Preferred Label : malignant peripheral nerve sheath tumor;
RADLEX synonyms : neurogenic sarcoma; malignant peripheral nerve sheath tumor (WHO grade III-IV); malignant schwannoma; neurofibrosarcoma;
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) [MeSH];
UMLS CUI (RADLEX) : C0206729;
Origin ID : RID4444;
Automatic exact mappings (from CISMeF team)
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) [MeSH]
