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DeCS
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Descriptor English:
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Neurofibrosarcoma
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Descriptor Spanish:
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Neurofibrosarcoma
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Descriptor Portuguese:
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Neurofibrossarcoma
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Synonyms English:
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MPNST
Malignant Neurilemmoma
Malignant Neurilemmomas
Malignant Neurilemoma
Malignant Neurilemomas
Malignant Peripheral Nerve Sheath Tumor
Malignant Peripheral Nerve Sheath Tumors
Malignant Schwannoma
Malignant Schwannomas
Neurilemmoma, Malignant
Neurilemmosarcoma
Neurilemmosarcomas
Neurilemoma, Malignant
Neurofibrosarcomas
Neurogenic Sarcoma
Neurogenic Sarcomas
Peripheral Nerve Sheath Tumors, Malignant
Sarcoma, Neurogenic
Sarcomas, Neurogenic
Schwannoma, Malignant
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Tree Number:
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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
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Definition English:
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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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Indexing Annotation English:
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coordinate IM with precoordinated organ/neoplasm term (IM)
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History Note English:
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1994; for Malignant Peripheral Nerve Sheath Tumors, use Neurilemoma 2000-2018
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Allowable Qualifiers English:
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Record Number:
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31253
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Unique Identifier:
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D018319
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Occurrence in VHL:
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Similar:
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DeCS
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