{
  "id": 9488,
  "label": "extramammary Paget disease",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0008177",
  "properties": {
    "xrefs": [
      "EFO:1000249",
      "GARD:0004192",
      "ICDO:8542/3",
      "MEDGEN:45280",
      "MESH:D010145",
      "MedDRA:10033366",
      "MedDRA:10068223",
      "NCIT:C3302",
      "OMIM:167300",
      "ONCOTREE:EMPD",
      "Orphanet:2800",
      "UMLS:C0030186",
      "icd11.foundation:1796624917"
    ],
    "synonyms": [
      "Extramammary Paget's disease",
      "Paget disease Extramammary",
      "Paget's disease of skin",
      "Paget's disease of the skin",
      "Paget's skin disease",
      "extramammary Paget disease",
      "EMPD",
      "Paget disease, EXTRAMAMMARY",
      "cutaneous Paget's disease"
    ],
    "definition": "A malignant neoplasm in which there is infiltration of the skin by neoplastic large cells with abundant pale cytoplasm and large nuclei with prominent nucleoli (Paget cells). It may affect the anus, penis, scrotum, and vulva."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 3,
  "parents": [
    {
      "id": 20398,
      "label": "Paget disease",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6712
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "MEDGEN:277958",
          "NCIT:C7073",
          "UMLS:C1368019"
        ],
        "synonyms": [
          "Paget cell neoplasm",
          "Paget disease",
          "Paget's cell neoplasm",
          "Paget's disease"
        ],
        "definition": "A malignant neoplasm composed of large cells with large nuclei, prominent nucleoli, and abundant pale cytoplasm (Paget cells). Paget cell neoplasms include Paget disease of the nipple and extramammary Paget disease which may affect the vulva, penis, anus, skin and scrotum."
      },
      "child_count": 5,
      "reference_id": "MONDO:0021165"
    }
  ],
  "children": [
    {
      "id": 4330,
      "label": "vulval Paget disease",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        9488,
        21365
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:2097",
          "GARD:0023083",
          "ICD9:233.39",
          "MEDGEN:266154",
          "NCIT:C4027",
          "SCTID:254898001",
          "UMLS:C1275217"
        ],
        "synonyms": [
          "Paget disease of the vulva",
          "Paget's disease of the vulva",
          "Paget's disease of vulva",
          "mammalian vulva Paget disease",
          "vulva Paget's disease",
          "vulval Paget's disease",
          "vulvar Paget's disease"
        ],
        "categories": [
          {
            "ref": "MONDO:0005039",
            "name": "reproductive system disorder"
          }
        ],
        "definition": "An uncommon intraepithelial malignant neoplasm of eccrine or apocrine origin, arising from the vulva. It usually affects post-menopausal women. In approximately 10-20% of the cases there is an associated anorectal, or urothelial carcinoma or a skin appendage adenocarcinoma identified. It presents as a red, eczematous lesion. Microscopically, it is characterized by the presence of the typical Paget cells which are large, round cells with abundant cytoplasm and prominent nuclei."
      },
      "child_count": 0,
      "reference_id": "MONDO:0002207"
    },
    {
      "id": 4702,
      "label": "anal Paget disease",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        4703,
        9488
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:3446",
          "GARD:0023203",
          "MEDGEN:233981",
          "NCIT:C5598",
          "UMLS:C1332274"
        ],
        "synonyms": [
          "Paget disease of the anus",
          "Paget's disease of anus",
          "Paget's disease of the anus",
          "anal Paget disease",
          "anal Paget's disease",
          "anus Paget disease",
          "anus mammary Paget's disease"
        ],
        "categories": [
          {
            "ref": "MONDO:0004335",
            "name": "digestive system disorder"
          }
        ],
        "definition": "A slowly spreading, erythematous eczematoid plaque in the anal region. Histologically, the basal part or whole thickness of the squamous epithelium is infiltrated by large cells with abundant pale cytoplasm and large nuclei. Half of the cases are associated with an internal malignancy, most often a colorectal adenocarcinoma. The other half of the cases, have a high local recurrence rate and they may become invasive (WHO)."
      },
      "child_count": 4,
      "reference_id": "MONDO:0002651"
    },
    {
      "id": 4704,
      "label": "Paget disease of the penis",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        7886,
        9488
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:3448",
          "GARD:0021638",
          "MEDGEN:66374",
          "NCIT:C27817",
          "Orphanet:398053",
          "SCTID:398768004",
          "UMLS:C0221286"
        ],
        "synonyms": [
          "Paget disease of the penis",
          "Paget's disease of penis",
          "Paget's disease of the penis",
          "adenocarcinoma of penis",
          "penile adenocarcinoma",
          "penis Paget disease",
          "penis adenocarcinoma",
          "penis mammary Paget's disease",
          "adenocarcinoma of the penis",
          "penis Paget's disease"
        ],
        "categories": [
          {
            "ref": "MONDO:0005039",
            "name": "reproductive system disorder"
          }
        ],
        "definition": "A premalignant condition morphologically characterized by the presence of the characteristic Paget cells in the intraepithelial tissue of the penis. It presents as a smooth raised reddish area that may or may not be painful. -- 2003"
      },
      "child_count": 0,
      "reference_id": "MONDO:0002653"
    }
  ],
  "roots": [
    {
      "id": 20398,
      "label": "Paget disease"
    }
  ]
}