Concept information
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Neoplasms, Connective and Soft Tissue
Neoplasms, Connective Tissue
Neoplasms, Fibrous Tissue
Fibrosarcoma
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Peripheral Nervous System Diseases
Peripheral Nervous System Neoplasms
Nerve Sheath Neoplasms
Neurofibroma
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Nervous System Neoplasms
Peripheral Nervous System Neoplasms
Nerve Sheath Neoplasms
Neurofibroma
Preferred term
Neurofibrosarcoma
Type
-
Topical Descriptor
Broader concept
Entry terms
- Neurogenic Sarcoma
- Neurogenic Sarcomas
- Sarcoma, Neurogenic
- Sarcomas, Neurogenic
Note
- coordinate IM with precoordinated organ/neoplasm term (IM)
Scope note
- 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)
History note
- 1994; for Malignant Peripheral Nerve Sheath Tumors, use Neurilemoma 2000-2018
In other languages
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Finnish
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neurofibrosarcoma
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Swedish
URI
http://www.yso.fi/onto/mesh/D018319
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