{"orpha":86902,"name":"Follicular dendritic cell sarcoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare dendritic cell neoplasm characterized by a proliferation of spindled to ovoid cells with morphological and immunophenotypic features of follicular dendritic cells. Conventional follicular dendritic cell sarcomas are negative for EBV. The tumor arises as a painless, slow-growing mass in lymph nodes (most often cervical), extranodal sites (such as tonsils, gastrointestinal tract, soft tissue, mediastinum, or lung, among others), or both. Paraneoplastic pemphigus may occur in rare cases. Predictive factors are tumor size, presence of coagulative necrosis, mitotic count, and presence of significant cytological atypia.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-86902/","orphanet_url":"https://www.orpha.net/en/disease/detail/86902","names":{"fr":{"name":"Sarcome des cellules folliculaires dendritiques","definition":"Tumeur rare des cellules dendritiques, caractérisée par une prolifération de cellules fusiformes ou ovoïdes présentant des caractéristiques morphologiques et immunophénotypiques des cellules dendritiques folliculaires. Les sarcomes conventionnels de cellules dendritiques folliculaires sont négatifs pour l'EBV. La tumeur se présente sous la forme d'une masse indolore à croissance lente dans les ganglions lymphatiques (le plus souvent cervicaux), dans des sites extranodaux (comme les amygdales, le tractus gastro-intestinal, les tissus mous, le médiastin ou les poumons, entre autres), ou affecte les deux sites. Un pemphigus paranéoplasique peut être observé dans de rares cas. Les facteurs prédictifs sont la taille de la tumeur, la présence de nécrose coagulante, le nombre de mitoses et la présence de nombreuses anomalies cytologiques."},"de":{"name":"Sarkom, follikuläres dendritisches","definition":"Eine seltene Neoplasie dendritischer Zellen, die durch eine Proliferation spindelförmiger bis eiförmiger Zellen mit morphologischen und immunphänotypischen Merkmalen follikulärer dendritischer Zellen gekennzeichnet ist. Konventionelle follikuläre dendritische Zellsarkome sind negativ für EBV. Der Tumor entsteht als schmerzlose, langsam wachsende Gewebeverdichtung in Lymphknoten (meist im Gebärmutterhals), extranodalen Lokalisationen (z. B. Mandeln, Magen-Darm-Trakt, Weichteilgewebe, Mediastinum oder Lunge) oder in beiden. In seltenen Fällen kann ein paraneoplastischer Pemphigus auftreten. Prädiktive Faktoren sind die Tumorgröße, das Vorhandensein einer koagulativen Nekrose, die Mitosezahl und das Vorhandensein einer signifikanten zytologischen Atypie."},"es":{"name":"Sarcoma de células dendríticas foliculares","definition":"Es una neoplasia de células dendríticas poco frecuente caracterizada por la proliferación de células entre fusiformes a ovaladas con características morfológicas e inmunofenotípicas de las células foliculares dendríticas. Los sarcomas de células foliculares dendríticas convencionales son negativos para el VEB. El tumor aparece como una masa indolora de crecimiento lento en los ganglios linfáticos (con mayor frecuencia en los cervicales), en sitios extraganglionares (tales como las amígdalas, el tracto gastrointestinal, los tejidos blandos, el mediastino o el pulmón, entre otros) o en ambos. El pénfigo paraneoplásico puede ocurrir en casos poco frecuentes. Los factores predictivos son el tamaño del tumor, la presencia de necrosis coagulativa, el recuento mitótico y la presencia de una marcada atipia citológica."},"it":{"name":"Sarcoma a cellule follicolari dendritiche","definition":"Si tratta di una neoplasia rara a cellule dendritiche, caratterizzata da una proliferazione di cellule fusiformi o ovoidali con le caratteristiche morfologiche ed immunofenotipiche delle cellule follicolari dendritiche. I sarcomi convenzionali a cellule follicolari dendritiche sono negativi per l'EBV. Il tumore si manifesta sotto forma di massa indolore a crescita lenta localizzata nei linfonodi (di solito quelli cervicali), in siti extranodali (tonsille, tratto gastrointestinale, tessuti molli, mediastino o polmoni) o in entrambe le aree. Raramente si associa al pemfigo paraneoplastico. Costituiscono fattori predittivi le dimensioni del tumore, la presenza di necrosi coagulativa, il numero delle mitosi e la presenza di significative atipie citologiche."},"nl":{"name":"Folliculaire dendritische celsarcoom","definition":"Een zeldzaam neoplasma van dendritische cel, gekarakteriseerd door een proliferatie van spoelvormige tot ovoïde cellen met morfologische en immunofenotypische kenmerken van folliculaire dendritische cellen. Sarcomen van conventionele folliculaire dendritische cellen zijn negatief voor EBV. De tumor ontstaat als een pijnloze, traag groeiende massa in lymfeklieren (meestal cervicaal), op extranodale plaatsen (zoals onder meer keelamandelen, maag-darmstelsel, weke delen, mediastinum, of long), of beiden. Paraneoplastische pemfigus kan voorkomen in zeldzame gevallen. Voorspellende factoren zijn omvang van de tumor, aanwezigheid van coagulatienecrose, aantal mitosen, en aanwezigheid van significante cytologische atypie."},"pt":{"name":"Sarcoma de células foliculares dendríticas"},"pl":{"name":"Mięsak z komórek dendrytycznych grudki"},"cs":{"name":"Sarkom z folikulárních dendritických buněk"},"uk":{"name":"Фолікулярна дендритноклітинна саркома","definition":"A rare dendritic cell neoplasm characterized by a proliferation of spindled to ovoid cells with morphological and immunophenotypic features of follicular dendritic cells. Conventional follicular dendritic cell sarcomas are negative for EBV. The tumor arises as a painless, slow-growing mass in lymph nodes (most often cervical), extranodal sites (such as tonsils, gastrointestinal tract, soft tissue, mediastinum, or lung, among others), or both. Paraneoplastic pemphigus may occur in rare cases. Predictive factors are tumor size, presence of coagulative necrosis, mitotic count, and presence of significant cytological atypia."},"zh":{"name":"滤泡树突状细胞肉瘤"},"tr":{"name":"Foliküler dendritik hücre sarkomu"}},"xrefs":[{"source":"ICD-10","code":"C96.4","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"2B31.5","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"15445528"},{"source":"MONDO","code":"0005764","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D054740","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1260325","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10075332","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":289656,"name":"Epstein-Barr Virus-associated mesenchymal tumor","classifications":[202]},{"orpha":3394,"name":"Soft tissue sarcoma","classifications":[202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["All ages"],"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}