannoma tongue--case report

In such patients, the incidence of malignant transformation to neurofibrosarcoma is increased.

Deep-lobe parotid tumor presenting in the parapharyngeal space

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.

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Chromosomes 17 and 11 involved in marker formation in neurofibrosarcoma in von Recklinghausen disease: a cytogenetic and in situ hybridization study.

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(24-41) In Canada geese in particular, neurofibrosarcoma, lipoma, liposarcoma, fibroma, and spindle cell sarcoma have all been reported.

Infiltrative spinal lipoma in a Canada goose (Branta canadensis)

(1,2) Sarcomas of the elderly are leiomyosarcoma, angiosarcoma, spindle cell sarcoma, alveolar soft part sarcoma, fibrosarcoma, neurofibrosarcoma, and mixed mesodermal tumors of the vagina.

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