{
  "id": 17182,
  "label": "mixed neuronal-glial tumor",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0016729",
  "properties": {
    "xrefs": [
      "GARD:0020728",
      "MEDGEN:105377",
      "NCIT:C4747",
      "Orphanet:251934",
      "UMLS:C0474844",
      "icd11.foundation:1792897751"
    ],
    "synonyms": [
      "neuronal and Glio-neuronal neoplasm",
      "neuronal and Glio-neuronal tumor",
      "neuronal and Glio-neuronal tumour",
      "neuronal and mixed neuronal-glial tumor",
      "neuronal and mixed neuronal-glial tumors",
      "neuronal and mixed neuronal-glial tumour",
      "neuronal and mixed neuronal-glial tumours"
    ],
    "categories": [
      {
        "ref": "MONDO:0005071",
        "name": "nervous system disorder"
      }
    ],
    "definition": "A group of central nervous system neoplasms with a variable amount of neuronal and, less consistently, glial differentiation. They occur at a low frequency and usually carry a favorable prognosis. Representative examples include dysplastic cerebellar gangliocytoma, desmoplastic infantile ganglioglioma, desmoplastic infantile astrocytoma, and dysembryoplastic neuroepithelial tumor. (Adapted from WHO)"
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 12,
  "parents": [
    {
      "id": 20418,
      "label": "neuroepithelial neoplasm",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        20456
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "MEDGEN:60215",
          "MESH:D018302",
          "NCIT:C3787",
          "ONCOTREE:PRNET",
          "UMLS:C0206715"
        ],
        "synonyms": [
          "neoplasm of neuroepithelial tissue",
          "neoplasm of neuroepithelium",
          "neoplasm of the neuroepithelium",
          "neuroepithelial neoplasm",
          "neuroepithelial neoplasms",
          "neuroepithelial tissue neoplasm",
          "neuroepithelial tissue tumor",
          "neuroepithelial tissue tumour",
          "neuroepithelial tumor",
          "neuroepithelial tumors",
          "neuroepithelial tumour",
          "neuroepithelial tumours",
          "tumor of neuroepithelial tissue",
          "tumor of neuroepithelium",
          "tumor of the neuroepithelium",
          "tumour of neuroepithelial tissue",
          "tumour of neuroepithelium",
          "tumour of the neuroepithelium",
          "primary neuroepithelial tumor",
          "primary neuroepithelial tumour"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A neoplasm of the nervous system that arises from the neuroepithelial tissues. Representative examples include astrocytic tumors, oligodendroglial tumors, ependymal tumors, and primitive neuroectodermal tumors."
      },
      "child_count": 6,
      "reference_id": "MONDO:0021193"
    }
  ],
  "children": [
    {
      "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": 7161,
      "label": "dysembryoplastic neuroepithelial tumor",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17182,
        20429
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:2679",
          "EFO:0005551",
          "GARD:0010640",
          "ICDO:9413/0",
          "MEDGEN:266109",
          "NCIT:C9505",
          "ONCOTREE:DNT",
          "Orphanet:251946",
          "SCTID:87211000119104",
          "UMLS:C1266177"
        ],
        "synonyms": [
          "DNET",
          "DNT",
          "dysembryoplastic neuroepithelial neoplasm",
          "dysembryoplastic neuroepithelial tumor (morphologic abnormality)",
          "dysembryoplastic neuroepithelial tumour (morphologic abnormality)"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A benign glial-neuronal neoplasm. It is usually supratentorial, located, generally, in the cortex and occurs in children and young adults with a long-standing history of partial seizures. A histologic hallmark of this tumor is the 'specific glioneuronal element', characterized by columns, made up of bundles of axons, oriented perpendicularly to the cortical surface.B"
      },
      "child_count": 0,
      "reference_id": "MONDO:0005505"
    },
    {
      "id": 17181,
      "label": "extraventricular neurocytoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17182
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0081314",
          "GARD:0020727",
          "ICDO:9506/1",
          "MEDGEN:457401",
          "NCIT:C92555",
          "ONCOTREE:EVN",
          "Orphanet:251927",
          "SCTID:716787002",
          "UMLS:C2985175"
        ],
        "synonyms": [
          "EVN",
          "extraventricular neurocytoma (WHO grade II)"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "Extraventricular neurocytoma (EVN), a variant of central neurocytoma, is a rare neuronal neoplasm, composed of round cells with neuronal differentiation, which is located outside of the ventricular system, usually within the spinal cord or cerebral hemispheres and that manifests with headache, nausea, vomiting, complex partial seizures or focal neurological deficits. In some cases it may exhibit atypical features consistent with aggressive clinical behavior."
      },
      "child_count": 0,
      "reference_id": "MONDO:0016727"
    },
    {
      "id": 17183,
      "label": "gangliocytoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17182
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:2426",
          "GARD:0010638",
          "ICDO:9492/0",
          "MEDGEN:1830324",
          "MESH:D005729",
          "NANDO:2200097",
          "NCIT:C6934",
          "ONCOTREE:GNC",
          "Orphanet:251937",
          "UMLS:C5779630"
        ],
        "synonyms": [
          "GNC"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A well differentiated, slow growing neuroepithelial neoplasm composed of neoplastic, mature ganglion cells."
      },
      "child_count": 0,
      "reference_id": "MONDO:0016730"
    },
    {
      "id": 17184,
      "label": "desmoplastic infantile astrocytoma/ganglioglioma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17182
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0020729",
          "MEDGEN:1665232",
          "Orphanet:251940",
          "UMLS:C4757951"
        ],
        "synonyms": [
          "DIA/DIG"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "Desmoplastic infantile astrocytoma/ganglioglioma are mixed neuronal-glial tumors representing a histological spectrum of the same tumor. They are usually supratentorially located, large, cystic masses with a peripheral solid component, characterized by prominent desmoplastic stroma and pleomorphic populations of neoplastic cells with either astrocytic or ganglionic differentiation and poorly differentiated cells in variable proportions. They usually present in the first 18 months of age with rapid head growth, bulging anterior fontanel and bone structures over the tumor, signs of raised intracranial pressure (headache, vomiting, papilledema), focal neurological signs and sometimes seizures."
      },
      "child_count": 0,
      "reference_id": "MONDO:0016731"
    },
    {
      "id": 17185,
      "label": "ganglioglioma",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        17182
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:5078",
          "EFO:0003094",
          "GARD:0002430",
          "ICDO:9505/1",
          "MEDGEN:60216",
          "MESH:D018303",
          "MedDRA:10017701",
          "NANDO:2200096",
          "NCIT:C3788",
          "ONCOTREE:GNG",
          "Orphanet:251949",
          "SCTID:87191000119100",
          "UMLS:C0206716",
          "icd11.foundation:1287417975"
        ],
        "synonyms": [
          "ganglioglioma",
          "adult ganglioglioma",
          "childhood ganglioglioma",
          "mixed cell tumors containing both neural ganglionic cells and neural glial cell components",
          "mixed cell tumours containing both neural ganglionic cells and neural glial cell components"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A well differentiated, slow growing neuroepithelial neoplasm composed of neoplastic, mature ganglion cells and neoplastic glial cells. Some gangliogliomas show anaplastic features in their glial component and are considered to be WHO grade III. Rare cases of newly diagnosed gangliogliomas with grade IV (glioblastoma) changes in the glial component have also been reported. (Adapted from WHO)"
      },
      "child_count": 2,
      "reference_id": "MONDO:0016733"
    },
    {
      "id": 17187,
      "label": "papillary glioneuronal tumor",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17182
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0081283",
          "GARD:0020730",
          "ICDO:9509/1",
          "MEDGEN:457400",
          "NCIT:C92554",
          "ONCOTREE:PGNT",
          "Orphanet:251962",
          "UMLS:C2985174"
        ],
        "synonyms": [
          "PGNT",
          "papillary glioneuronal tumor (WHO grade I)",
          "papillary glioneuronal tumour (WHO grade I)",
          "pseudopapillary ganglioglioneurocytoma",
          "pseudopapillary neurocytoma with glial differentiation"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A WHO grade I, indolent and relatively circumscribed brain tumor. Morphologically it is characterized by the presence of astrocytes that line vascular and hyalinized pseudopapillae. In between the pseudopapillae aggregates of neurocytes, large neurons, and ganglioid cells are present."
      },
      "child_count": 0,
      "reference_id": "MONDO:0016735"
    },
    {
      "id": 17188,
      "label": "rosette-forming glioneuronal tumor of fourth ventricule",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17182
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0081284",
          "GARD:0013593",
          "MEDGEN:1377578",
          "NCIT:C129431",
          "Orphanet:251975",
          "UMLS:C4331262"
        ],
        "synonyms": [
          "DNT of the cerebellum",
          "RGNT",
          "dysembryoplastic neuroepithelial tumor of cerebellum",
          "dysembryoplastic neuroepithelial tumour of cerebellum",
          "rosette-forming glioneuronal tumor"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A central nervous system neoplasm mostly occurring in the fourth ventricle region. It is characterized by the presence of neurocytes forming pseudorosettes and astrocytes which contain Rosenthal fibers. Cytologic atypia is minimal."
      },
      "child_count": 0,
      "reference_id": "MONDO:0016736"
    },
    {
      "id": 18917,
      "label": "Lhermitte-Duclos disease",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17182,
        19709
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0006901",
          "ICDO:9493/0",
          "MEDGEN:140251",
          "NCIT:C8419",
          "Orphanet:65285",
          "UMLS:C0391826"
        ],
        "synonyms": [
          "LDD",
          "dysplastic cerebellar gangliocytoma",
          "dysplastic gangliocytoma of cerebellum",
          "dysplastic gangliocytoma of the cerebellum"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "Lhermitte-Duclos disease (LDD) is a very rare disorder characterized by abnormal development and enlargement of the cerebellum, and an increased intracranial pressure."
      },
      "child_count": 0,
      "reference_id": "MONDO:0019002"
    },
    {
      "id": 19014,
      "label": "central neurocytoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        4729,
        17182
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:14174",
          "GARD:0010641",
          "ICDO:9506/1",
          "MEDGEN:104924",
          "MESH:D018306",
          "NCIT:C3791",
          "ONCOTREE:CNC",
          "Orphanet:73256",
          "UMLS:C0206719",
          "icd11.foundation:1247650801",
          "icd11.foundation:2039766091"
        ],
        "synonyms": [
          "central neurocytoma (WHO grade II)",
          "CNC"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "Central neurocytoma is a very rare brain tumor of young adults (over 100 cases reported worldwide). It is typically found in the lateral ventricles and occasionally in the third ventricle. Symptoms are those of increased intracranial pressure: headache, nausea and vomiting, drowsiness, vision problems and mental changes. Total removal of the tumor is the therapy of choice. Post-operative prognosis is generally good."
      },
      "child_count": 0,
      "reference_id": "MONDO:0019134"
    },
    {
      "id": 21071,
      "label": "desmoplastic infantile astrocytoma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17182
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0009617",
          "ICDO:9412/1",
          "MEDGEN:141595",
          "NCIT:C9476",
          "ONCOTREE:DIA",
          "UMLS:C0457179"
        ],
        "synonyms": [
          "DIA",
          "Desmoplastic astrocytoma of infancy",
          "Desmoplastic infantile astrocytoma"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A WHO grade I large cystic tumor that occurs almost exclusively in infants, with a prominent desmoplastic stroma having a neuroepithelial population consisting mainly of neoplastic astrocytes. It involves the superficial cerebral cortex and leptomeninges, and often attaches to the dura. Although clinically it presents as large tumor, it generally has a good prognosis following surgical resection. (Adapted from WHO)"
      },
      "child_count": 0,
      "reference_id": "MONDO:0022963"
    },
    {
      "id": 21072,
      "label": "desmoplastic infantile ganglioglioma",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17182
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0081259",
          "GARD:0008648",
          "MEDGEN:231168",
          "NCIT:C4738",
          "ONCOTREE:DIG",
          "UMLS:C1321878"
        ],
        "synonyms": [
          "DIG",
          "Desmoplastic infantile ganglioglioma"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "A WHO grade I large cystic tumor that occurs almost exclusively in infants, with a prominent desmoplastic stroma having a neuroepithelial population of neoplastic astrocytes together with a variable neuronal component. It involves the superficial cerebral cortex and leptomeninges, and often attaches to the dura. Although clinically it presents as a large tumor, it generally has a good prognosis following surgical resection. (Adapted from WHO)"
      },
      "child_count": 0,
      "reference_id": "MONDO:0022965"
    }
  ],
  "roots": [
    {
      "id": 20418,
      "label": "neuroepithelial neoplasm"
    }
  ]
}