{"Name":"Neuroendocrine tumor of pancreas","DiseaseID__c":"GARD:0013034","id":13034,"encodedName":"neuroendocrine-tumor-of-pancreas","IsDeleted":false,"Disease_Name_Full__c":"Neuroendocrine tumor of pancreas","Xref_IDs__c":"C1337011; C27720; MEDGEN:277875; MONDO:0019954; ORPHA:97253","USA_Estimate__c":"5,000","No_of_Specialist_Tagsa__c":3,"No_of_ClinGen_records__c":0,"No_of_GeneReviews__c":0,"No_of_HHS_records__c":0,"World_Estimate__c":"8,000 to 80,000","No_of_HRSA_records__c":0,"Evidence_Based_Score__c":0,"No_of_Disease_Descriptions__c":3,"Disease_Characteristics_Score__c":6,"No_of_Age_at_Onset__c":1,"Description_Source__c":"MONDO:0019954","Disease_Description__c":"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) (see these terms).","GARD_Name__c":"Neuroendocrine tumor of pancreas","GARD_Synonym__c":"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; neuroendocrine pancreatic tumor; pancreatic net; pancreatic neuroendocrine tumor; pnet; 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","Curated_Disease_Description_Source__c":"GARD:0013034","Curated_Disease_Description__c":"A Pancreatic neuroendocrine tumor, also called an islet cell tumor, is a type of neuroendocrine tumor (NET) that typically arises in the pancreas. However in some cases, a pancreatic NET occurs outside of the pancreas. A NET arises from cells that produce hormones, so the tumor can also produce hormones. It may be benign (not cancerous) or malignant (cancerous). Pancreatic NETs are called either functional or nonfunctional. A functional pancreatic NET causes specific symptoms because it makes extra hormones, such as gastrin, insulin, or glucagon. Examples of types of functional pancreatic NETs include insulinomas, glucagonomas, gastrinomas, VIPomas, and somatostatinomas. Symptoms depend on the type of hormone being made. A nonfunctional pancreatic NET generally does not cause specific symptoms, but may eventually cause symptoms relating to its location and size as it grows or spreads. Most pancreatic NETs are not inherited and occur sporadically in people with no family history of NETs. However, about 10% are associated with a hereditary cancer or tumor syndrome such as multiple endocrine neoplasia type 1 (MEN1), which has autosomal dominant inheritance.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":"5,000","Age_at_Onset_Snippet_Text__c":"as an Adult","SourceID__c":"ORPHA:97253","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Grouping","MONDO_ID__c":"MONDO:0019954","ORPHANET_ID__c":"ORPHA:97253","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Tumor neuroendocrino del páncreas","Spanish_Description_Source__c":"ORPHA:97253","Spanish_Description__c":"El tumor neuroendocrino pancreático (pNET), describe un grupo de tumores endocrinos que se originan en el páncreas y que, por lo general, su comportamiento es poco agresivo, aunque pueden resultar potencialmente malignos. Pueden ser funcionantes, exhibiendo un síndrome de hipersecreción hormonal, o no funcionantes, presentándose con síntomas no específicos e incluyen el insulinoma, el glucagonoma, el VIPoma, el somatostatinoma (SSoma), el PPoma y el síndrome de Zollinger-Ellison (ZES, o gastrinoma) y otros tumores producidos por hormonas ectópicas (tales como el GRFoma) (consulte estos términos).","Spanish_Disease_Name__c":"tumor neuroendocrino del páncreas","Spanish_GARD_Synonym__c":"neoplasia neuroendocrina del páncreas bien diferenciada; neoplasia neuroendocrina pancreática bien diferenciada; pnet; tne bien diferenciado del páncreas; tne bien diferenciado pancreático; tne pancreático; tumor neuroendocrino pancreático","Category_Linearization__c":"ORPHA:97978","icd10_id__c":null,"mesh_id__c":null,"omim_id__c":null,"snomed_id__c":null,"umls_id__c":null,"GARD_Disease__c":[{"Curated_Disease_Description__c":"A Pancreatic neuroendocrine tumor, also called an islet cell tumor, is a type of neuroendocrine tumor (NET) that typically arises in the pancreas. However in some cases, a pancreatic NET occurs outside of the pancreas. A NET arises from cells that produce hormones, so the tumor can also produce hormones. It may be benign (not cancerous) or malignant (cancerous). Pancreatic NETs are called either functional or nonfunctional. A functional pancreatic NET causes specific symptoms because it makes extra hormones, such as gastrin, insulin, or glucagon. Examples of types of functional pancreatic NETs include insulinomas, glucagonomas, gastrinomas, VIPomas, and somatostatinomas. Symptoms depend on the type of hormone being made. A nonfunctional pancreatic NET generally does not cause specific symptoms, but may eventually cause symptoms relating to its location and size as it grows or spreads. Most pancreatic NETs are not inherited and occur sporadically in people with no family history of NETs. However, about 10% are associated with a hereditary cancer or tumor syndrome such as multiple endocrine neoplasia type 1 (MEN1), which has autosomal dominant inheritance.","Curated_Disease_Description_Source__c":"GARD:0013034","GARD_Synonym__c":"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; neuroendocrine pancreatic tumor; pancreatic net; pancreatic neuroendocrine tumor; pnet; 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","Name":"Neuroendocrine tumor of pancreas","Curated_USA_Estimate__c":"5,000","estimateUsa":"5,000"}],"Organization_Supported_Diseases__c":[{"Account_Name__c":"Pancreatic Cancer UK","Website__c":"https://www.pancreaticcancer.org.uk/"},{"Account_Name__c":"Neuroendocrine Tumor Research Foundation","Website__c":"https://netrf.org/"},{"Account_Name__c":"Pancreatic Cancer Action Network","Website__c":"https://www.pancan.org/"},{"Account_Name__c":"National Pancreas Foundation","Website__c":"https://pancreasfoundation.org/"},{"Account_Name__c":"Neuroendocrine Cancer UK","Website__c":"https://www.neuroendocrinecancer.org.uk/"},{"Account_Name__c":"Pancreatica","Website__c":"https://pancreatica.org/"},{"Account_Name__c":"The Healing NET Foundation","Website__c":"https://www.thehealingnet.org/"},{"Account_Name__c":"NorCal CarciNet Community","Website__c":"https://norcalcarcinet.org/"},{"Account_Name__c":"International Neuroendocrine Cancer Alliance","Website__c":"https://incalliance.org/"},{"Account_Name__c":"Neuroendocrine Cancer Awareness Network","Website__c":"https://www.netcancerawareness.org/"}],"GARD_Disease_Tag__c":[{"Tag_Name__c":"Cancer - Oncologist","Tag_Category__c":"Specialist"},{"Tag_Name__c":"Cancer","Tag_Category__c":"Disease Category","category_description":"Cancer is a disease in which some of the body's cells grow uncontrollably and can spread to other parts of the body.","curated_tag_name":"Cancer"},{"Tag_Name__c":"Endocrine","Tag_Category__c":"Disease Category;Specialist","category_description":"Endocrine diseases affect hormone production or how the body responds to a specific hormone(s).","curated_tag_name":"Endocrine diseases"},{"Tag_Name__c":"Gastroenterology","Tag_Category__c":"Disease Category;Specialist","category_description":"Gastrointestinal diseases, or digestive diseases, affect the esophagus, stomach, small intestine, large intestine, liver, gallbladder, or pancreas.","curated_tag_name":"Gastrointestinal diseases"},{"Tag_Name__c":"Pancreatic tumor","Tag_Category__c":"Account","curated_tag_name":"Pancreatic tumors"}],"Age_At_Onset__c":[{"Age_At_Onset__c":"Adult","Provided_By__c":"ORPHA:97253"}],"External_Identifier_Disease__c":[{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=277875","Source__c":"C1337011","Xref__c":"MEDGEN:277875"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C1337011","Source__c":"C1337011","Xref__c":"C1337011"},{"URL__c":"https://www.orpha.net/en/disease/detail/97253","Source__c":"C1337011; MONDO:0019954","Xref__c":"ORPHA:97253"},{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C27720","Source__c":"C1337011; MONDO:0019954","Xref__c":"C27720"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0019954","Source__c":"GARD:0013034","Xref__c":"MONDO:0019954"}],"Inheritance__c":["Autosomal dominant"],"tags":{"Specialist":["Cancer - Oncologist","Endocrine","Gastroenterology"],"Disease Category":["Cancer","Endocrine","Gastroenterology"],"Account":["Pancreatic tumor"]},"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"," neuroendocrine pancreatic tumor"," pancreatic net"," pancreatic neuroendocrine tumor"," pnet"," 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"]}