{"orpha":97261,"name":"GRFoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["GRF tumor","Growth hormone releasing factor tumor"],"definition":"GRFoma is a type of pancreatic endocrine tumor that hypersecretes growth hormone-releasing factor (GRF or GHRH) and that clinically resembles a pituitary adenoma as patients present with acromegaly. In addition to the pancreas, this tumor can also occur in the lungs or small intestine, are usually large > 6cm and approximately 1/3 have metastasized at the time of diagnosis. It often co-occurs with Zollinger-Ellison syndrome or multiple endocrine neoplasia type 1 (MEN 1).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-97261/","orphanet_url":"https://www.orpha.net/en/disease/detail/97261","names":{"fr":{"name":"GRFome","synonyms":["Tumeur secrétant le GRF","Tumeur secrétant le facteur de libération de l'hormone de croissance","Tumeur à GRF"],"definition":"Le GRFome est un type de tumeur endocrine pancréatique , caractérisé par l'hypersécrétion du facteur de libération de l'hormone de croissance (GRF ou GHRH) et ressemblant sur le plan clinique à un adénome hypophysaire , avec une acromégalie. Cette tumeur peut également toucher les poumons ou l'intestin grêle. Le GRFome mesure généralement plus de 6 cm de large et environ 1/3 a métastasé au moment du diagnostic. Il co-survient souvent avec le syndrome de Zollinger-Ellison ou la néoplasie endocrinienne multiple type 1 (NEM 1)."},"de":{"name":"GRFom","synonyms":["GRF (Growth hormone releasing factor)-Tumor"]},"es":{"name":"GRFoma","definition":"Es un tipo de tumor endocrino pancreático que hipersecreta la hormona liberadora de la hormona del crecimiento (GRF o GHRH) y que se asemeja clínicamente a un adenoma hipofisario cuando los pacientes presentan acromegalia. Además del páncreas, este tumor también puede desarrollarse en los pulmones o en el intestino delgado, suele ser grande (> 6 cm) y aproximadamente 1/3 de los pacientes presenta metástasis en el momento del diagnóstico. Asocia con frecuencia el síndrome de Zollinger-Ellison o la neoplasia endocrina múltiple tipo 1."},"it":{"name":"GRFoma","synonyms":["Tumore causato dal fattore di rilascio dell'ormone della crescita","Tumore da GRF"]},"nl":{"name":"GRFoom","synonyms":["GRF-tumor","Tumor door groeihormoon-'releasing' factor","Tumor door groeihormoonvrijmakende factor"],"definition":"Een type tumor van endocriene pancreas, met hypersecretie van groeihormoon-releasing factor (GRF) en klinisch gelijkaardig aan een hypofyseadenoom zoals patiënten met acromegalie presenteren. Naast pancreas kan deze tumor ook voorkomen in long of dunne darm. De tumor is doorgaans groot (> 6 cm) en ongeveer 1/3 is gemetastaseerd ten tijde van de diagnose. Vaak komt de tumor gelijktijdig voor met syndroom van Zollinger-Ellison of multipele endocriene neoplasie type 1 (MEN1)."},"pt":{"name":"GRFoma"},"pl":{"name":"GRFoma","synonyms":["Guz GRF","Gyz wydzielający somatoliberynę"]},"cs":{"name":"GRFom","synonyms":["GRF nádor","Nádor produkující somatoliberin","Nádor produkující hormon stimulující uvolňování růstového hormonu"]},"uk":{"name":"GRFoma","synonyms":["Пухлина GRF","Пухлина, що вивільняє фактор росту"],"definition":"GRFoma is a type of pancreatic endocrine tumor (see this term) that hypersecretes growth hormone-releasing factor (GRF or GHRH) and that clinically resembles a pituitary adenoma (see this term) as patients present with acromegaly. In addition to the pancreas, this tumor can also occur in the lungs or small intestine, are usually large > 6cm and approximately 1/3 have metastasized at the time of diagnosis. It often co-occurs with Zollinger-Ellison syndrome or multiple endocrine neoplasia type 1 (MEN 1; see these terms)."},"zh":{"name":"生长激素释放因子肿瘤","synonyms":["生长激素释放因子肿瘤"],"definition":"GRFoma是一种过度分泌生长激素释放因子（GRF或GHRH）的胰腺内分泌肿瘤(见该词条)，临床上与肢端肥大症患者的垂体腺瘤(见该词条)相似。除了胰腺，这种肿瘤也可以发生在肺或小肠，通常大 inf 6厘米，约1/3的病人在诊断时肿瘤已经转移。它通常与Zollinger-Ellison综合征或多发性内分泌肿瘤1型（MEN1；见该词条）共同出现。"},"tr":{"name":"GRFom","synonyms":["GRF tümörü","Büyüme hormonu salgılayan faktör tümörü"]}},"xrefs":[{"source":"ICD-10","code":"C25.9","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"E16.8","qualifier":"ND","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C10.1","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1245945716"},{"source":"MONDO","code":"0019955","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0346406","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97978,"name":"Rare endocrine disease"},"parents":[{"orpha":506060,"name":"Functioning neuroendocrine tumor of pancreas","classifications":[152,193,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2017-02-01","source":"26742109[PMID]_22983831[PMID]_6090497[PMID]_22442262[PMID]_6290540[PMID]_6292724[PMID]_22261919[PMID]_22261872[PMID]_23582916[PMID]_18798544[PMID]_10940685[PMID]_26162406[PMID]_18941127[PMID]_22052063[PMID]","terms":[{"hpo":"HP:0000845","label":"Elevated circulating growth hormone concentration","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001438","label":"Abnormality of abdomen morphology","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002039","label":"Anorexia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000280","label":"Coarse facial features","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002019","label":"Constipation","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:0002240","label":"Hepatomegaly","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030144","label":"Hypoactive bowel sounds","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003118","label":"Increased circulating cortisol level","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002017","label":"Nausea and vomiting","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100526","label":"Neoplasm of the lung","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002894","label":"Neoplasm of the pancreas","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0007410","label":"Palmoplantar hyperhidrosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0004396","label":"Poor appetite","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002044","label":"Zollinger-Ellison syndrome","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000820","label":"Abnormality of the thyroid gland","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0008256","label":"Adrenocortical adenoma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001541","label":"Ascites","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001081","label":"Cholelithiasis","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:0003072","label":"Hypercalcemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000837","label":"Increased circulating gonadotropin level","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000870","label":"Increased circulating prolactin concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001046","label":"Intermittent jaundice","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0006723","label":"Intestinal carcinoid","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:0100833","label":"Neoplasm of the small intestine","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002897","label":"Parathyroid adenoma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002893","label":"Pituitary adenoma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0008200","label":"Primary hyperparathyroidism","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001031","label":"Subcutaneous lipoma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100521","label":"Neoplasm of the thymus","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002666","label":"Pheochromocytoma","frequency":"Very rare (<4-1%)","criteria":null}]}}