{"orpha":251623,"name":"Pituicytoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare glial tumor originating from pituicytes, the specialized glial cells of the neurohypophysis, characterized by a sellar or suprasellar mass manifesting with clinical signs secondary to mass effect. Typical manifestations are visual disturbances, headaches, and hypopituitarism. Pituicytomas are low-grade tumors, and prognosis is good after total resection.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-251623/","orphanet_url":"https://www.orpha.net/en/disease/detail/251623","names":{"fr":{"name":"Pituicytome","definition":"Tumeur gliale rare prenant naissance dans les pituicytes, les cellules gliales spécialisées de la neurohypophyse, caractérisée par une masse sellaire ou suprasellaire se manifestant par des signes cliniques secondaires à un effet de masse. Les manifestations typiques sont des troubles de la vision, des céphalées et une insuffisance hypophysaire. Les pituicytomes sont des tumeurs de bas grade, dont le pronostic après résection totale est bon."},"de":{"name":"Pituizytom","definition":"Ein glialer Tumor, der von den Pituizyten (spezifische Gliazellen der Neurohypophyse) ausgeht. Er ist durch eine sellare oder suprasellare Gewebeverdichtung gekennzeichnet, die mit klinischen Symptomen einhergehen, die auf den Masseneffekt zurückzuführen sind. Typische Manifestationen sind Sehstörungen, Kopfschmerzen und Hypopituitarismus. Hypophysentumore sind niedriggradige Tumore, die nach vollständiger Resektion eine gute Prognose haben."},"es":{"name":"Pituicitoma","definition":"Es un tumor glial poco frecuente que se origina a partir de los pituicitos, células gliales especializadas de la neurohipófisis, y que está caracterizado por una masa selar o supraselar, que se manifiesta con signos clínicos secundarios al efecto masa. Los hallazgos típicos son trastornos visuales, cefaleas e hipopituitarismo. Los pituicitomas son tumores de bajo grado y el pronóstico es bueno después de la resección completa."},"it":{"name":"Pituicitoma","definition":"Si tratta di una neoplasia gliale rara, che origina dai pituiciti (le cellule gliali specializzate della neuroipofisi) ed è caratterizzata da una massa nella sella o sopra-sellare. La malattia esordisce con i segni clinici secondari alla presenza della massa, in particolare con disturbi della vista, cefalea e ipopituitarismo. I pituicitomi sono tumori a basso grado di malignità, con una prognosi favorevole dopo la resezione completa."},"nl":{"name":"Pituïcytoom","definition":"Een zeldzame gliale tumor die ontstaat uit pituïcyten, de gespecialiseerde gliacellen van de neurohypofyse, gekarakteriseerd door een sellaire of suprasellaire massa die zich manifesteert met klinische tekenen secundair aan massa-effect. Typische manifestaties zijn visuele stoornissen, hoofdpijn, en hypopituïtarisme. Pituïcytomen zijn laaggradige tumoren, en de prognose is goed na totale excisie."},"pt":{"name":"Pituicitoma"},"pl":{"name":"Pituicytoma"},"cs":{"name":"Pituicytom"},"uk":{"name":"Пітуїцитома","definition":"A rare glial tumor originating from pituicytes, the specialized glial cells of the neurohypophysis, characterized by a sellar or suprasellar mass manifesting with clinical signs secondary to mass effect. Typical manifestations are visual disturbances, headaches, and hypopituitarism. Pituicytomas are low-grade tumors, and prognosis is good after total resection."},"zh":{"name":"垂体细胞瘤","definition":"一种罕见的神经胶质瘤，起源于垂体细胞，这是神经垂体的特殊胶质细胞，以鞍区或鞍上区肿块为特征，临床症状继发于肿块效应。典型的表现是视力障碍、头痛和垂体功能减退。垂体细胞瘤为低级别肿瘤，全切除后预后良好。"},"tr":{"name":"Pituisitom"}},"xrefs":[{"source":"ICD-10","code":"D44.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2A00.0Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"34496826"},{"source":"MONDO","code":"0006372","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2986550","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10089140","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":251592,"name":"Low-grade astrocytoma","classifications":[181,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":171.0,"area":"Worldwide","status":"Validated","source":"33971477[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"33971477[PMID]_ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-27","source":"21960498[PMID]_25947224[PMID]_23887161[PMID]_23701182[PMID]_23237862[PMID]_20586639[PMID]_19525896[PMID]_10716149[PMID]","terms":[{"hpo":"HP:0011752","label":"Neoplasm of the posterior pituitary","frequency":"Obligate (100%)","criteria":null},{"hpo":"HP:0011754","label":"Pituicytoma","frequency":"Obligate (100%)","criteria":null},{"hpo":"HP:0012503","label":"Abnormality of the pituitary gland","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002315","label":"Headache","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001123","label":"Visual field defect","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011043","label":"Abnormality of circulating adrenocorticotropin level","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000141","label":"Amenorrhea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030018","label":"Decreased female libido","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000824","label":"Decreased response to growth hormone stimulation test","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0008214","label":"Decreased serum estradiol","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0040171","label":"Decreased serum testosterone concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000044","label":"Hypogonadotropic hypogonadism","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0040075","label":"Hypopituitarism","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000802","label":"Impotence","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000870","label":"Increased circulating prolactin concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002354","label":"Memory impairment","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0008245","label":"Pituitary hypothyroidism","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000863","label":"Central diabetes insipidus","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0100829","label":"Galactorrhea","frequency":"Very rare (<4-1%)","criteria":null}]}}