Preferred Name

Neurofibrosarcoma
Synonyms
Definitions

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 (MeSH).

ID

http://uri.neuinfo.org/nif/nifstd/birnlex_12609

createdDate

2007-10-05

definingCitation

DeVita et al., Cancer: Principles & Practice of Oncology, 5th ed, p1662; Mayo Clin Proc 1990 Feb;65(2):164-72

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 (MeSH).

editorial note

http://uri.neuinfo.org/nif/nifstd/readable/uncurated

hasBirnlexCurator

http://uri.neuinfo.org/nif/nifstd/readable/Bill_Bug

hasCurationStatus

http://uri.neuinfo.org/nif/nifstd/readable/uncurated

hasDefinitionSource

http://uri.neuinfo.org/nif/nifstd/readable/MeSH_defSource

hasExternalSource

http://uri.neuinfo.org/nif/nifstd/readable/MeSH

label

Neurofibrosarcoma

MeshUid

D018319

modifiedDate

2007-10-05

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 (MeSH).

http://uri.neuinfo.org/nif/nifstd/readable/uncurated

preferred label

Neurofibrosarcoma

synonym

Neurogenic Sarcoma

subClassOf

http://uri.neuinfo.org/nif/nifstd/birnlex_12602

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