Orthopedics > OCOSH Classification > Bone Diseases > Orthopaedic Oncology > Connective and Soft Tissue Neoplasms > Connective Tissue Neoplasms > Fibrous Tissue Neoplasms > Fibrosarcoma > Neurofibrosarcoma

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A malignant tumor that arises from small cutaneous nerves, is locally aggressive, and has a potential for metastasis. Characteristic histopathologic features include proliferating atypical spindle cells with slender wavy and pointed nuclei, hypocellular areas, and areas featuring organized whorls of fibroblastic proliferation. The most common primary sites are the extremities, retroperitoneum, and trunk. These tumors tend to present in childhood, often in association with NEUROFIBROMATOSIS 1. (From DeVita et al., Cancer: Principles & Practice of Oncology, 5th ed, p1662; Mayo Clin Proc 1990 Feb;65(2):164-72)

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Malignant Peripheral Nerve Sheath Tumors

Location: http://www.thedoctorsdoctor.com/diseases/mpnst.htm

The malignant peripheral nerve sheath tumor (MPNST) is the malignant counterpart to benign soft tissue tumors such as neurofibromas and schwannomas. This term is preferred to older designations such as malignant schwannomas and neurofibrosarcomas.
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Neurofibrosarcoma PathWeb

Location: http://pathweb.uchc.edu/eAtlas/CNS/701.htm

eAtlas. Includes illustration, etiology, pathogenesis, epidemiology, gross and microscopic description, clinical correlate and reference. Link to medline search
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Neurofibrosarcoma Patient Information

Location: http://www.childrenshospital.org/az/Site1086/mainpageS1086P0.html

Neurofibrosarcoma (Peripheral Nerve Sheath Tumor)
Peripheral nerves are nerves that receive messages from the central nervous system (brain and spinal cord) leading them to stimulate voluntary movement. Neurofibrosarcoma, also known as peripheral nerve sheath tumor, is a malignant tumor that develops in the cells surrounding these peripheral nerves. It can...
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Neurogenic Sarcoma DDB

Location: http://www.diseasesdatabase.com/ddb32055.htm

Neurogenic sarcoma aka/or Neurofibrosarcoma
risk factors may include + Neurofibromatosis type 1
Neurogenic sarcoma: Definition(s) via UMLS "A malignant tumor that arises from small cutaneous nerves, is locally aggressive, and has a potential for metastasis. Characteristic histopathologic features include proliferating atypical spindle cells with slender wavy and pointed nuclei, hypocellular areas, and areas featuring...
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Neurosarcoma Wheeless

Location: http://www.wheelessonline.com/ortho/neurosarcoma

Discussion: - also called malignant schwannoma or fibrosarcoma of nerve sheath; - extracompartmental high grade tumor that develops from sheaths (Schwann cells) of peripheral nerves; - usually is a primary lesion occuring along proximal major nerve trunks, ...
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Posterior mediastinal neurofibrosarcoma

Location: http://www.smw.ch/docs/oeil/1999/129-21-288-99.html

Halkic N, Zeini SA, Bouchardy J, Boumghar M. Posterior mediastinal neurofibrosarcoma in a patient with von Recklinghausen's disease. Schweiz Med Wochenschr 1999;129:818.
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What is a Malignant Peripheral Nerve Sheath Tumour

Location: http://www.cancerbackup.org.uk/QAs/SofttissuesarcomasQAs/AllQAs/related_faqs/QAs/377

A malignant peripheral nerve sheath tumour (MPNST) is a type of cancer called a soft tissue sarcoma. Soft tissue sarcomas are cancers that develop in the supporting or connective tissues of the body (such as muscle, fat, nerves and blood vessels). MPNSTs probably start from Schwann cells, which form the protective...
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