{"orpha":157826,"name":"Congenital epulis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Congenital gingival cell tumor","Congenital granular cell tumor","Neumann tumor"],"definition":"A rare soft tissue tumor characterized by a benign space occupying lesion in neonates, most typically located on the gingival mucosa overlying the anterior alveolar ridge of the maxilla near the canine, although the mandibular region may also be involved. Females are much more frequently affected than males. The tumor mostly presents as a single lesion, potentially interfering with feeding and respiration. Metastasis, malignant transformation, or recurrence after excision have not been reported.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-157826/","orphanet_url":"https://www.orpha.net/en/disease/detail/157826","names":{"fr":{"name":"Epulis congénital","synonyms":["Tumeur de Neumann","Tumeur gingivale à cellules granuleuses congénitale"],"definition":"Tumeur rare des tissus mous caractérisée par une tumeur bénigne se développant au détriment des tissus adjacents, apparaissant chez les nouveau-nés, le plus souvent située sur la muqueuse gingivale recouvrant la crête alvéolaire mandibulaire antérieure près de la canine, bien que la région mandibulaire puisse également être concernée. Les femmes sont beaucoup plus fréquemment affectées que les hommes. La tumeur se présente le plus souvent comme une lésion isolée, pouvant engendrer une gêne alimentaire et respiratoire. Aucune métastase, transformation maligne ou récidive après excision n'a été rapportée."},"de":{"name":"Epulis, kongenitale","synonyms":["Granularzelltumor, kongenitaler"],"definition":"Ein seltener Weichgewebetumor, der bei Neugeborenen eine gutartige, raumfordernde Läsion darstellt, die in der Regel auf der Zahnfleischschleimhaut über dem vorderen Alveolarkamm des Oberkiefers in der Nähe des Eckzahns auftritt, obwohl auch die Unterkieferregion betroffen sein kann. Frauen sind wesentlich häufiger betroffen als Männer. Der Tumor tritt meist als einzelne Läsion auf und kann die Nahrungsaufnahme und Atmung beeinträchtigen. Über Metastasen, eine maligne Transformation oder ein Rezidiv nach der Exzision wurde nicht berichtet."},"es":{"name":"Epulis congénito","synonyms":["Tumor congénito de células gingivales","Tumor congénito de células granulares","Tumor de Neumann"],"definition":"Es un tumor de tejidos blandos poco frecuente caracterizado por una lesión benigna ocupante de espacio en neonatos, localizada, por lo general, en la mucosa gingival que recubre la cresta alveolar anterior del maxilar próximo al canino, aunque también puede afectar a la región mandibular. Las mujeres se ven afectadas con mucha mayor frecuencia que los varones. El tumor se presenta principalmente como una lesión única, pudiendo interferir con la alimentación y la respiración. No se han descrito metástasis, transformación maligna ni recidiva después de la escisión."},"it":{"name":"Epulide congenita","synonyms":["Tumore congenito delle cellule gengivali","Tumore congenito delle cellule granulari"],"definition":"Si tratta di una neoplasia rara dei tessuti molli, caratterizzata da una lesione benigna espansiva osservata nei neonati, di solito localizzata sulla mucosa gengivale che riveste la cresta alveolare anteriore della mascella vicino ai canini, ma che può interessare anche la regione mandibolare. Le femmine sono molto più colpite. Di solito il tumore si presenta sotto forma di lesione isolata, che può interferire con l'alimentazione e la respirazione. Non sono stati descritti casi di metastasi, evoluzione maligna o ricorrenza dopo l'asportazione."},"nl":{"name":"Congenitale epulis","synonyms":["Congenitale gingivale celtumor","Congenitale granulaire celtumor","Tumor van Neumann"],"definition":"Een zeldzame tumor van weke delen, gekarakteriseerd door een goedaardige, ruimte-innemende laesie bij neonaten, doorgaans gesitueerd op slijmvlies van tandvlees dat het voorste deel van de alveolaire kam van het bovenkaakbeen nabij de hoektanden bedekt, hoewel de onderkaak ook betrokken kan zijn. Vrouwen worden veel vaker getroffen dan mannen. De tumor presenteert zich meestal als een enkelvoudige laesie, en beïnvloedt mogelijk inname van voedsel en ademhaling. Metastase, maligne transformatie, of recidive na excisie werd nog niet gerapporteerd."},"pt":{"name":"Epulis, congénita"},"pl":{"name":"Wrodzony nadziąślak","synonyms":["Wrodzony guz z komórek ziarnistych","Wrodzony ziarniniak dziąsłowy"]},"cs":{"name":"Kongenitální epulis","synonyms":["Vrozený nádor dásní","Vrozený nádor z granulárních buněk"]},"uk":{"name":"Вроджений епуліс","synonyms":["Вроджена клітинна пухлина ясен","Вроджена зерноклітинна пухлина","Пухлина Неймана"],"definition":"A rare soft tissue tumor characterized by a benign space occupying lesion in neonates, most typically located on the gingival mucosa overlying the anterior alveolar ridge of the maxilla near the canine, although the mandibular region may also be involved. Females are much more frequently affected than males. The tumor mostly presents as a single lesion, potentially interfering with feeding and respiration. Metastasis, malignant transformation, or recurrence after excision have not been reported."},"zh":{"name":"先天性牙龈瘤","synonyms":["先天性颗粒细胞瘤"],"definition":"一种罕见的软组织肿瘤，其特征是新生儿良性占位性病变，常位于犬齿附近的上颌骨前牙槽嵴上的牙龈黏膜上，也可能累及下颌骨区域。女性比男性更易患病。肿瘤主要表现为单一病灶，可能干扰进食和呼吸。尚无转移、恶性转化和术后复发的报道。"},"tr":{"name":"Doğumsal epulis","synonyms":["Neumann tümörü","Doğumsal gingival hücreli tümörü","Doğumsal granüler hücreli tümör"]}},"xrefs":[{"source":"ICD-10","code":"K06.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"KC23","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1616915738"},{"source":"MONDO","code":"0015528","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0376319","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":71209,"name":"Rare soft tissue tumor","classifications":[202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Infancy","Neonatal"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"1-9 / 1 000 000","value":0.0,"area":"Europe","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}