Diseases

Neurofibrosarcoma

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)

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

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 Record

Classification

Broader headings

Related Concepts

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MeSH Record

Synonyms

20 entry terms
  • Sarcoma, Neurogenic
  • Neurofibrosarcomas
  • Neurogenic Sarcoma
  • Neurogenic Sarcomas
  • Sarcomas, Neurogenic
  • MPNST
  • Malignant Neurilemmoma
  • Malignant Neurilemoma
  • Malignant Peripheral Nerve Sheath Tumor
  • Malignant Peripheral Nerve Sheath Tumors
  • Neurilemmosarcoma
  • Peripheral Nerve Sheath Tumors, Malignant
  • Schwannoma, Malignant
  • Malignant Neurilemmomas
  • Malignant Neurilemomas
  • Malignant Schwannoma
  • Malignant Schwannomas
  • Neurilemmoma, Malignant
  • Neurilemmosarcomas
  • Neurilemoma, Malignant

MeSH Record

Aspects Covered

39 allowable subheadings

Indexed with the subheadings blood, blood supply, cerebrospinal fluid, chemically induced, chemistry, classification, complications, congenital, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, secondary, surgery, therapy, ultrastructure, urine, veterinary, virology.

MeSH Record

Indexing Annotation

coordinate IM with precoordinated organ/neoplasm term (IM)

MeSH Record

History Note

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

MeSH Record

Previous Indexing

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

MeSH Hierarchy

Tree Numbers

  1. C04w.557.450.565w.590w.350w.590
  2. C04w.557.450.795w.350w.590
  3. C04w.557.580.600w.580w.795
  4. C10w.551w.775.500w.750w.750
  5. C10w.668w.829w.725.500w.600w.600

AMA Style

References

  1. National Library of Medicine. Neurofibrosarcoma. Medical Subject Headings (MeSH). 2026. Unique ID D018319. http://id.nlm.nih.gov/mesh/2026/D018319
  2. Neurofibrosarcoma. In: Wikidata. https://www.wikidata.org/wiki/Q18555964