{
  "id": 3043,
  "label": "central nervous system primitive neuroectodermal neoplasm",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0000640",
  "properties": {
    "xrefs": [
      "DOID:0060103",
      "GARD:0027536",
      "MEDGEN:854517",
      "NANDO:2200099",
      "NANDO:2200100",
      "NCIT:C5398",
      "UMLS:C3887678",
      "icd11.foundation:1711526170"
    ],
    "synonyms": [
      "CNS PNET",
      "CNS primitive neuroectodermal neoplasm",
      "CNS primitive neuroectodermal tumor",
      "CNS primitive neuroectodermal tumour",
      "central nervous system PNET",
      "central nervous system neuroectodermal tumor",
      "central nervous system neuroectodermal tumour",
      "central nervous system primitive neuroectodermal neoplasm",
      "central nervous system primitive neuroectodermal tumor",
      "central nervous system primitive neuroectodermal tumor (WHO grade IV)",
      "central nervous system primitive neuroectodermal tumour",
      "central nervous system primitive neuroectodermal tumour (WHO grade IV)",
      "central primitive neuroectodermal neoplasm",
      "central primitive neuroectodermal tumor",
      "central primitive neuroectodermal tumour",
      "primitive neuroectodermal tumor of central nervous system",
      "primitive neuroectodermal tumour of central nervous system"
    ],
    "categories": [
      {
        "ref": "MONDO:0005071",
        "name": "nervous system disorder"
      }
    ],
    "definition": "A neuroectodermal tumor that involves the central nervous system."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 8,
  "parents": [
    {
      "id": 4753,
      "label": "central nervous system cancer",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        7479,
        7694
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:3620",
          "EFO:0000326",
          "GARD:0027614",
          "ICD9:239.7",
          "MEDGEN:87593",
          "MESH:D016543",
          "NANDO:2100007",
          "NCIT:C4627",
          "SCTID:126951006",
          "UMLS:C0348374"
        ],
        "synonyms": [
          "CNS neoplasm",
          "central nervous system tumor",
          "central nervous system tumour",
          "CNS cancer",
          "CNS malignant neoplasms",
          "CNS neoplasms, malignant",
          "cancer of CNS",
          "cancer of central nervous system",
          "cancer of the CNS",
          "cancer of the central nervous system",
          "central nervous system cancer",
          "central nervous system neoplasms, malignant",
          "central nervous system tumours",
          "malignant CNS neoplasm",
          "malignant CNS neoplasms",
          "malignant CNS tumor",
          "malignant CNS tumour",
          "malignant central nervous system neoplasm",
          "malignant central nervous system tumor",
          "malignant central nervous system tumour",
          "malignant neoplasm of CNS",
          "malignant neoplasm of central nervous system",
          "malignant neoplasm of the CNS",
          "malignant neoplasm of the central nervous system",
          "malignant tumor of CNS",
          "malignant tumor of central nervous system",
          "malignant tumor of the CNS",
          "malignant tumor of the central nervous system",
          "malignant tumour of CNS",
          "malignant tumour of central nervous system",
          "malignant tumour of the CNS",
          "malignant tumour of the central nervous system"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A malignant neoplasm involving the central nervous system"
      },
      "child_count": 40,
      "reference_id": "MONDO:0002714"
    },
    {
      "id": 7121,
      "label": "primitive neuroectodermal tumor",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        7212,
        20418
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:171",
          "EFO:0005235",
          "ICDO:9473/3",
          "ICDO:9503/3",
          "MEDGEN:64627",
          "MESH:D017599",
          "NCIT:C3716",
          "ONCOTREE:PNET",
          "UMLS:C0206663"
        ],
        "synonyms": [
          "PNET",
          "neuroectodermal neoplasm",
          "neuroectodermal tumor",
          "neuroectodermal tumour",
          "primitive neuroectodermal neoplasm",
          "primitive neuroectodermal tumor",
          "primitive neuroectodermal tumor (PNET)",
          "primitive neuroectodermal tumour (PNET)",
          "neuroepithelioma"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A malignant neoplasm that originates in the neuroectoderm. The neuroectoderm constitutes the portion of the ectoderm of the early embryo that gives rise to the central and peripheral nervous systems and includes some glial cell precursors."
      },
      "child_count": 10,
      "reference_id": "MONDO:0005462"
    }
  ],
  "children": [
    {
      "id": 4822,
      "label": "adult central nervous system primitive neuroectodermal neoplasm",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        3043
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:3865",
          "GARD:0027620",
          "MEDGEN:231039",
          "NCIT:C5411",
          "UMLS:C1332196"
        ],
        "synonyms": [
          "adult CNS PNET",
          "adult CNS primitive neuroectodermal neoplasm",
          "adult CNS primitive neuroectodermal tumor",
          "adult CNS primitive neuroectodermal tumour",
          "adult central nervous system primitive neuroectodermal neoplasm",
          "adult central nervous system primitive neuroectodermal tumor",
          "adult central nervous system primitive neuroectodermal tumour",
          "adult central primitive neuroectodermal neoplasm",
          "adult central primitive neuroectodermal tumor",
          "adult central primitive neuroectodermal tumour",
          "central nervous system primitive neuroectodermal neoplasm of adults"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A central nervous system embryonal tumor, not otherwise specified that occurs in adults."
      },
      "child_count": 1,
      "reference_id": "MONDO:0002795"
    },
    {
      "id": 4825,
      "label": "childhood central nervous system primitive neuroectodermal neoplasm",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        3043,
        8005
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:3870",
          "GARD:0027621",
          "MEDGEN:232347",
          "NCIT:C5961",
          "UMLS:C1332957"
        ],
        "synonyms": [
          "Central nervous system embryonal tumor, NOS",
          "central nervous system primitive neuroectodermal neoplasm of childhood",
          "childhood CNS PNET",
          "childhood CNS primitive neuroectodermal neoplasm",
          "childhood CNS primitive neuroectodermal tumor",
          "childhood CNS primitive neuroectodermal tumour",
          "childhood central nervous system PNET",
          "childhood central nervous system primitive neuroectodermal neoplasm",
          "childhood central nervous system primitive neuroectodermal tumor",
          "childhood central nervous system primitive neuroectodermal tumour",
          "childhood central primitive neuroectodermal neoplasm",
          "childhood central primitive neuroectodermal tumor",
          "childhood central primitive neuroectodermal tumour",
          "paediatric CNS PNET",
          "paediatric CNS primitive neuroectodermal neoplasm",
          "paediatric CNS primitive neuroectodermal tumour",
          "paediatric central nervous system primitive neuroectodermal neoplasm",
          "paediatric central nervous system primitive neuroectodermal tumour",
          "paediatric central primitive neuroectodermal neoplasm",
          "paediatric central primitive neuroectodermal tumour",
          "pediatric CNS PNET",
          "pediatric CNS primitive neuroectodermal neoplasm",
          "pediatric CNS primitive neuroectodermal tumor",
          "pediatric central nervous system primitive neuroectodermal neoplasm",
          "pediatric central nervous system primitive neuroectodermal tumor",
          "pediatric central primitive neuroectodermal neoplasm",
          "pediatric central primitive neuroectodermal tumor"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A central nervous system embryonal tumor, not otherwise specified that occurs in childhood."
      },
      "child_count": 4,
      "reference_id": "MONDO:0002798"
    },
    {
      "id": 5116,
      "label": "intracranial primitive neuroectodermal tumor",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        3043,
        3853
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:4788",
          "GARD:0027631",
          "MEDGEN:577349",
          "NCIT:C5817",
          "UMLS:C0346292"
        ],
        "synonyms": [
          "brain primitive neuroectodermal tumor",
          "brain primitive neuroectodermal tumour",
          "intracranial primitive neuroectodermal neoplasm",
          "primitive neuroectodermal tumor of brain",
          "primitive neuroectodermal tumour of brain"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A primitive neuroectodermal tumor that involves the brain."
      },
      "child_count": 8,
      "reference_id": "MONDO:0003142"
    },
    {
      "id": 5871,
      "label": "spinal cord neuroblastoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        3043,
        4784,
        5449
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:6871",
          "GARD:0023783",
          "MEDGEN:234803",
          "NCIT:C5155",
          "UMLS:C1336046"
        ],
        "synonyms": [
          "neuroblastoma of spinal cord",
          "spinal cord neuroblastoma"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A neuroblastoma that affects the spinal cord."
      },
      "child_count": 0,
      "reference_id": "MONDO:0004024"
    },
    {
      "id": 6767,
      "label": "ganglioneuroma",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        3043,
        4464,
        7851,
        17182,
        20329,
        23497
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:4817",
          "EFO:0000500",
          "GARD:0020731",
          "HP:0003005",
          "ICD9:215.9",
          "ICDO:9490/0",
          "MEDGEN:6545",
          "MedDRA:10017709",
          "NCIT:C3049",
          "ONCOTREE:GN",
          "Orphanet:251992",
          "SCTID:116371000119107",
          "UMLS:C0017075",
          "birnlex:12617"
        ],
        "synonyms": [
          "ganglioneuroma",
          "ganglioneuroma (Schwannian Stroma-dominant)",
          "ganglioneuroma (disease)",
          "ganglioneuroma, benign",
          "neural Crest tumor, benign",
          "GN"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A benign neuroblastic tumor of the sympathetic nervous system that occurs in childhood. Ganglioneuroma typically arises from the sympathetic trunk in the mediastinum. Histologic features include spindle cell proliferation (resembling a neurofibroma) and the presence of large ganglion cells. Common presenting features include a palpable abdominal mass, hepatomegaly, and a thoracic mass detected on routine chest X-ray."
      },
      "child_count": 6,
      "reference_id": "MONDO:0005033"
    },
    {
      "id": 7942,
      "label": "spinal cord primitive neuroectodermal tumor",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        3043,
        5449
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:6872",
          "EFO:1000545",
          "GARD:0027755",
          "MEDGEN:234805",
          "NCIT:C5406",
          "UMLS:C1336048"
        ],
        "synonyms": [
          "primitive neuroectodermal neoplasm of spinal cord",
          "primitive neuroectodermal neoplasm of the spinal cord",
          "primitive neuroectodermal tumor of spinal cord",
          "primitive neuroectodermal tumor of the spinal cord",
          "primitive neuroectodermal tumour of spinal cord",
          "primitive neuroectodermal tumour of the spinal cord",
          "spinal cord PNET",
          "spinal cord primitive neuroectodermal neoplasm",
          "spinal cord primitive neuroectodermal tumor"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A central nervous system embryonal tumor, not otherwise specified arising from the spinal cord."
      },
      "child_count": 0,
      "reference_id": "MONDO:0006426"
    },
    {
      "id": 17174,
      "label": "ependymoblastoma",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        3043,
        17173
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0080903",
          "DOID:4794",
          "GARD:0020720",
          "MEDGEN:152150",
          "MedDRA:10014966",
          "NCIT:C4915",
          "ONCOTREE:ETANTR",
          "Orphanet:251880",
          "SCTID:715901002",
          "UMLS:C0700367"
        ],
        "synonyms": [
          "ETMR, C19MC-altered",
          "embryonal tumor with Multilayered Rosettes",
          "embryonal tumor with Multilayered Rosettes with C19MC amplification",
          "embryonal tumor with Multilayered Rosettes, C19MC-altered",
          "embryonal tumor with abundant neuropil and true Rosettes",
          "embryonal tumour with Multilayered Rosettes",
          "embryonal tumour with Multilayered Rosettes with C19MC amplification",
          "embryonal tumour with Multilayered Rosettes, C19MC-altered",
          "embryonal tumour with abundant neuropil and true Rosettes",
          "ependymoblastoma",
          "ETANTR",
          "ETMR",
          "embryonal tumor with abundant neuropil and true rosettes",
          "embryonal tumour with abundant neuropil and true rosettes",
          "neuroectodermal tumors primitive",
          "neuroectodermal tumours primitive"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "Ependymoblastoma is a rare type of primitive neuroectodermal tumor (PNET) that usually occurs in young children under the age of 2 and is histologically distinguished by the production of ependymoblastic rosettes. It is associated with an aggressive course and a poor prognosis."
      },
      "child_count": 6,
      "reference_id": "MONDO:0016715"
    },
    {
      "id": 25262,
      "label": "central nervous system tumor with bcor internal tandem duplication",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        3043
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0081315",
          "GARD:0028073",
          "MEDGEN:1811171",
          "NCIT:C186556",
          "UMLS:C5670630"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A central nervous system embryonal tumor that is characterized by the presence of uniform oval or spindle-shaped cells with round or oval nuclei, pseudorosette formation, and heterozygous internal tandem duplication in exon 15 of the BCOR gene."
      },
      "child_count": 0,
      "reference_id": "MONDO:0858966"
    }
  ],
  "roots": [
    {
      "id": 4753,
      "label": "central nervous system cancer"
    },
    {
      "id": 7121,
      "label": "primitive neuroectodermal tumor"
    }
  ]
}