{
  "id": 17201,
  "label": "neurofibroma",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0016755",
  "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."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 11,
  "parents": [
    {
      "id": 4613,
      "label": "nerve sheath neoplasm",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        3627,
        20287
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:3193",
          "GARD:0023161",
          "MEDGEN:64639",
          "MESH:D018317",
          "NCIT:C4972",
          "ONCOTREE:NST",
          "UMLS:C0206727"
        ],
        "synonyms": [
          "neoplasm of nerve sheath",
          "neoplasm of the nerve sheath",
          "nerve sheath neoplasm",
          "nerve sheath tumor",
          "tumor of nerve sheath",
          "tumour of nerve sheath"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A benign or malignant neoplasm arising from the perineural cells in the sheaths surrounding the nerves. Representative examples include neurofibroma, schwannoma, and malignant peripheral nerve sheath tumor."
      },
      "child_count": 12,
      "reference_id": "MONDO:0002547"
    },
    {
      "id": 17200,
      "label": "benign peripheral nerve sheath tumor",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        17197
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0007180",
          "MEDGEN:1843306",
          "Orphanet:252131",
          "UMLS:C5577926"
        ],
        "synonyms": [
          "BPNST"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ]
      },
      "child_count": 3,
      "reference_id": "MONDO:0016752"
    }
  ],
  "children": [
    {
      "id": 3645,
      "label": "mediastinum neurofibroma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        5083,
        17201
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:12064",
          "GARD:0022943",
          "MEDGEN:277354",
          "NCIT:C6631",
          "UMLS:C1334674"
        ],
        "synonyms": [
          "mediastinal neurofibroma",
          "mediastinum neurofibroma",
          "neurofibroma of mediastinum",
          "neurofibroma of the mediastinum"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A neurofibroma that arises from the posterior mediastinum. Excision is usually curative."
      },
      "child_count": 0,
      "reference_id": "MONDO:0001426"
    },
    {
      "id": 3973,
      "label": "neurofibroma of spinal cord",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17201,
        20444
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:13742",
          "GARD:0023012",
          "MEDGEN:234804",
          "NCIT:C5145",
          "UMLS:C1336047"
        ],
        "synonyms": [
          "neurofibroma of spinal cord",
          "spinal cord neurofibroma"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A neurofibroma that arises from the spinal cord."
      },
      "child_count": 0,
      "reference_id": "MONDO:0001789"
    },
    {
      "id": 4480,
      "label": "Pacinian tumor",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17201
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:2669",
          "GARD:0023129",
          "ICD9:215.9",
          "ICDO:9507/0",
          "MEDGEN:87273",
          "NCIT:C4328",
          "SCTID:404033003",
          "UMLS:C0334599"
        ],
        "synonyms": [
          "Pacinian neurofibroma",
          "Pacinian tumor (morphologic abnormality)",
          "Pacinian tumour (morphologic abnormality)"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A neurofibroma characterized by the presence of structures which resemble Vater-Pacini corpuscles."
      },
      "child_count": 0,
      "reference_id": "MONDO:0002383"
    },
    {
      "id": 4722,
      "label": "neurofibrosarcoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17201
      ],
      "type_id": 0,
      "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)"
      },
      "child_count": 0,
      "reference_id": "MONDO:0002675"
    },
    {
      "id": 5243,
      "label": "epithelioid neurofibroma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17201
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:5149",
          "GARD:0023437",
          "ICD9:215.9",
          "MEDGEN:226902",
          "NCIT:C6558",
          "SCTID:404032008",
          "UMLS:C1275264"
        ],
        "synonyms": [
          "epithelioid neurofibroma"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A rare neurofibroma with epithelioid morphology."
      },
      "child_count": 0,
      "reference_id": "MONDO:0003302"
    },
    {
      "id": 5244,
      "label": "neurofibroma of gallbladder",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        7085,
        17201,
        20329,
        24071
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:5150",
          "GARD:0023438",
          "MEDGEN:232540",
          "NCIT:C5746",
          "UMLS:C1333751"
        ],
        "synonyms": [
          "gall bladder neurofibroma",
          "gallbladder neurofibroma",
          "neurofibroma of gall bladder",
          "neurofibroma of gallbladder",
          "neurofibroma of the gallbladder"
        ],
        "categories": [
          {
            "ref": "MONDO:0004335",
            "name": "digestive system disorder"
          },
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A non-metastasizing encapsulated neoplasm arising from nerves in the gallbladder. Morphologically, it is characterized by the presence of fibroblasts and Schwann cells."
      },
      "child_count": 0,
      "reference_id": "MONDO:0003303"
    },
    {
      "id": 5245,
      "label": "plexiform neurofibroma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17201
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:5151",
          "GARD:0023439",
          "HP:0009732",
          "ICD9:215.9",
          "ICDO:9550/0",
          "MEDGEN:64640",
          "MESH:D018318",
          "NCIT:C3797",
          "SCTID:403818001",
          "UMLS:C0206728"
        ],
        "synonyms": [
          "plexiform neurofibroma",
          "plexiform neurofibroma (disease)"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "An elongated and multinodular neurofibroma, formed when the tumor involves either multiple trunks of a plexus or multiple fascicles of a large nerve, such as the sciatic. Some plexiform neurofibromas resemble a bag of worms, others produce a massive ropy enlargement of the nerve. (Adapted from WHO.)"
      },
      "child_count": 0,
      "reference_id": "MONDO:0003304"
    },
    {
      "id": 5246,
      "label": "cellular neurofibroma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17201
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:5152",
          "GARD:0023440",
          "MEDGEN:275699",
          "NCIT:C41427",
          "UMLS:C1516371"
        ],
        "synonyms": [
          "cellular neurofibroma"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A neurofibroma characterized by the presence of areas with increased cellularity."
      },
      "child_count": 0,
      "reference_id": "MONDO:0003305"
    },
    {
      "id": 5247,
      "label": "atypical neurofibroma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17201
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:5153",
          "GARD:0023441",
          "MEDGEN:267320",
          "NCIT:C41426",
          "UMLS:C1510961"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A neurofibroma characterized by the presence of cellular pleomorphism."
      },
      "child_count": 0,
      "reference_id": "MONDO:0003306"
    },
    {
      "id": 6523,
      "label": "neurofibroma of the heart",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        3568,
        17201,
        20329,
        24071
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:9300",
          "GARD:0024101",
          "MEDGEN:242755",
          "NCIT:C5359",
          "UMLS:C1096349"
        ],
        "synonyms": [
          "Cardiac neurofibroma",
          "heart neurofibroma",
          "neurofibroma of heart",
          "neurofibroma of the heart"
        ],
        "categories": [
          {
            "ref": "MONDO:0004995",
            "name": "cardiovascular disorder"
          },
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A rare neurofibroma that affects the heart."
      },
      "child_count": 0,
      "reference_id": "MONDO:0004752"
    },
    {
      "id": 6595,
      "label": "neurofibroma of the esophagus",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17201,
        20522
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:961",
          "GARD:0024117",
          "MEDGEN:232164",
          "NCIT:C5704",
          "UMLS:C1333463"
        ],
        "synonyms": [
          "esophageal neurofibroma",
          "esophagus neurofibroma",
          "neurofibroma of esophagus",
          "neurofibroma of oesophagus",
          "neurofibroma of the esophagus",
          "oesophagus neurofibroma"
        ],
        "categories": [
          {
            "ref": "MONDO:0004335",
            "name": "digestive system disorder"
          },
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A non-metastasizing encapsulated neoplasm arising from nerves in the esophagus. Morphologically, it is characterized by the presence of fibroblasts and Schwann cells."
      },
      "child_count": 0,
      "reference_id": "MONDO:0004837"
    }
  ],
  "roots": [
    {
      "id": 4613,
      "label": "nerve sheath neoplasm"
    },
    {
      "id": 17200,
      "label": "benign peripheral nerve sheath tumor"
    }
  ]
}