{
  "id": 19314,
  "label": "neuroendocrine neoplasm",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0019496",
  "properties": {
    "xrefs": [
      "DOID:169",
      "EFO:1001901",
      "GARD:0009316",
      "ICD9:209",
      "ICD9:209-209",
      "ICD9:239.7",
      "MEDGEN:64652",
      "MESH:D018358",
      "NCIT:C188218",
      "NCIT:C3809",
      "Orphanet:877",
      "SCTID:255046005",
      "UMLS:C0206754"
    ],
    "synonyms": [
      "APUDoma",
      "neuroendocrine neoplasm",
      "neuroendocrine tumor",
      "neuroendocrine tumour"
    ],
    "categories": [
      {
        "ref": "MONDO:0005151",
        "name": "endocrine system disorder"
      }
    ],
    "definition": "Endocrine tumors, also referred to as neuroendocrine tumors (NETs), are defined by a common phenotype which is characterized by the expression of general markers (neuron specific enolase, chromogranin, synaptophysin) and hormone secretion products. These tumors may be localized in any part of the body and are generally discovered in non-specific situations, i.e. not immediately suggestive of NETs (tests for inherited predisposition to tumors or for a clinical syndrome caused by abnormal hormone secretion)."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 14,
  "parents": [
    {
      "id": 4223,
      "label": "endocrine gland neoplasm",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6798,
        6875
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "EFO:0003769",
          "ICD9:239.7",
          "MEDGEN:4044",
          "NCIT:C3010",
          "SCTID:387922007",
          "UMLS:C0014132"
        ],
        "synonyms": [
          "endocrine gland neoplasm (disease)",
          "endocrine gland tumor",
          "endocrine gland tumour",
          "endocrine neoplasm",
          "endocrine system neoplasm",
          "endocrine system tumor",
          "endocrine system tumour",
          "endocrine tumor",
          "endocrine tumour",
          "neoplasm of endocrine gland",
          "tumor of endocrine gland",
          "tumour of endocrine gland",
          "malignant endocrine tumor",
          "malignant endocrine tumour"
        ],
        "categories": [
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          }
        ],
        "definition": "A benign or malignant neoplasm arising from the epithelial cells of an endocrine organ. Representative examples include pituitary gland adenoma, pituitary gland carcinoma, thyroid gland carcinoma, carcinoid tumor, and neuroendocrine carcinoma."
      },
      "child_count": 28,
      "reference_id": "MONDO:0002082"
    }
  ],
  "children": [
    {
      "id": 2912,
      "label": "paraganglioma",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        4464,
        19314,
        20691,
        24270
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0050773",
          "EFO:1000453",
          "GARD:0022770",
          "ICD9:239.7",
          "ICDO:8680/1",
          "MEDGEN:10571",
          "MESH:D010235",
          "NCIT:C3308",
          "ONCOTREE:PGNG",
          "SCTID:127027008",
          "UMLS:C0030421"
        ],
        "synonyms": [
          "Paraganglionic neoplasm",
          "Paraganglionic tumor",
          "Paraganglionic tumour",
          "neoplasm of paraganglion",
          "neoplasm of the paraganglion",
          "paraganglioma",
          "paragangliomas",
          "paraganglion neoplasm",
          "paraganglion tumor",
          "paraganglion tumour",
          "tumor of paraganglion",
          "tumor of the paraganglion",
          "tumour of paraganglion",
          "tumour of the paraganglion",
          "chemodectoma"
        ],
        "categories": [
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          }
        ],
        "definition": "A benign or malignant neoplasm arising from paraganglia located along the sympathetic or parasympathetic nerves. Infrequently, it may arise outside the usual distribution of the sympathetic and parasympathetic paraganglia. Tumors arising from the adrenal gland medulla are called pheochromocytomas. Morphologically, paragangliomas usually display a nesting (Zellballen) growth pattern. There are no reliable morphologic criteria to distinguish between benign and malignant paragangliomas. The only definitive indicator of malignancy is the presence of regional or distant metastases."
      },
      "child_count": 48,
      "reference_id": "MONDO:0000448"
    },
    {
      "id": 4255,
      "label": "neuroendocrine carcinoma",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6734,
        19314,
        20310
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:1800",
          "GARD:0023067",
          "ICDO:8246/3",
          "MEDGEN:104919",
          "MESH:D018278",
          "NCIT:C3773",
          "SCTID:253000007",
          "UMLS:C0206695"
        ],
        "synonyms": [
          "NEC",
          "neuroendocrine cancer",
          "neuroendocrine carcinoma"
        ],
        "categories": [
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          }
        ],
        "definition": "A malignant neuroendocrine neoplasm composed of cells containing secretory granules that stain positive for NSE and chromogranin. The neoplastic cells are often round and form clusters or trabecular sheets. Representative examples are small cell carcinoma, large cell neuroendocrine carcinoma, and Merkel cell carcinoma."
      },
      "child_count": 36,
      "reference_id": "MONDO:0002120"
    },
    {
      "id": 4557,
      "label": "prostate neuroendocrine neoplasm",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        19314,
        20463
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:2992",
          "GARD:0023144",
          "MEDGEN:277537",
          "NCIT:C5545",
          "ONCOTREE:PRNE",
          "UMLS:C1335515"
        ],
        "synonyms": [
          "neuroendocrine neoplasm of prostate",
          "neuroendocrine neoplasm of prostate gland",
          "neuroendocrine neoplasm of the prostate",
          "neuroendocrine tumour of the prostate",
          "prostate gland NET",
          "prostate gland neuroendocrine neoplasm",
          "prostate gland neuroendocrine tumor",
          "prostate gland neuroendocrine tumor, well differentiated, low or intermediate grade",
          "prostate gland neuroendocrine tumour",
          "prostate neuroendocrine neoplasm",
          "prostate neuroendocrine carcinoma"
        ],
        "categories": [
          {
            "ref": "MONDO:0005039",
            "name": "reproductive system disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          }
        ],
        "definition": "A neoplasm with neuroendocrine differentiation that arises from the prostate gland. This category includes carcinoid tumors and small cell carcinomas."
      },
      "child_count": 2,
      "reference_id": "MONDO:0002477"
    },
    {
      "id": 4561,
      "label": "ovarian neuroendocrine neoplasm",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        9481,
        19314,
        20310
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:3002",
          "GARD:0023145",
          "MEDGEN:277459",
          "NCIT:C5237",
          "UMLS:C1335172"
        ],
        "synonyms": [
          "neuroendocrine neoplasm of ovary",
          "neuroendocrine neoplasm of the ovary",
          "neuroendocrine tumour of ovary",
          "ovarian neuroendocrine neoplasm",
          "ovary NET",
          "ovary neuroendocrine neoplasm",
          "ovary neuroendocrine tumor",
          "ovary neuroendocrine tumor, well differentiated, low or intermediate grade",
          "ovary neuroendocrine tumour"
        ],
        "categories": [
          {
            "ref": "MONDO:0005039",
            "name": "reproductive system disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          }
        ],
        "definition": "An epithelial neoplasm with neuroendocrine differentiation that arises from the ovary. It includes carcinoid tumor, small cell carcinoma pulmonary type, and large cell neuroendocrine carcinoma."
      },
      "child_count": 6,
      "reference_id": "MONDO:0002481"
    },
    {
      "id": 4564,
      "label": "breast neuroendocrine neoplasm",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        19314,
        20340
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:3009",
          "GARD:0023146",
          "MEDGEN:231969",
          "NCIT:C5169",
          "UMLS:C1332635"
        ],
        "synonyms": [
          "breast NET",
          "breast endocrine neoplasm",
          "breast neuroendocrine neoplasm",
          "breast neuroendocrine tumor",
          "breast neuroendocrine tumor, well differentiated, low or intermediate grade",
          "breast neuroendocrine tumour",
          "neuroendocrine neoplasm of breast",
          "neuroendocrine neoplasm of the breast",
          "neuroendocrine tumour of the breast"
        ],
        "categories": [
          {
            "ref": "MONDO:0002657",
            "name": "breast disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          }
        ],
        "definition": "A neoplasm that arises from the breast and is composed of cells of neuroendocrine origin. Most cases are neuroendocrine carcinomas. Primary carcinoid tumors of the breast are very rare."
      },
      "child_count": 2,
      "reference_id": "MONDO:0002485"
    },
    {
      "id": 7051,
      "label": "carcinoid tumor",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        19314
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "EFO:0004243",
          "GARD:0024176",
          "HP:0100570",
          "ICD9:209.60",
          "ICDO:8240/3",
          "ICDO:8241/3",
          "MEDGEN:2838",
          "MESH:D002276",
          "NANDO:2200396",
          "NCIT:C2915",
          "SCTID:443492008",
          "UMLS:C0007095"
        ],
        "synonyms": [
          "NET G1",
          "carcinoid",
          "carcinoid tumor",
          "carcinoid tumor (disease)",
          "carcinoid tumour (disease)",
          "neuroendocrine neoplasm G1",
          "neuroendocrine tumor G1",
          "neuroendocrine tumour G1"
        ],
        "categories": [
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          }
        ],
        "definition": "A slow growing neuroendocrine tumor, composed of uniform, round, or polygonal cells having monotonous, centrally located nuclei and small nucleoli, infrequent mitoses, and no necrosis. The tumor may show a variety of patterns, such as solid, trabecular, and acinar. Electron microscopy shows small secretory granules. Immunohistochemical studies reveal NSE, as well as chromogranin immunoreactivity. Malignant histology (cellular pleomorphism, hyperchromatic nuclei, prominent nucleoli, necrosis, and mitoses) can occasionally be seen. Such cases may have an aggressive clinical course. Gastrointestinal tract and lung are common sites of involvement."
      },
      "child_count": 7,
      "reference_id": "MONDO:0005369"
    },
    {
      "id": 7117,
      "label": "lung neuroendocrine neoplasm",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        19314,
        20356
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:5410",
          "EFO:0005220",
          "GARD:0024190",
          "ICD9:209.61",
          "MEDGEN:277303",
          "NCIT:C5670",
          "ONCOTREE:LNET",
          "SCTID:707594002",
          "UMLS:C1334452"
        ],
        "synonyms": [
          "lung NET",
          "lung neuroendocrine neoplasm",
          "lung neuroendocrine tumor",
          "lung neuroendocrine tumor, well differentiated, low or intermediate grade",
          "lung neuroendocrine tumour",
          "neuroendocrine neoplasm of lung",
          "neuroendocrine neoplasm of the lung",
          "pulmonary neuroendocrine neoplasm",
          "pulmonary neuroendocrine tumor",
          "pulmonary neuroendocrine tumour"
        ],
        "categories": [
          {
            "ref": "MONDO:0005087",
            "name": "respiratory system disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          }
        ],
        "definition": "A low, intermediate, or high grade malignant neoplasm with neuroendocrine differentiation that arises from the lung. This category includes typical carcinoid tumor, atypical carcinoid tumor, small cell carcinoma, large cell neuroendocrine carcinoma, and combined carcinoma."
      },
      "child_count": 10,
      "reference_id": "MONDO:0005454"
    },
    {
      "id": 16044,
      "label": "laryngeal neuroendocrine neoplasm",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        7231,
        19314,
        20312,
        21538
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:5457",
          "GARD:0019758",
          "MEDGEN:232692",
          "NCIT:C6023",
          "Orphanet:100083",
          "SCTID:707625001",
          "UMLS:C1334374",
          "icd11.foundation:1502591214"
        ],
        "synonyms": [
          "laryngeal neuroendocrine neoplasm",
          "larynx NET",
          "larynx neuroendocrine neoplasm",
          "larynx neuroendocrine tumor",
          "larynx neuroendocrine tumor, well differentiated, low or intermediate grade",
          "larynx neuroendocrine tumour",
          "neuroendocrine neoplasm of larynx",
          "neuroendocrine neoplasm of the larynx",
          "laryngeal neuroendocrine tumor",
          "laryngeal neuroendocrine tumour"
        ],
        "categories": [
          {
            "ref": "MONDO:0005087",
            "name": "respiratory system disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          },
          {
            "ref": "MONDO:0024623",
            "name": "otorhinolaryngologic disease"
          }
        ],
        "definition": "A benign or malignant neoplasm with neuroendocrine differentiation that arises from the larynx. This category includes paraganglioma, carcinoid tumor, small cell carcinoma, and large cell neuroendocrine carcinoma."
      },
      "child_count": 4,
      "reference_id": "MONDO:0015070"
    },
    {
      "id": 16045,
      "label": "middle ear neuroendocrine tumor",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        19314,
        20527
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0019759",
          "MEDGEN:931137",
          "Orphanet:100084",
          "SCTID:717918002",
          "UMLS:C4305468",
          "icd11.foundation:1437498002"
        ],
        "synonyms": [
          "middle ear NET",
          "middle ear neuroendocrine neoplasm",
          "middle ear neuroendocrine tumor",
          "middle ear neuroendocrine tumor, well differentiated, low or intermediate grade",
          "neuroendocrine neoplasm of middle ear",
          "neuroendocrine tumor of middle ear",
          "neuroendocrine tumour of middle ear"
        ],
        "categories": [
          {
            "ref": "MONDO:0002409",
            "name": "auditory system disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          },
          {
            "ref": "MONDO:0024623",
            "name": "otorhinolaryngologic disease"
          }
        ],
        "definition": "A neuroendocrine neoplasm that involves the middle ear."
      },
      "child_count": 2,
      "reference_id": "MONDO:0015071"
    },
    {
      "id": 17682,
      "label": "hereditary pheochromocytoma-paraganglioma",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        16218,
        19314
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0011984",
          "MEDGEN:895844",
          "OMIMPS:168000",
          "Orphanet:29072",
          "UMLS:C4274332"
        ],
        "synonyms": [
          "familial pheochromocytoma-paraganglioma",
          "hereditary paraganglioma-pheochromocytoma syndrome",
          "hereditary pheochromocytoma-paraganglioma",
          "SDHx-related paraganglioma-pheochromocytoma",
          "hereditary paraganglioma-pheochromocytoma"
        ],
        "categories": [
          {
            "ref": "MONDO:0002254",
            "name": "syndromic disease"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          }
        ],
        "definition": "Neoplasm predisposition characterized by an increased risk of paragangliomas (tumors that arise from neuroendocrine tissues distributed along the paravertebral axis from the base of the skull to the pelvis) and pheochromocytomas (paragangliomas that are confined to the adrenal medulla)."
      },
      "child_count": 18,
      "reference_id": "MONDO:0017366"
    },
    {
      "id": 19678,
      "label": "bronchial endocrine tumor",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        4833,
        19314
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0019360",
          "Orphanet:97287"
        ],
        "synonyms": [
          "bronchial NET",
          "bronchus NET",
          "bronchus neuroendocrine neoplasm",
          "bronchus neuroendocrine tumor",
          "bronchus neuroendocrine tumor, well differentiated, low or intermediate grade",
          "bronchus neuroendocrine tumour",
          "neuroendocrine neoplasm of bronchus",
          "bronchial neuroendocrine tumor",
          "bronchial neuroendocrine tumour"
        ],
        "categories": [
          {
            "ref": "MONDO:0005087",
            "name": "respiratory system disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          }
        ],
        "definition": "A neuroendocrine neoplasm that involves the bronchus."
      },
      "child_count": 0,
      "reference_id": "MONDO:0019963"
    },
    {
      "id": 19679,
      "label": "thymic neuroendocrine tumor",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6916,
        19314
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0019361",
          "MEDGEN:234428",
          "NCIT:C6430",
          "ONCOTREE:TNET",
          "Orphanet:97289",
          "UMLS:C1336746"
        ],
        "synonyms": [
          "neuroendocrine neoplasm of thymus",
          "thymus NET",
          "thymus neuroendocrine neoplasm",
          "thymus neuroendocrine tumor",
          "thymus neuroendocrine tumor, well differentiated, low or intermediate grade",
          "thymus neuroendocrine tumour"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "Thymic endocrine tumor is a rare, malignant, primary thymic neoplasm originating from neuroendocrine cells, presenting as a mass within the anterior mediastinum. Patients typically present with nonspecific symptoms, such as chest pain, cough, shortness of breath, or in some cases, superior vena cava syndrome, although patients could be asymptomatic during the early stages or present with multiple endocrine neoplasia type I. Ectopic production of ACTH and serotonin can lead to Cushing syndrome and carcinoid sydrome, respectively."
      },
      "child_count": 2,
      "reference_id": "MONDO:0019964"
    },
    {
      "id": 20702,
      "label": "uterine corpus neuroendocrine neoplasm",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        19314,
        20461
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0025347",
          "MEDGEN:925580",
          "NCIT:C126771",
          "UMLS:C4288048"
        ],
        "synonyms": [
          "body of uterus NET",
          "body of uterus neuroendocrine neoplasm",
          "body of uterus neuroendocrine tumor",
          "body of uterus neuroendocrine tumor, well differentiated, low or intermediate grade",
          "body of uterus neuroendocrine tumour",
          "neuroendocrine neoplasm of body of uterus",
          "uterine corpus neuroendocrine neoplasm",
          "uterine corpus neuroendocrine tumor",
          "uterine corpus neuroendocrine tumour"
        ],
        "categories": [
          {
            "ref": "MONDO:0005039",
            "name": "reproductive system disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          }
        ],
        "definition": "An epithelial neoplasm with neuroendocrine differentiation that arises from the uterine corpus. It includes carcinoid tumor, small cell carcinoma pulmonary type, and large cell neuroendocrine carcinoma."
      },
      "child_count": 4,
      "reference_id": "MONDO:0021650"
    },
    {
      "id": 21456,
      "label": "digestive system neuroendocrine neoplasm",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6151,
        19314,
        20434
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0002437",
          "MEDGEN:232237",
          "MESH:C535650",
          "NCIT:C27721",
          "Orphanet:100092",
          "UMLS:C1333799"
        ],
        "synonyms": [
          "alimentary part of gastrointestinal system NET",
          "alimentary part of gastrointestinal system neuroendocrine neoplasm",
          "alimentary part of gastrointestinal system neuroendocrine tumor",
          "alimentary part of gastrointestinal system neuroendocrine tumor, well differentiated, low or intermediate grade",
          "alimentary part of gastrointestinal system neuroendocrine tumour",
          "carcinoid tumor of digestive system",
          "carcinoid tumour of digestive system",
          "digestive system NET",
          "digestive system neuroendocrine neoplasm",
          "digestive system neuroendocrine tumor",
          "digestive system neuroendocrine tumor, well differentiated, low or intermediate grade",
          "digestive system neuroendocrine tumour",
          "gastro-enteropancreatic neuroendocrine tumor",
          "gastro-enteropancreatic neuroendocrine tumour",
          "gastroenteropancreatic endocrine tumor",
          "gastroenteropancreatic endocrine tumour",
          "gastroenteropancreatic neuroendocrine neoplasm",
          "gastrointestinal neuroendocrine neoplasm",
          "gastrointestinal system neuroendocrine neoplasm",
          "neuroendocrine neoplasm of alimentary part of gastrointestinal system",
          "neuroendocrine neoplasm of digestive system",
          "GEP tumors",
          "GEP tumours",
          "GEP-NEN",
          "GEP-NET",
          "carcinoid tumor",
          "carcinoid tumour"
        ],
        "categories": [
          {
            "ref": "MONDO:0004335",
            "name": "digestive system disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          }
        ],
        "definition": "A neoplasm with neuroendocrine differentiation arising from the digestive system. It includes neuroendocrine tumors (well-differentiated endocrine tumors or carcinoid tumors and well differentiated endocrine carcinomas) and neuroendocrine carcinomas (poorly differentiated neuroendocrine carcinomas, small cell carcinomas, and large cell neuroendocrine carcinomas)."
      },
      "child_count": 21,
      "reference_id": "MONDO:0024503"
    }
  ],
  "roots": [
    {
      "id": 4223,
      "label": "endocrine gland neoplasm"
    }
  ]
}