{"orpha":251919,"name":"Pineal parenchymal tumor of intermediate differentiation","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare type of pineal parenchymal tumor (PPT) of intermediate-grade malignancy manifesting with visual disturbances, headaches, loss of coordination and balance, nausea and vomiting due to obstructive hydrocephalus, and that is classified as either grade II PPTID (pineal parenchymal tumor of intermediate differentiation) or grade III PPTID according to the degree of neuronal differentiation and mitotic activity.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-251919/","orphanet_url":"https://www.orpha.net/en/disease/detail/251919","names":{"fr":{"name":"Tumeur du parenchyme pinéal à différenciation intermédiaire","definition":"Type rare de tumeur du parenchyme pinéal (TPP) de malignité de grade intermédiaire. Elle se manifeste par des troubles de la vue, des céphalées, une perte de la coordination et de l'équilibre, des nausées et des vomissements, causés par une hydrocéphalie obstructive. Elle est classée en TPPDI (tumeur du parenchyme pinéal à différenciation intermédiaire) de grade II ou III en fonction du degré de différenciation neuronale et de l'activité mitotique."},"de":{"name":"Parenchymatöser Pinealistumor intermediärer Differenzierung","definition":"Eine seltene Form des pinealen Parenchymtumors (PPT) mit intermediärer Malignität, der sich durch Sehstörungen, Kopfschmerzen, Koordinations- und Gleichgewichtsstörungen, Übelkeit und Erbrechen aufgrund eines obstruktiven Hydrozephalus manifestiert und je nach Grad der neuronalen Differenzierung und mitotischen Aktivität entweder als PPTID II (pinealer Parenchymtumor mit intermediärer Differenzierung) oder PPTID III klassifiziert wird."},"es":{"name":"Tumor del parénquima pineal de diferenciación intermedia","definition":"Es un tipo poco frecuente de tumor del parénquima pineal (PPT) con una malignidad de grado intermedio que se manifiesta con alteraciones visuales, cefalea, pérdida de coordinación y equilibrio, nauseas y vómitos debidos a una hidrocefalia obstructiva. Se clasifican como PPTID grado II (tumor del parénquima pineal de diferenciación intermedia) o PPTID grado III en función del nivel de diferenciación neuronal y de la actividad mitótica."},"it":{"name":"Tumore del parenchima pineale a differenziazione intermedia","definition":"Il tumore del parenchima pineale a differenziazione intermedia (PPTID) definisce una forma rara di tumore del parenchima pineale (PPT), a malignità intermedia, che si presenta con disturbi visivi, cefalee, perdita della coordinazione e dell'equilibrio, nausea e vomito da idrocefalo ostruttivo. Si distingue in PPTID allo stadio II o PPTID allo stadio III, a seconda della differenziazione neuronale e dell'attività mitotica."},"nl":{"name":"Pineale parenchymale tumor van intermediaire differentiatie","definition":"Een zeldzaam type van pineale parenchymale tumor (PPT) met intermediaire graad van maligniteit, die zich manifesteert met visuele stoornissen, hoofdpijn, verlies van coördinatie en evenwicht, misselijkheid en braken door obstructieve hydrocefalie, en die afhankelijk van de mate van neuronale differentiatie en mitotische activiteit wordt geclassificeerd als graad II PPTID (pineale parenchymale tumor met intermediaire differentiatie) of graad III PPTID."},"pt":{"name":"Tumor do parênquima pineal de diferenciação intermédia"},"pl":{"name":"Guz miąższu szyszynki o pośrednim zróżnicowaniu"},"cs":{"name":"Pineální parenchymatózní nádor středního stupně diferenciace"},"uk":{"name":"Паренхіматозна пухлина шишкоподібної залози проміжного диференціювання","definition":"A rare type of pineal parenchymal tumor (PPT) of intermediate-grade malignancy manifesting with visual disturbances, headaches, loss of coordination and balance, nausea and vomiting due to obstructive hydrocephalus, and that is classified as either grade II PPTID (pineal parenchymal tumor of intermediate differentiation) or grade III PPTID according to the degree of neuronal differentiation and mitotic activity."},"zh":{"name":"松果体中分化实质瘤","definition":"松果体中分化肿瘤(PPTID)是一种少见的中度恶性松果体肿瘤，临床表现为视力障碍、头痛、协调平衡障碍、梗阻性脑积水引起恶心呕吐，根据神经元分化程度和核分裂活性分为II级PPTID和III级PPTID。"},"tr":{"name":"Ara farklılaşmanın epifiz parankimal tümörü"}},"xrefs":[{"source":"ICD-10","code":"D44.5","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2A00.20","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1915762610"},{"source":"ICD-11","code":"XH1S48","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"174460848"},{"source":"MONDO","code":"0006369","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1367859","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10644","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":251905,"name":"Pineal tumor of neuroepithelial tissue","classifications":[181,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}