{"orpha":97283,"name":"Somatostatinoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Somatostatinoma (SSoma) is an extremely rare pancreatic neuroendocrine tumor or duodenal endocrine tumor that originates either in the pancreas (50%) or the gastrointestinal tract (50%) and mainly presents with non-specific symptoms of abdominal pain, weight loss, jaundice and diarrhea but, in approximately 20% of pancreatic cases, leads to a somatostatin hypersecretion syndrome (somatostatinoma syndrome) characterized by diabetes mellitus, cholelithiasis, steatorrhea and hypochlorhydria.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-97283/","orphanet_url":"https://www.orpha.net/en/disease/detail/97283","names":{"fr":{"name":"Somatostatinome","definition":"Tumeur neuroendocrine du pancréas ou tumeur endocrine du duodénum, très rare, qui prend naissance soit dans le pancréas (50&nbsp;%), soit dans le tractus gastro-intestinal (50&nbsp;%) et se manifeste généralement par des symptômes non spécifiques tels que des douleurs abdominales, une perte de poids, une jaunisse et une diarrhée, mais qui, dans environ 20&nbsp;% des cas pancréatiques, entraîne un syndrome d'hypersécrétion de somatostatine (syndrome du somatostatinome) caractérisé par un diabète sucré, une cholélithiase, une stéatorrhée et une hypochlorhydrie."},"de":{"name":"Somatostatinom"},"es":{"name":"Somatostatinoma","definition":"El somatostatinoma (SSoma) es un tumor pancreático neuroendocrino extremadamente raro o un tumor endocrino del duodeno (consulte estos términos) que se origina en el páncreas (50%) o en el tracto gastrointestinal (50%). Por lo general, se presenta con síntomas no específicos de dolor abdominal, pérdida de peso, ictericia y diarrea pero, en aproximadamente el 20% de los casos pancreáticos, conduce a un síndrome de hipersecreción de somatostatina (síndrome del somatostatinoma) caracterizado por diabetes mellitus, colelitiasis, esteatorrea e hipoclorhidria."},"it":{"name":"Somatostatinoma","definition":"Il somatostatinoma (SSoma) è un tumore neuroendocrino pancreatico o endocrino del duodeno (si vedano questi termini) molto raro, che si sviluppa nel pancreas (50%) o nel tratto gastrointestinale (50%) ed esordisce prevalentemente con sintomi aspecifici come il dolore addominale, la perdita di peso, l'ittero e la diarrea; nel 20% circa dei casi pancreatici, causa la sindrome da ipersecrezione di somatostatina (sindrome da somatostatinoma), caratterizzata da diabete mellito, colelitiasi, steatorrea e ipocloridria."},"nl":{"name":"Somatostatinoom","definition":"Een extreem zeldzame neuro-endocriene tumor van pancreas of duodenum die ontstaat in pancreas (50%) of in gastro-intestinaal stelsel (50%), en die zich voornamelijk presenteert met aspecifieke symptomen als abdominale pijn, gewichtsverlies, geelzucht en diarree, maar die in ongeveer 20% van de pancreatische gevallen leidt tot een syndroom van hypersecretie van somatostatine (somatostatinoomsyndroom) gekenmerkt door diabetes mellitus, cholelithiasis, steatorroe en hypochloorhydrie."},"pt":{"name":"Somatostatinoma"},"pl":{"name":"Somatostatinoma"},"cs":{"name":"Somatostatinom"},"uk":{"name":"Соматостатинома","definition":"Somatostatinoma (SSoma) is an extremely rare pancreatic neuroendocrine tumor or duodenal endocrine tumor (see these terms) that originates either in the pancreas (50%) or the gastrointestinal tract (50%) and mainly presents with non-specific symptoms of abdominal pain, weight loss, jaundice and diarrhea but, in approximately 20% of pancreatic cases, leads to a somatostatin hypersecretion syndrome (somatostatinoma syndrome) characterized by diabetes mellitus, cholelithiasis, steatorrhea and hypochlorhydria."},"zh":{"name":"生长抑素瘤","definition":"生长抑素瘤（SSoma）是一种极为罕见的胰腺神经内分泌肿瘤或十二指肠内分泌肿瘤（见该类词条），起源于胰腺（50%）或胃肠道（50%），主要表现为腹痛、体重减轻、黄疸和腹泻等非特异性症状，但是，在大约20%的胰腺病例中，会导致生长抑素过度分泌综合征（生长抑素瘤综合征），其特征是糖尿病、胆石症、脂肪酸血症和低氯酸症。"},"tr":{"name":"Somatostatinom"}},"xrefs":[{"source":"ICD-10","code":"C17.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C10.1","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"219879696"},{"source":"MONDO","code":"6976","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D013005","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0037661","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"4900","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10041329","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97978,"name":"Rare endocrine disease"},"parents":[{"orpha":100076,"name":"Duodenal neuroendocrine tumor","classifications":[152,193,202,233]},{"orpha":506060,"name":"Functioning neuroendocrine tumor of pancreas","classifications":[152,193,202]},{"orpha":100077,"name":"Jejunal neuroendocrine tumor","classifications":[152,193,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Unknown"],"onset":["Adult","Elderly"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.0025,"area":"Europe","status":"Validated","source":"[EXPERT]_17645474[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Europe","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2017-02-01","source":"26742109[PMID]_11340706[PMID]_17645474[PMID]_22997445[PMID]_22261872[PMID]_23582916[PMID]_18798544[PMID]_10940685[PMID]_2440390[PMID]_18941127[PMID]_22052063[PMID]","terms":[{"hpo":"HP:0002039","label":"Anorexia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012432","label":"Chronic fatigue","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002019","label":"Constipation","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000819","label":"Diabetes mellitus","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002014","label":"Diarrhea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002574","label":"Episodic abdominal pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0005609","label":"Gallbladder dysfunction","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002240","label":"Hepatomegaly","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0004840","label":"Hypochromic microcytic anemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002017","label":"Nausea and vomiting","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002894","label":"Neoplasm of the pancreas","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100833","label":"Neoplasm of the small intestine","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0004396","label":"Poor appetite","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002570","label":"Steatorrhea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001438","label":"Abnormality of abdomen morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001541","label":"Ascites","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012334","label":"Extrahepatic cholestasis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002239","label":"Gastrointestinal hemorrhage","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001046","label":"Intermittent jaundice","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0005214","label":"Intestinal obstruction","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001406","label":"Intrahepatic cholestasis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030145","label":"Lack of bowel sounds","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000820","label":"Abnormality of the thyroid gland","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0008256","label":"Adrenocortical adenoma","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000845","label":"Elevated circulating growth hormone concentration","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0003072","label":"Hypercalcemia","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0003118","label":"Increased circulating cortisol level","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000837","label":"Increased circulating gonadotropin level","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000870","label":"Increased circulating prolactin concentration","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002865","label":"Medullary thyroid carcinoma","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002897","label":"Parathyroid adenoma","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002893","label":"Pituitary adenoma","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0008200","label":"Primary hyperparathyroidism","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001031","label":"Subcutaneous lipoma","frequency":"Very rare (<4-1%)","criteria":null}]}}