{
  "id": 4722,
  "label": "neurofibrosarcoma",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0002675",
  "properties": {
    "xrefs": [
      "DOID:3512",
      "GARD:0008211",
      "MEDGEN:104927",
      "MESH:D018319",
      "UMLS:C0206729"
    ],
    "synonyms": [
      "neurosarcoma [obs]"
    ],
    "categories": [
      {
        "ref": "MONDO:0005071",
        "name": "nervous system disorder"
      }
    ],
    "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)"
  },
  "isLeaf": true,
  "isRoot": false,
  "child_count": 0,
  "parents": [
    {
      "id": 17201,
      "label": "neurofibroma",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        4613,
        17200
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:962",
          "GARD:0007191",
          "ICD9:215.9",
          "ICDO:9540/0",
          "MEDGEN:45058",
          "MESH:D009455",
          "MedDRA:10029267",
          "NCIT:C3272",
          "ONCOTREE:NFIB",
          "Orphanet:252183",
          "SCTID:404029005",
          "UMLS:C0027830",
          "icd11.foundation:221014277"
        ],
        "synonyms": [
          "neurofibroma",
          "neurofibroma (WHO grade I)",
          "neurofibroma, benign",
          "NFIB"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "An intraneural or extraneural neoplasm arising from nerve tissues and neural sheaths. It is composed of perineurial-like fibroblasts and Schwann cells. It usually presents as a localized cutaneous lesion and less often as a circumscribed peripheral nerve mass. Patients with neurofibromatosis type 1 present with multiple masses. Neurofibromas which arise from major nerves and plexiform neurofibromas are precursor lesions to malignant peripheral nerve sheath tumors."
      },
      "child_count": 22,
      "reference_id": "MONDO:0016755"
    }
  ],
  "children": [],
  "roots": [
    {
      "id": 17201,
      "label": "neurofibroma"
    }
  ]
}