{
  "id": 8401,
  "label": "somatostatinoma",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0006976",
  "properties": {
    "xrefs": [
      "DOID:4430",
      "EFO:1001187",
      "GARD:0004900",
      "ICD9:235.5",
      "ICDO:8156/1",
      "MEDGEN:52416",
      "MESH:D013005",
      "MedDRA:10041329",
      "NCIT:C3379",
      "Orphanet:97283",
      "SCTID:253006001",
      "UMLS:C0037661",
      "icd11.foundation:219879696"
    ],
    "synonyms": [
      "Delta cell tumor",
      "Delta cell tumour",
      "somatostatin cell tumor",
      "somatostatin producing tumor",
      "somatostatin producing tumour",
      "somatostatin-producing NET",
      "somatostatin-producing neuroendocrine tumor",
      "somatostatin-producing neuroendocrine tumour",
      "somatostatin-producing tumor",
      "somatostatin-producing tumour",
      "somatostatinoma",
      "tumor of Delta cells",
      "tumor of the Delta cells",
      "tumour of Delta cells",
      "tumour of the Delta cells",
      "Somatomedin-secreting carcinoid",
      "ampullary somatostatinoma",
      "carcinoid somatostatinoma",
      "malignant islet cell tumor",
      "malignant islet cell tumour",
      "somatostatin-secreting pancreatic neoplasm"
    ],
    "categories": [
      {
        "ref": "MONDO:0004335",
        "name": "digestive system disorder"
      },
      {
        "ref": "MONDO:0005151",
        "name": "endocrine system disorder"
      }
    ],
    "definition": "A rare, usually malignant neuroendocrine tumor arizing from delta cells. This neoplasm produces large amounts of somatostatin, which may result in a syndrome characterized by diarrhea, steatorrhea, weight loss, and gastric hyposecretion. Sixty percent are found in the pancreas and 40% in the duodenum or jejunum. The peak incidence occurs between 40 and 60 years of age; women are affected more than men by 2:1."
  },
  "isLeaf": true,
  "isRoot": false,
  "child_count": 0,
  "parents": [
    {
      "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": 19671,
      "label": "pancreatic neuroendocrine tumor",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        2885,
        7431
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "EFO:1000045",
          "GARD:0013034",
          "ICDO:8150/1",
          "MEDGEN:277875",
          "NCIT:C27720",
          "ONCOTREE:PANET",
          "Orphanet:97253",
          "UMLS:C1337011"
        ],
        "synonyms": [
          "islet cell tumor",
          "islet cell tumors - pancreas",
          "islet cell tumors of the pancreas",
          "islet cell tumour",
          "islet cell tumours - pancreas",
          "islet cell tumours of the pancreas",
          "pancreatic NET",
          "pancreatic neuroendocrine tumor",
          "well differentiated pancreatic endocrine neoplasm",
          "well differentiated pancreatic endocrine tumor",
          "well differentiated pancreatic endocrine tumour",
          "well-differentiated NEN of pancreas",
          "well-differentiated neuroendocrine neoplasm of pancreas",
          "well-differentiated pancreatic NEN",
          "well-differentiated pancreatic neuroendocrine neoplasm",
          "PANET",
          "neuroendocrine tumor of pancreas",
          "neuroendocrine tumour of pancreas",
          "pancreatic endocrine tumor",
          "pancreatic endocrine tumour"
        ],
        "categories": [
          {
            "ref": "MONDO:0004335",
            "name": "digestive system disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          }
        ],
        "definition": "Pancreatic endocrine tumor, also known as pancreatic neuroendocrine tumor (PNET), describes a group of endocrine tumors originating in the pancreas that are usually indolent and benign, but may have the potential to be malignant. They can be functional, exhibiting a hormonal hypersecretion syndrome, but can be non-functional presenting with non-specific symptoms and include insulinoma, glucagonoma, VIPoma, somatostatinoma (SSoma), PPoma and Zollinger-Ellison syndrome (ZES, or gastrinoma) and other ectopic hormone producing tumors (such as GRFoma)."
      },
      "child_count": 22,
      "reference_id": "MONDO:0019954"
    }
  ],
  "children": [],
  "roots": [
    {
      "id": 7051,
      "label": "carcinoid tumor"
    },
    {
      "id": 19671,
      "label": "pancreatic neuroendocrine tumor"
    }
  ]
}