{"orpha":86900,"name":"Interdigitating dendritic cell sarcoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Interdigitating cell sarcoma","Reticulum cell sarcoma"],"definition":"A rare dendritic cell tumor characterized by a neoplasm composed of spindle to ovoid cells with phenotypic features similar to those of interdigitating dendritic cells. Solitary lymph node involvement is common, although extranodal localization (in particular skin and soft tissue) has also been reported. Patients usually present with an asymptomatic mass, sometimes with systemic symptoms such as fatigue, fever, and night sweats. Generalized lymphadenopathy, splenomegaly, or hepatomegaly may be seen in rare cases. The clinical course is generally aggressive.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-86900/","orphanet_url":"https://www.orpha.net/en/disease/detail/86900","names":{"fr":{"name":"Sarcome des cellules dendritiques interdigitées","synonyms":["Sarcome des cellules interdigitées","Sarcome des cellules réticulaires interdigitées"],"definition":"Tumeur rare des cellules dendritiques caractérisée par un néoplasme composé de cellules fusiformes à ovoïdes présentant des caractéristiques phénotypiques similaires à celles des cellules dendritiques interdigitées. L'atteinte seule des ganglions lymphatiques est fréquente, bien qu'une localisation extranodale (en particulier la peau et les tissus mous) ait également été rapportée. Les patients manifestent généralement une masse asymptomatique, parfois accompagnée de symptômes systémiques tels que fatigue, fièvre et sueurs nocturnes. Une lymphadénopathie généralisée, une splénomégalie ou une hépatomégalie peuvent être observées dans de rares cas. L'évolution clinique est généralement agressive."},"de":{"name":"Interdigitierendes dendritisches Zellsarkom","synonyms":["Interdigitierendes Sarkom der dendritischen Zellen","Zellsarkom, retikuläres"],"definition":"Ein seltener dendritischer Zelltumor, der durch eine Neoplasie aus spindelförmigen bis eiförmigen Zellen gekennzeichnet ist, die phänotypische Merkmale aufweisen, die denen von interdigierenden dendritischen Zellen ähneln. Häufig sind einzelne Lymphknoten befallen, obwohl auch über die Lokalisation außerhalb der Lymphknoten (insbesondere in der Haut und im Weichgewebe) berichtet wurde. Die Patienten stellen sich in der Regel mit einer asymptomatischen Gewebeverdichtung vor, manchmal auch mit systemischen Symptomen wie Müdigkeit, Fieber und Nachtschweiß. In seltenen Fällen kann es zu einer generalisierten Lymphadenopathie, Splenomegalie oder Hepatomegalie kommen. Der klinische Verlauf ist im Allgemeinen aggressiv."},"es":{"name":"Sarcoma de células dendríticas interdigitante","synonyms":["Sarcoma de células interdigitantes","Sarcoma de células reticulares"],"definition":"Es un tumor de células dendríticas poco frecuente caracterizado por una neoplasia compuesta por células entre fusiformes a ovaladas con características fenotípicas similares a las que poseen las células dendríticas interdigitantes. La afectación aislada de los ganglios linfáticos es frecuente, aunque también se ha descrito afectación de localización extraganglionar (en particular cutánea y de los tejidos blandos). Los pacientes se suelen presentar con una masa asintomática, en ocasiones con síntomas sistémicos tales como cansancio, fiebre y sudores nocturnos. En algunos casos, se puede observar linfadenopatía generalizada, esplenomegalia o hepatomegalia. Por lo general, el curso clínico es agresivo."},"it":{"name":"Sarcoma a cellule dendritiche interdigitate","synonyms":["Sarcoma a cellule interdigitate","Sarcoma a cellule reticolari"],"definition":"Si tratta di un tumore raro delle cellule dendritiche, caratterizzato da una neoplasia composta da cellule fusiformi o ovoidali con caratteristiche fenotipiche simili a quelle delle cellule dendritiche interdigitate. Di solito interessa solo i linfonodi, ma sono state documentate anche localizzazioni extranodali (in particolare a livello della cute e dei tessuti molli). In genere i pazienti presentano una massa asintomatica, talvolta associata a sintomi sistemici (affaticamento, febbre e sudorazione notturna). Occasionalmente sono presenti linfoadenopatia generalizzata, splenomegalia ed epatomegalia. Di solito il decorso clinico è aggressivo."},"nl":{"name":"Sarcoom van interdigiterende dendritische cellen","synonyms":["Sarcoom van interdigiterende cellen","Sarcoom van reticulumcellen"],"definition":"Een zeldzame tumor van dendritische cel, gekarakteriseerd door een neoplasma bestaande uit spoelvormige tot ovoïde cellen met fenotypische kenmerken gelijkend op die van interdigiterende dendritische cellen. Betrokkenheid van solitaire lymfeklier komt vaak voor, hoewel extranodale lokalisatie (vooral huid en weke delen) ook reeds werd gerapporteerd. Patiënten presenteren zich meestal met een asymptomatische massa, en soms met systemische symptomen zoals vermoeidheid, koorts, en nachtzweten. Gegeneraliseerde lymfadenopathie, splenomegalie, of hepatomegalie kunnen waargenomen worden in zeldzame gevallen. Het klinische verloop is doorgaans agressief."},"pt":{"name":"Sarcoma de células dendríticas interdigitantes"},"pl":{"name":"Mięsak ze splatających się komórek dendrytycznych","synonyms":["Interdigitating cell sarcoma","Reticulum cell sarcoma"]},"cs":{"name":"Sarkom z interdigitujících dendritických buněk","synonyms":["Sarkom z interdigitujících buněk"]},"uk":{"name":"Інтердигітальна дендритноклітинна саркома","synonyms":["Інтердигітальна клітинна саркома","Саркома з клітин сітківки"],"definition":"A rare dendritic cell tumor characterized by a neoplasm composed of spindle to ovoid cells with phenotypic features similar to those of interdigitating dendritic cells. Solitary lymph node involvement is common, although extranodal localization (in particular skin and soft tissue) has also been reported. Patients usually present with an asymptomatic mass, sometimes with systemic symptoms such as fatigue, fever, and night sweats. Generalized lymphadenopathy, splenomegaly, or hepatomegaly may be seen in rare cases. The clinical course is generally aggressive."},"zh":{"name":"指突状树突细胞肉瘤","synonyms":["指突状树突细胞肉瘤"]},"tr":{"name":"İç içe geçmiş dendritik hücre sarkomu","synonyms":["Retikulum hücreli sarkom","İnterdijitasyon hücre sarkomu"]}},"xrefs":[{"source":"OMIM","code":"267730","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"C96.4","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"2B31.4","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"214592620"},{"source":"MONDO","code":"0009975","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D054739","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1260326","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10038804","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":98289,"name":"Dendritic cell tumor","classifications":[194,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}