{
  "id": 19999,
  "label": "pleuropulmonary blastoma type 3",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0020557",
  "properties": {
    "xrefs": [
      "GARD:0019717",
      "ICD9:162.9",
      "MEDGEN:328132",
      "NCIT:C45628",
      "Orphanet:99935",
      "SCTID:707673006",
      "UMLS:C1710499"
    ],
    "synonyms": [
      "type III pleuropulmonary blastoma"
    ],
    "categories": [
      {
        "ref": "MONDO:0002254",
        "name": "syndromic disease"
      },
      {
        "ref": "MONDO:0005087",
        "name": "respiratory system disorder"
      }
    ],
    "definition": "A pleuropulmonary blastoma characterized by a solid pattern and sarcomatous features. It usually follows an aggressive clinical course."
  },
  "isLeaf": true,
  "isRoot": false,
  "child_count": 0,
  "parents": [
    {
      "id": 12140,
      "label": "pleuropulmonary blastoma",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        7531,
        8005,
        23957
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:4769",
          "EFO:0009052",
          "GARD:0008757",
          "ICD9:162.9",
          "ICDO:8973/3",
          "MEDGEN:266105",
          "MESH:C537516",
          "NANDO:2200080",
          "NCIT:C5669",
          "OMIM:601200",
          "ONCOTREE:PPB",
          "Orphanet:64742",
          "SCTID:707670009",
          "UMLS:C1266144"
        ],
        "synonyms": [
          "childhood pulmonary blastoma",
          "paediatric pulmonary blastoma",
          "pediatric pulmonary blastoma",
          "pleuropulmonary blastoma",
          "pleuropulmonary blastoma (morphologic abnormality)",
          "pulmonary blastoma of childhood",
          "PPB",
          "PPB familial tumor and dysplasia syndrome",
          "PPB familial tumour and dysplasia syndrome"
        ],
        "categories": [
          {
            "ref": "MONDO:0002254",
            "name": "syndromic disease"
          },
          {
            "ref": "MONDO:0005087",
            "name": "respiratory system disorder"
          }
        ],
        "definition": "A malignant neoplasm affecting the lungs and/or the pleura. Pleuropulmonary blastoma is seen in children. Microscopically, the tumor may show features of chondrosarcoma, leiomyosarcoma, rhabdomyosarcoma, liposarcoma, or undifferentiated sarcoma. In approximately 25% of patients with pleuropulmonary blastoma, there are other lesions or neoplasms that may affect patients or their families, including lung or kidney cysts, and ovarian or testicular neoplasms. Heterozygous germline mutations in DICER1 gene have been identified in families harboring pleuropulmonary blastomas."
      },
      "child_count": 12,
      "reference_id": "MONDO:0011014"
    }
  ],
  "children": [],
  "roots": [
    {
      "id": 12140,
      "label": "pleuropulmonary blastoma"
    }
  ]
}