{"orpha":213630,"name":"Primitive neuroectodermal tumor of the corpus uteri","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Malignant peripheral neuroectodermal tumor of the corpus uteri","Peripheral neuroectodermal cancer of the corpus uteri"],"definition":"Primitive neuroectodermal tumor of the corpus uteri is a rare cancer of corpus uteri derived from neural crest cells, characterized by small, round neoplastic cells with variable degree of neural, glial and ependymal differentiation. Macroscopically, the tumor is often a large, poorly circumscribed polypoid mass with necrotic areas and hemorrhage. It usually presents with lower abdominal or pelvic pain, irregular vaginal bleeding or discharge, pelvic mass and uterine enlargement.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-213630/","orphanet_url":"https://www.orpha.net/en/disease/detail/213630","names":{"fr":{"name":"Tumeur neuroectodermique primitive du corps de l'utérus","synonyms":["Cancer neuroectodermique périphérique du corps de l'utérus","Tumeur neuroectodermique périphérique maligne du corps de l'utérus"],"definition":"Un cancer rare du corps de l'utérus issu des cellules des crêtes neurales, et caractérisé par de petites cellules néoplasiques rondes présentant des degrés variables de différenciation épendymaire, gliale et neurale. Sur le plan macroscopique, la tumeur se présente souvent sous la forme d'une large masse polypoïde peu circonscrite, avec des zones nécrotiques et une hémorragie. Elle se manifeste habituellement par une douleur pelvienne ou dans le bas ventre, une perte ou un saignement vaginal irrégulier, une masse pelvienne et une dilatation utérine."},"de":{"name":"Primitiver neuroektodermaler Tumor des Corpus uteri","synonyms":["Maligner peripherer neuroektodermaler Tumor des Corpus uteri","PNET des Uterus"],"definition":"Der primitive neuroektodermale Tumor des Corpus uteri ist ein seltener Krebs des Gebärmutterkörpers, der sich von Neuralleistenzellen ableitet und durch kleine, runde neoplastische Zellen mit variablem Grad der neuralen, glialen und ependymalen Differenzierung gekennzeichnet ist. Makroskopisch ist der Tumor oft eine große, schlecht umschriebene polypoide Masse mit nekrotischen Bereichen und Blutungen. Er präsentiert sich in der Regel mit Unterbauch- oder Beckenschmerzen, unregelmäßigen vaginalen Blutungen oder Ausfluss, Beckenmasse und Uterusvergrößerung."},"es":{"name":"Tumor neuroectodérmico primitivo del cuerpo uterino","synonyms":["Cáncer neuroectodérmico periférico del cuerpo uterino","Tumor neuroectodérmico periférico maligno del cuerpo uterino"],"definition":"Es un cáncer poco frecuente del cuerpo uterino derivado de células de la cresta neural. Está caracterizado por células neoplásicas pequeñas y redondas con un grado variable de diferenciación neural, glial y ependimaria. Macroscópicamente, el tumor se presenta a menudo como una masa polipoide grande, mal delimitada, con áreas necróticas y hemorragia. Por lo general, se presenta con dolor abdominal inferior o pélvico, sangrado o flujo vaginal irregular, masa pélvica y agrandamiento uterino."},"it":{"name":"Tumore neuroectodermico primitivo del corpo dell'utero","synonyms":["Cancro neuroectodermico periferico del corpo uterino"],"definition":"Il tumore neuroectodermico primitivo del corpo dell'utero è una neoplasia rara che origina delle cellule della cresta neurale ed è caratterizzata da piccole cellule neoplastiche arrotondate, con differenziazione neurale, gliale ed ependimale di grado variabile. Dal punto di vista macroscopico, spesso consiste di una massa polipoide di ampie dimensioni, scarsamente circoscritta, che presenta aree necrotiche ed emorragie. Di solito esordisce con dolore pelvico o nella parte inferiore dell'addome, emorragie o perdite vaginali irregolari, presenza di una massa pelvica e allargamento dell'utero"},"nl":{"name":"Primitieve neuro-ectodermale tumor van corpus uteri","synonyms":["Maligne perifere neuro-ectodermale tumor van baarmoederlichaam","Maligne perifere neuro-ectodermale tumor van corpus uteri","Perifere neuro-ectodermale kanker van baarmoederlichaam","Perifere neuro-ectodermale kanker van corpus uteri","Primitieve neuro-ectodermale tumor van baarmoederlichaam"],"definition":"Primitieve neuro-ectodermale tumor van het corpus uteri is een zeldzame kanker van het baarmoederlichaam die is afgeleid van cellen van de neurale lijst, en die gekarakteriseerd wordt door kleine, ronde neoplastische cellen met variabele gradaties van neurale, gliale en ependymale differentiatie. Macroscopisch is de tumor vaak een grote, vaag begrensde polypoïde massa met necrotische gebieden en hemorragie. Het presenteert zich meestal met pijn in de onderbuik of het bekken, onregelmatige vaginale bloeding of afscheiding, pelviene massa en vergroting van de baarmoeder."},"pt":{"name":"Tumor maligno neuroectodérmico periférico do corpo uterino"},"pl":{"name":"Pierwotny guz neuroektodermalny trzonu macicy","synonyms":["Obwodowy guz neuroektodermalny trzonu macicy","Złośliwy obwodowy guz neuroektodermalny trzonu macicy"]},"cs":{"name":"Maligní periferní neuroektodermální nádor těla děložního","synonyms":["Periferní neuroektodermální rakovina těla děložního"]},"uk":{"name":"Примітивна нейроектодермальна пухлина тіла матки","synonyms":["Злоякісна периферична нейроектодермальна пухлина тіла матки","Периферичний нейроектодермальний рак тіла матки"],"definition":"Primitive neuroectodermal tumor of the corpus uteri is a rare cancer of corpus uteri derived from neural crest cells, characterized by small, round neoplastic cells with variable degree of neural, glial and ependymal differentiation. Macroscopically, the tumor is often a large, poorly circumscribed polypoid mass with necrotic areas and hemorrhage. It usually presents with lower abdominal or pelvic pain, irregular vaginal bleeding or discharge, pelvic mass and uterine enlargement."},"zh":{"name":"子宫体原始神经外胚层瘤","synonyms":["子宫体恶性外周神经外胚层肿瘤"],"definition":"子宫体原发神经外胚层肿瘤是一种罕见的来源于神经嵴细胞的子宫体肿瘤，其特征是小而圆的肿瘤细胞伴不同程度的神经、胶质和室管膜分化。肉眼上该肿瘤为大的、边界不清的息肉样肿块，伴有坏死和出血。本病通常表现为下腹或盆腔疼痛、不规则阴道出血或分泌物、盆腔肿块和子宫增大。"},"tr":{"name":"Korpus uteri'nin primitif nöroektodermal tümörü","synonyms":["Korpus uteri'nin periferik nöroektodermal kanseri","Korpus uteri'nin habis periferik nöroektodermal tümörü"]}},"xrefs":[{"source":"ICD-10","code":"C54.0","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C54.1","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C54.2","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C54.3","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C54.8","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2B5F.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1713777531"},{"source":"MONDO","code":"0016263","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4707724","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":213620,"name":"Sarcoma of the corpus uteri","classifications":[202,205]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}