{
  "id": 18046,
  "label": "malignant peripheral nerve sheath tumor",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0017827",
  "properties": {
    "xrefs": [
      "DOID:5940",
      "EFO:0000760",
      "GARD:0010872",
      "ICD9:171.9",
      "ICDO:9540/3",
      "ICDO:9560/3",
      "MEDGEN:155614",
      "MedDRA:10029236",
      "NANDO:2200102",
      "NCIT:C3798",
      "ONCOTREE:MPNST",
      "Orphanet:3148",
      "SCTID:404037002",
      "UMLS:C0751690",
      "icd11.foundation:71413945"
    ],
    "synonyms": [
      "neurofibrosarcoma",
      "MPNST",
      "Malig. periph. nerve sheath tum.",
      "malignant neoplasm of peripheral nerve sheath",
      "malignant neurilemmoma",
      "malignant neurilemoma",
      "malignant neurofibroma",
      "malignant peripheral nerve sheath neoplasm",
      "malignant peripheral nerve sheath tumor",
      "malignant peripheral nerve sheath tumor (morphologic abnormality)",
      "malignant peripheral nerve sheath tumor [dup] (morphologic abnormality)",
      "malignant peripheral nerve sheath tumour (morphologic abnormality)",
      "malignant peripheral nerve sheath tumour [dup] (morphologic abnormality)",
      "malignant schwannoma",
      "malignant tumor of peripheral nerve sheath",
      "malignant tumor of the peripheral nerve sheath",
      "malignant tumour of peripheral nerve sheath",
      "malignant tumour of the peripheral nerve sheath",
      "neurofibrosarcoma, malignant",
      "neurogenic sarcoma",
      "schwannoma, malignant"
    ],
    "categories": [
      {
        "ref": "MONDO:0005071",
        "name": "nervous system disorder"
      }
    ],
    "definition": "Malignant peripheral nerve sheath tumor (MPNST) is a rare and often aggressive soft tissue sarcoma occurring in a wide range of anatomical sites."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 7,
  "parents": [
    {
      "id": 4336,
      "label": "central nervous system sarcoma",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        4753,
        18239
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:2133",
          "GARD:0023088",
          "MEDGEN:272444",
          "NCIT:C5153",
          "UMLS:C1332892"
        ],
        "synonyms": [
          "CNS sarcoma",
          "central nervous system sarcoma",
          "sarcoma of CNS",
          "sarcoma of central nervous system",
          "sarcoma of the CNS",
          "sarcoma of the central nervous system"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A sarcoma that arises from the central nervous system."
      },
      "child_count": 24,
      "reference_id": "MONDO:0002217"
    },
    {
      "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": 17197,
      "label": "tumor of cranial and spinal nerves",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        20456
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0012697",
          "MEDGEN:1842705",
          "Orphanet:252057",
          "UMLS:C5679636"
        ],
        "synonyms": [
          "rare tumor of cranial and spinal nerves",
          "rare tumour of cranial and spinal nerves"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ]
      },
      "child_count": 4,
      "reference_id": "MONDO:0016749"
    },
    {
      "id": 20329,
      "label": "peripheral nervous system cancer",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        3627,
        7479
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "ICD9:171.9",
          "MEDGEN:155556",
          "NCIT:C4961",
          "SCTID:254986007",
          "UMLS:C0751428"
        ],
        "synonyms": [
          "cancer of peripheral nervous system",
          "malignant PNS neoplasm",
          "malignant PNS tumor",
          "malignant PNS tumour",
          "malignant neoplasm of PNS",
          "malignant neoplasm of peripheral nerve",
          "malignant neoplasm of peripheral nervous system",
          "malignant neoplasm of the PNS",
          "malignant neoplasm of the peripheral nerve",
          "malignant neoplasm of the peripheral nervous system",
          "malignant neoplasms, peripheral nerve",
          "malignant peripheral nerve neoplasm",
          "malignant peripheral nerve tumor",
          "malignant peripheral nerve tumour",
          "malignant peripheral nervous system neoplasm",
          "malignant peripheral nervous system tumor",
          "malignant peripheral nervous system tumour",
          "malignant tumor of PNS",
          "malignant tumor of peripheral nerve",
          "malignant tumor of peripheral nervous system",
          "malignant tumor of the PNS",
          "malignant tumor of the peripheral nerve",
          "malignant tumor of the peripheral nervous system",
          "malignant tumour of PNS",
          "malignant tumour of peripheral nerve",
          "malignant tumour of peripheral nervous system",
          "malignant tumour of the PNS",
          "malignant tumour of the peripheral nerve",
          "malignant tumour of the peripheral nervous system",
          "peripheral nervous system cancer",
          "peripheral nervous system neoplasms, malignant"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "Malignant growth of cells in the peripheral nervous system (PNS)or Autonomic Nervous System (ANS), without specification as to location"
      },
      "child_count": 36,
      "reference_id": "MONDO:0021089"
    },
    {
      "id": 24071,
      "label": "malignant glioma",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        4753,
        20078,
        20287
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:3070",
          "GARD:0026148",
          "ICDO:9380/3",
          "MEDGEN:107826",
          "MedDRA:10018338",
          "NCIT:C4822",
          "UMLS:C0555198"
        ],
        "synonyms": [
          "glioma",
          "neuroglial tumor",
          "neuroglial tumour",
          "glial cell tumour",
          "glioma, malignant",
          "high grade glioma",
          "high-grade glioma",
          "malignant glial neoplasm",
          "malignant glial tumor",
          "malignant glial tumour",
          "malignant glioma",
          "malignant neuroglial neoplasm",
          "malignant neuroglial tumor",
          "malignant neuroglial tumour"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A grade III or grade IV glioma arising from the central nervous system. This category includes glioblastoma, anaplastic astrocytoma, anaplastic ependymoma, anaplastic oligodendroglioma, and anaplastic oligoastrocytoma."
      },
      "child_count": 36,
      "reference_id": "MONDO:0100342"
    }
  ],
  "children": [
    {
      "id": 4149,
      "label": "malignant cardiac peripheral nerve sheath neoplasm",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        5289,
        18046
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:14534",
          "GARD:0023052",
          "MEDGEN:233146",
          "NCIT:C5367",
          "UMLS:C1334569"
        ],
        "synonyms": [
          "CARDIAC schwannoma, malignant",
          "Cardiac MPNST",
          "Cardiac malignant peripheral nerve sheath tumor",
          "Cardiac malignant peripheral nerve sheath tumour",
          "MPNST of heart",
          "MPNST of the heart",
          "heart MPNST",
          "heart malignant peripheral nerve sheath tumor",
          "heart malignant peripheral nerve sheath tumour",
          "malignant Cardiac neurilemmoma",
          "malignant Cardiac peripheral nerve sheath neoplasm",
          "malignant Cardiac peripheral nerve sheath tumor",
          "malignant Cardiac peripheral nerve sheath tumour",
          "malignant Cardiac schwannoma",
          "malignant heart neurilemmoma",
          "malignant heart peripheral nerve sheath neoplasm",
          "malignant heart peripheral nerve sheath tumor",
          "malignant heart peripheral nerve sheath tumour",
          "malignant heart schwannoma",
          "malignant neurilemmoma of heart",
          "malignant neurilemmoma of the heart",
          "malignant peripheral nerve sheath neoplasm of heart",
          "malignant peripheral nerve sheath neoplasm of the heart",
          "malignant peripheral nerve sheath tumor of heart",
          "malignant peripheral nerve sheath tumor of the heart",
          "malignant peripheral nerve sheath tumour of heart",
          "malignant peripheral nerve sheath tumour of the heart",
          "malignant schwannoma of heart",
          "malignant schwannoma of the heart"
        ],
        "categories": [
          {
            "ref": "MONDO:0004995",
            "name": "cardiovascular disorder"
          },
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A very rare malignant peripheral nerve sheath tumor that arises from the heart."
      },
      "child_count": 0,
      "reference_id": "MONDO:0001990"
    },
    {
      "id": 5728,
      "label": "malignant melanocytic neoplasm of the peripheral nerve sheath",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        18046
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:6345",
          "GARD:0023703",
          "ICD9:171.9",
          "MEDGEN:99184",
          "NCIT:C4748",
          "SCTID:404039004",
          "UMLS:C0474847"
        ],
        "synonyms": [
          "malignant melanocytic neoplasm of peripheral nerve sheath",
          "malignant melanocytic neoplasm of the peripheral nerve sheath",
          "malignant melanocytic peripheral nerve sheath tumor",
          "malignant melanocytic peripheral nerve sheath tumour",
          "malignant melanotic peripheral nerve sheath tumor",
          "malignant melanotic peripheral nerve sheath tumour",
          "melanocytic MPNST",
          "melanotic malignant nerve sheath tumour",
          "melanotic malignant peripheral nerve sheath tumor (morphologic abnormality)",
          "melanotic malignant peripheral nerve sheath tumour (morphologic abnormality)"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A rare variant of malignant peripheral nerve sheath tumor. It is characterized by the presence of malignant cells that contain melanin."
      },
      "child_count": 2,
      "reference_id": "MONDO:0003863"
    },
    {
      "id": 6160,
      "label": "childhood malignant schwannoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        8005,
        18046
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:7732",
          "GARD:0023948",
          "MEDGEN:83582",
          "NCIT:C8094",
          "UMLS:C0279987"
        ],
        "synonyms": [
          "malignant peripheral nerve sheath tumor",
          "malignant peripheral nerve sheath tumour",
          "childhood MPNST",
          "childhood malignant neoplasm of peripheral nerve sheath",
          "childhood malignant neoplasm of the peripheral nerve sheath",
          "childhood malignant neurilemmoma",
          "childhood malignant peripheral nerve sheath neoplasm",
          "childhood malignant peripheral nerve sheath tumor",
          "childhood malignant peripheral nerve sheath tumour",
          "childhood malignant schwannoma",
          "childhood malignant tumor of peripheral nerve sheath",
          "childhood malignant tumor of the peripheral nerve sheath",
          "childhood malignant tumour of peripheral nerve sheath",
          "childhood malignant tumour of the peripheral nerve sheath",
          "childhood neurofibrosarcoma",
          "childhood neurogenic sarcoma",
          "paediatric MPNST",
          "paediatric malignant neoplasm of peripheral nerve sheath",
          "paediatric malignant neoplasm of the peripheral nerve sheath",
          "paediatric malignant neurilemmoma",
          "paediatric malignant peripheral nerve sheath neoplasm",
          "paediatric malignant peripheral nerve sheath tumour",
          "paediatric malignant schwannoma",
          "paediatric malignant tumour of peripheral nerve sheath",
          "paediatric malignant tumour of the peripheral nerve sheath",
          "pediatric MPNST",
          "pediatric malignant neoplasm of peripheral nerve sheath",
          "pediatric malignant neoplasm of the peripheral nerve sheath",
          "pediatric malignant neurilemmoma",
          "pediatric malignant peripheral nerve sheath neoplasm",
          "pediatric malignant peripheral nerve sheath tumor",
          "pediatric malignant schwannoma",
          "pediatric malignant tumor of peripheral nerve sheath",
          "pediatric malignant tumor of the peripheral nerve sheath"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A malignant peripheral nerve sheath tumor occurring in children."
      },
      "child_count": 0,
      "reference_id": "MONDO:0004345"
    },
    {
      "id": 6339,
      "label": "epithelioid malignant peripheral nerve sheath tumor",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18046
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:8353",
          "GARD:0024055",
          "MEDGEN:272104",
          "NCIT:C6561",
          "SCTID:404038007",
          "UMLS:C1321427"
        ],
        "synonyms": [
          "epithelioid MPNST",
          "epithelioid malignant peripheral nerve sheath tumor",
          "malignant epithelioid neoplasm of peripheral nerve sheath",
          "malignant epithelioid neoplasm of the peripheral nerve sheath",
          "malignant epithelioid peripheral nerve sheath neoplasm",
          "malignant epithelioid peripheral nerve sheath tumor",
          "malignant epithelioid peripheral nerve sheath tumour",
          "malignant epithelioid tumor of peripheral nerve sheath",
          "malignant epithelioid tumor of the peripheral nerve sheath",
          "malignant epithelioid tumour of peripheral nerve sheath",
          "malignant epithelioid tumour of the peripheral nerve sheath"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A rare variant of malignant peripheral nerve sheath tumor composed predominantly or exclusively of epithelioid cells."
      },
      "child_count": 0,
      "reference_id": "MONDO:0004540"
    },
    {
      "id": 6344,
      "label": "adult malignant schwannoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18046
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:8369",
          "GARD:0024059",
          "MEDGEN:75845",
          "NCIT:C7814",
          "UMLS:C0278622"
        ],
        "synonyms": [
          "malignant peripheral nerve sheath tumor",
          "malignant peripheral nerve sheath tumour",
          "adult MPNST",
          "adult malignant neoplasm of peripheral nerve sheath",
          "adult malignant neoplasm of the peripheral nerve sheath",
          "adult malignant neurilemmoma",
          "adult malignant peripheral nerve sheath neoplasm",
          "adult malignant peripheral nerve sheath tumor",
          "adult malignant peripheral nerve sheath tumour",
          "adult malignant schwannoma",
          "adult malignant tumor of peripheral nerve sheath",
          "adult malignant tumor of the peripheral nerve sheath",
          "adult malignant tumour of peripheral nerve sheath",
          "adult malignant tumour of the peripheral nerve sheath"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A malignant peripheral nerve sheath tumor occurring during adulthood."
      },
      "child_count": 0,
      "reference_id": "MONDO:0004545"
    },
    {
      "id": 6357,
      "label": "malignant glandular tumor of peripheral nerve sheath",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18046
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:8420",
          "GARD:0024069",
          "ICD9:171.9",
          "MEDGEN:232558",
          "NCIT:C6560",
          "SCTID:699659007",
          "UMLS:C1333821"
        ],
        "synonyms": [
          "glandular MPNST",
          "glandular malignant peripheral nerve sheath tumor",
          "glandular malignant peripheral nerve sheath tumour",
          "malignant glandular neoplasm of peripheral nerve sheath",
          "malignant glandular neoplasm of the peripheral nerve sheath",
          "malignant glandular peripheral nerve sheath neoplasm",
          "malignant glandular peripheral nerve sheath tumor",
          "malignant glandular peripheral nerve sheath tumour",
          "malignant glandular schwannoma",
          "malignant glandular tumor of peripheral nerve sheath",
          "malignant glandular tumor of the peripheral nerve sheath",
          "malignant glandular tumour of the peripheral nerve sheath"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A variant of malignant peripheral nerve sheath tumor characterized by the presence of glandular epithelium."
      },
      "child_count": 0,
      "reference_id": "MONDO:0004559"
    },
    {
      "id": 17202,
      "label": "malignant triton tumor",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18046
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:6707",
          "GARD:0020743",
          "ICD9:171.9",
          "ICDO:9561/3",
          "MEDGEN:90816",
          "NCIT:C4335",
          "Orphanet:252212",
          "SCTID:404040002",
          "UMLS:C0334616"
        ],
        "synonyms": [
          "MPNST with rhabdomyosarcoma",
          "MPNST with rhabdomyosarcomatous differentiation",
          "MTT",
          "malignant Triton tumor",
          "malignant neoplasm of peripheral nerve sheath with rhabdomyosarcoma",
          "malignant neoplasm of the peripheral nerve sheath with rhabdomyosarcoma",
          "malignant peripheral nerve sheath neoplasm with rhabdomyosarcoma",
          "malignant peripheral nerve sheath tumor with rhabdomyosarcoma",
          "malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differenciation",
          "malignant peripheral nerve sheath tumour with rhabdomyosarcoma",
          "malignant peripheral nerve sheath tumour with rhabdomyosarcomatous differenciation",
          "malignant tumor of peripheral nerve sheath with rhabdomyosarcoma",
          "malignant tumor of the peripheral nerve sheath with rhabdomyosarcoma",
          "malignant tumour of peripheral nerve sheath with rhabdomyosarcoma",
          "malignant tumour of the peripheral nerve sheath with rhabdomyosarcoma"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "Malignant triton tumor (MTT) is a rare aggressive subtype of malignant peripheral nerve sheath tumor (MPNST) characterized histopathologically by focal rhabdomyoblastic differentiation."
      },
      "child_count": 0,
      "reference_id": "MONDO:0016757"
    }
  ],
  "roots": [
    {
      "id": 4336,
      "label": "central nervous system sarcoma"
    },
    {
      "id": 4613,
      "label": "nerve sheath neoplasm"
    },
    {
      "id": 17197,
      "label": "tumor of cranial and spinal nerves"
    },
    {
      "id": 20329,
      "label": "peripheral nervous system cancer"
    },
    {
      "id": 24071,
      "label": "malignant glioma"
    }
  ]
}