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... > Neoplasms > Neoplasms by Histologic Type > Neoplasms, Connective and Soft Tissue > Sarcoma > Fibrosarcoma > Neurofibrosarcoma
... > Skin and Connective Tissue Diseases > Connective Tissue Diseases > Neoplasms, Connective Tissue > Neoplasms, Fibrous Tissue > Fibrosarcoma > Neurofibrosarcoma
... > Neoplasms > Neoplasms by Histologic Type > Neoplasms, Connective and Soft Tissue > Neoplasms, Connective Tissue > Neoplasms, Fibrous Tissue > Fibrosarcoma > Neurofibrosarcoma
... > Nervous System Diseases > Nervous System Neoplasms > Peripheral Nervous System Neoplasms > Nerve Sheath Neoplasms > Neurofibroma > Neurofibrosarcoma
... > Neoplasms > Neoplasms by Site > Nervous System Neoplasms > Peripheral Nervous System Neoplasms > Nerve Sheath Neoplasms > Neurofibroma > Neurofibrosarcoma
... > Nervous System Diseases > Neuromuscular Diseases > Peripheral Nervous System Diseases > Peripheral Nervous System Neoplasms > Nerve Sheath Neoplasms > Neurofibroma > Neurofibrosarcoma
... > Neoplasms > Neoplasms by Histologic Type > Neoplasms, Nerve Tissue > Nerve Sheath Neoplasms > Neurofibroma > Neurofibrosarcoma

Preferred term

Neurofibrosarcoma  

Type

  • mesh:Descriptor

Definition

  • 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) A malignant neurilemmoma with nerve sheath differentiation. It is often associated with NEUROFIBROMATOSIS 1 and RHABDOMYOSARCOMA.

Broader concept

Entry terms

  • Sarcoma, Neurogenic

Allowable Qualifier(s)

In other languages

URI

http://data.loterre.fr/ark:/67375/JVR-MCDL88V2-H

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