{"orpha":301,"name":"Ependymal tumor","type":"Clinical group","group":"Group of disorders","status":"active","flags":[],"synonyms":[],"definition":"A tumor of neurectodermal origin arising from ependymal cells that line the ventricles and central canal of the spinal cord, that can occur in both children and adults, and that is characterized by wide a range of clinical manifestations depending on the location of the tumor, such as intracranial hypertension for tumors originating in the posterior fossa, behavioural changes and pyramidal signs for supratentorial tumors, and dysesthesia for tumors of the spinal cord. They can be classified as myxopapillary ependymoma, subependymoma, ependymoma (low grade tumors) or anaplastic ependymoma (grade III tumors).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-301/","orphanet_url":"https://www.orpha.net/en/disease/detail/301","names":{"fr":{"name":"Tumeur épendymaire","definition":"Tumeur d'origine neurectodermique provenant des cellules épendymaires qui tapissent les ventricules et le canal central de la moelle épinière. Elle peut se développer chez l'enfant comme chez l'adulte et, en fonction de sa localisation, la tumeur se caractérise par de nombreuses manifestations cliniques, telles qu'une hypertension intracrânienne dans le cas des tumeurs originaires de la fosse postérieure, des troubles comportementaux et des signes d'atteinte pyramidale dans le cas des tumeurs supratentorielles et une dysesthésie dans le cas des tumeurs de la moelle épinière. Elles peuvent être classées en épendymomes myxopapillaires, sous-épendymomes, épendymomes (tumeurs de bas grade) ou épendymomes anaplasiques (tumeurs de grade III)."},"de":{"name":"Tumor, ependymaler","definition":"Ein Tumor neurektodermalen Ursprungs, der von den Ependymzellen ausgeht, die die Ventrikel und den Zentralkanal des Rückenmarks auskleiden. Er kann sowohl bei Kindern als auch bei Erwachsenen auftreten und ist durch eine Vielzahl klinischer Manifestationen gekennzeichnet, die je nach Lage des Tumors unterschiedlich sind, wie z. B. intrakranielle Hypertonie bei Tumoren, die aus der hinteren Schädelgrube stammen, Verhaltensänderungen und Pyramidenzeichen bei supratentoriellen Tumoren und Dysästhesie bei Tumoren des Rückenmarks. Sie können als myxopapilläres Ependymom, Subependymom, Ependymom (niedriggradige Tumore) oder anaplastisches Ependymom (Grad-III-Tumore) klassifiziert werden."},"es":{"name":"Tumor ependimario","definition":"Es un tumor de origen neuroectodérmico que se origina en las células ependimarias que recubren los ventrículos y el canal central de la médula espinal. Se puede desarrollar tanto en niños como en adultos y está caracterizado por una amplia gama de manifestaciones clínicas dependiendo de la localización del tumor, tales como hipertensión intracraneal en el caso de tumores originados en la fosa posterior, cambios de conducta y signos piramidales en el caso de tumores supratentoriales y disestesia en tumores de la médula espinal. Se puede clasificar en ependimoma mixopapilar, subependimoma, ependimoma (de bajo grado) o ependimoma anaplásico (de grado III)."},"it":{"name":"Tumore ependimale","definition":"Il tumore ependimale è un tumore di origine neuroectodermica che origina dalle cellule ependimali che rivestono i ventricoli e il canale centrale del midollo spinale. Il tumore ependimale si manifesta sia nell'infanzia che nell'età adulta con un'ampia gamma di segni clinici, che dipendono dalla sua localizzazione e comprendono l'ipertensione intracranica, nei tumori che originano nella fossa posteriore, i cambiamenti del comportamento e i segni piramidali, nei tumori sopratentoriali, e la disestesia, nei tumori localizzati nel midollo spinale. Rientrano in questo gruppo di tumori l'ependimoma mixopapillare, il subependimoma, l'ependimoma (tumori benigni o a basso grado di malignità) e l'ependimoma anaplastico (tumori maligni o di grado III)."},"nl":{"name":"Ependymale tumor","definition":"Een tumor van neuro-ectodermale oorsprong die ontstaat uit ependymale cellen die ventrikels en centraal kanaal van ruggenmerg begrenzen. De tumor kan voorkomen in zowel kinderen als volwassenen, en wordt gekarakteriseerd door een brede waaier van klinische manifestaties, afhankelijk van de locatie van de tumor, zoals intracraniële hypertensie voor tumoren die ontstaan in fossa posterior, gedragsveranderingen en piramidale tekenen voor supratentoriële tumoren, en dysesthesie voor tumoren van ruggenmerg. Ze kunnen geclassificeerd worden als myxopapillair ependymoom, subependymoom, ependymoom (laaggradige tumoren) of anaplastisch ependymoom (graad III-tumoren)."},"pt":{"name":"Ependimoma"},"pl":{"name":"Guz pochodzenia ependymalnego"},"cs":{"name":"Ependymální nádor"},"uk":{"name":"Епендимальна пухлина","definition":"A tumor of neurectodermal origin arising from ependymal cells that line the ventricles and central canal of the spinal cord, that can occur in both children and adults, and that is characterized by wide a range of clinical manifestations depending on the location of the tumor, such as intracranial hypertension for tumors originating in the posterior fossa, behavioural changes and pyramidal signs for supratentorial tumors, and dysesthesia for tumors of the spinal cord. They can be classified as myxopapillary ependymoma, subependymoma, ependymoma (low grade tumors) or anaplastic ependymoma (grade III tumors)."},"zh":{"name":"室管膜肿瘤"},"tr":{"name":"Ependimal tümör"}},"xrefs":[{"source":"OMIM","code":"137800","qualifier":"NTBT","icd_relation":null,"validated":true},{"source":"MONDO","code":"0003266","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"3266","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1333407","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[{"type":"Referred to","orpha":251633,"name":"OBSOLETE: Low-grade ependymoma","status":"inactive"}],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":182067,"name":"Glial tumor","classifications":[181,202]}],"children":[{"orpha":251646,"name":"Anaplastic ependymoma","type":"Disease"},{"orpha":251636,"name":"Ependymoma","type":"Disease"},{"orpha":251643,"name":"Myxopapillary ependymoma","type":"Disease"},{"orpha":530792,"name":"RELA fusion-positive ependymoma","type":"Disease"},{"orpha":251639,"name":"Subependymoma","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["All ages"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.2,"area":"Europe","status":"Validated","source":"22227039[PMID]_22033323[PMID]_RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Point prevalence","qualification":"Class only","class":"1-9 / 100 000","value":0.0,"area":"Europe","status":"Validated","source":"22033323[PMID]_RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Lifetime Prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":3.85,"area":"Europe","status":"Validated","source":"22033323[PMID]_RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.2,"area":"United Kingdom","status":"Validated","source":"22227039[PMID]_RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.43,"area":"United States","status":"Validated","source":"Central Brain Tumor OTHERistry of the United States 2010-2014[OTHER]_29117289[PMID]"}],"phenotypes":null}