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Neurofibrosarcoma

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 and Practice of Oncology, 5th ed, p1662; Mayo Clin Proc 1990 Feb;65(2):164-72)

Year introduced: 1994

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Tree Number(s): C04.557.450.565.590.350.590, C04.557.450.795.350.590, C04.557.580.600.580.795, C10.551.775.500.750.750, C10.668.829.725.500.600.600

MeSH Unique ID: D018319

Entry Terms:

  • Neurofibrosarcomas
  • Sarcoma, Neurogenic
  • Neurogenic Sarcoma
  • Neurogenic Sarcomas
  • Sarcomas, Neurogenic
  • Malignant Peripheral Nerve Sheath Tumors
  • Neurilemmosarcoma
  • Neurilemmosarcomas
  • MPNST
  • Peripheral Nerve Sheath Tumors, Malignant
  • Malignant Neurilemoma
  • Malignant Neurilemomas
  • Neurilemoma, Malignant
  • Schwannoma, Malignant
  • Malignant Schwannoma
  • Malignant Schwannomas
  • Malignant Peripheral Nerve Sheath Tumor
  • Malignant Neurilemmoma
  • Malignant Neurilemmomas
  • Neurilemmoma, Malignant

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