{"orpha":91351,"name":"Pituitary dermoid and epidermoid cysts","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Pituitary dermoid and epidermoid cysts is a rare, acquired pituitary hormone deficiency characterized by the presence of rare, benign tumor in the sellar region. Clinical presentation is either acute or insidious, and is variable according to the cyst location, size and potential rupture. Most commonly patients present with headache, visual disturbances, and pituitary dysfunction.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-91351/","orphanet_url":"https://www.orpha.net/en/disease/detail/91351","names":{"fr":{"name":"Kystes dermoïde et épidermoïde de l'hypophyse","definition":"Insuffisance hypophysaire acquise rare caractérisée par la présence d'une tumeur bénigne rare dans la région sellaire. La présentation clinique est aiguë ou insidieuse, variant en fonction de l'emplacement du kyste, de sa taille et de son éventuelle rupture. Les patients présentent généralement des maux de tête, des troubles de la vision et un déficit hypophysaire."},"de":{"name":"Hypophysen- und Epidermoidzysten"},"es":{"name":"Quistes hipofisarios dermoides y epidermoides","definition":"Los quistes hipofisarios dermoides y epidermoides son una deficiencia de hormona hipofisaria adquirida, poco frecuente, caracterizada por la presencia de un tumor benigno poco frecuente en la región selar. La presentación clínica es aguda o insidiosa, y varía según la ubicación, tamaño y potencial rotura del quiste. La mayoría de los afectados presenta cefalea, trastornos visuales y disfunción pituitaria."},"it":{"name":"Cisti ipofisarie dermoidi ed epidermoidi","definition":"Le cisti ipofisarie dermoidi ed epidermoidi sono un difetto raro acquisito degli ormoni ipofisi; sono caratterizzate dalla presenza di un tumore raro benigno nella regione della sella. L'esordio può essere acuto o insidioso; il quadro clinico varia a seconda della localizzazione della cisti, delle sue dimensioni e dalla probabilità di rottura. Di solito i pazienti presentano cefalea, disturbi visivi e disfunzione ipofisaria."},"nl":{"name":"Hypofysaire dermoïde en epidermoïde cysten","definition":"Hypofysaire dermoïde en epidermoïde cysten is een zeldzame, verworven hypofysaire hormoondeficiëntie die gekarakteriseerd wordt door de aanwezigheid vaneen zeldzame, goedaardige tumor in het sellaire gebied. De klinische presentatie is acuut of sluipend, en varieert naargelang de locatie, omvang en eventuele scheuring van cysten. Meestal presenteren patiënten zich met hoofdpijn, visuele stoornissen, en disfunctie van de hypofyse."},"pt":{"name":"Quistos pituitários dermóides e epidermóides"},"pl":{"name":"Torbiele skórzaste i nabłonkowe przysadki","definition":"*Torbiele skórzaste i nabłonkowe przysadki powodują rzadko występujący, nabyty niedobór hormonów przysadki; charakteryzują się obecnością rzadko występującego, łagodnego guza w okolicy siodła. Obraz kliniczny jest ostry lub podstępny i zróżnicowany: zależy od lokalizacji torbieli, jej rozmiaru i potencjalnego pęknięcia. Najczęściej pacjenci zgłaszają się z bólem głowy, zaburzeniami widzenia i dysfunkcją przysadki."},"cs":{"name":"Dermoidní a epidermoidní cysty hypofýzy"},"uk":{"name":"Дермоїдні та епідермоїдні кісти гіпофіза","definition":"Pituitary dermoid and epidermoid cysts is a rare, acquired pituitary hormone deficiency characterized by the presence of rare, benign tumor in the sellar region. Clinical presentation is either acute or insidious, and is variable according to the cyst location, size and potential rupture. Most commonly patients present with headache, visual disturbances, and pituitary dysfunction."},"zh":{"name":"垂体皮样囊肿和表皮样囊肿"},"tr":{"name":"Hipofiz dermoid ve epidermoid kistler"}},"xrefs":[{"source":"ICD-10","code":"D35.2","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"5A61.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"2050453664"},{"source":"MONDO","code":"0019615","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5190781","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97978,"name":"Rare endocrine disease"},"parents":[{"orpha":100091,"name":"Adrenal/paraganglial tumor","classifications":[193,202]},{"orpha":95503,"name":"Pituitary hormone deficiency of tumoral origin","classifications":[193]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adult","Elderly"],"prevalence":[],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2017-02-08","source":"11081162[PMID]_1886651[PMID]_15808736[PMID]_9384413[PMID]_9864727[PMID]_2417151[PMID]_6827329[PMID]_6661939[PMID]_15808736[PMID]_18701295[PMID]_25865027[PMID]","terms":[{"hpo":"HP:0000830","label":"Anterior hypopituitarism","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0010885","label":"Avascular necrosis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002017","label":"Nausea and vomiting","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0011750","label":"Neoplasm of the anterior pituitary","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000141","label":"Amenorrhea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000651","label":"Diplopia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012505","label":"Enlarged pituitary gland","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100829","label":"Galactorrhea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003324","label":"Generalized muscle weakness","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010514","label":"Hyperpituitarism","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000135","label":"Hypogonadism","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000870","label":"Increased circulating prolactin concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000876","label":"Oligomenorrhea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000871","label":"Panhypopituitarism","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002331","label":"Recurrent paroxysmal headache","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0007663","label":"Reduced visual acuity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030907","label":"Thunderclap headache","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011442","label":"Abnormality of central motor function","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011730","label":"Abnormality of central sensory function","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001287","label":"Meningitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000798","label":"Oligozoospermia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001250","label":"Seizure","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001959","label":"Polydipsia","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001117","label":"Sudden loss of visual acuity","frequency":"Very rare (<4-1%)","criteria":null}]}}