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)

Also Known As:

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

Neoplasms: 1361496
- Neoplasms by Histologic Type: 2
  - Connective and Soft Tissue Neoplasms: 1
    - Sarcoma: 23591
      - Fibrosarcoma: 5538
        Neurofibrosarcoma: 1068
    - Connective Tissue Neoplasms: 9
      - Fibrous Tissue Neoplasms
        Fibrosarcoma: 5538
        Neurofibrosarcoma: 1068
  - Nerve Tissue Neoplasms: 1
    - Nerve Sheath Neoplasms: 280
      - Neurofibroma: 865
        Neurofibrosarcoma: 1068
Nervous System Diseases: 14737
- Neuromuscular Diseases: 2048
  - Peripheral Nervous System Diseases: 11768
    - Peripheral Nervous System Neoplasms: 49
      - Nerve Sheath Neoplasms: 280
        Neurofibroma: 865
        Neurofibrosarcoma: 1068
- Nervous System Neoplasms: 237
  - Peripheral Nervous System Neoplasms: 49
    - Nerve Sheath Neoplasms: 280
      - Neurofibroma: 865
        Neurofibrosarcoma: 1068

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Disease

Drug/Important Bio-Agent (IBA)