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)

Annotation

coordinate IM with precoordinated organ/neoplasm term (IM)

DUI

D018319 MeSH Browser

CUI

M0027554

Previous indexing

Fibrosarcoma (1966-1978); Neurofibroma (1969-1993)

History note

1994; for Malignant Peripheral Nerve Sheath Tumors, use Neurilemoma 2000-2018

Public note

1994; for Malignant Peripheral Nerve Sheath Tumors, see Neurilemoma 2000-2018