{"orpha":86903,"name":"Dendritic cell sarcoma not otherwise specified","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare dendritic cell tumor characterized by a neoplasm composed of spindled to ovoid cells with phenotypic features similar to those of normal indeterminate cells. The tumor cells consistently express S100 protein and CD1a, while langerin, specific B- and T-cell markers, CD30, the histiocytic marker CD163, and the follicular dendritic cell markers CD21, CD23, and CD35 are negative. Birbeck granules are absent on ultrastructural examination. Patients typically present with multiple papules, nodules, or plaques of the skin. Primary lymph node or splenic involvement is less common. Systemic symptoms are usually absent. The clinical course is highly variable.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-86903/","orphanet_url":"https://www.orpha.net/en/disease/detail/86903","names":{"fr":{"name":"Sarcome des cellules dendritiques sans autre spécification","definition":"Tumeur des cellules dendritiques caractérisée par une tumeur néoplasme composée de cellules allant de fusiformes à ovoïdes, présentant des caractéristiques phénotypiques similaires à celles des cellules indéterminées normales. Les cellules tumorales expriment systématiquement la protéine S100 et le CD1a, tandis que la langérine, les marqueurs spécifiques des cellules B et T, le CD30, le marqueur histiocytaire CD163 et les marqueurs des cellules dendritiques folliculaires CD21, CD23 et CD35 sont négatifs. Les granules de Birbeck sont absents à l'examen ultrastructural. Les patients présentent généralement de multiples papules, nodules ou plaques sur la peau. L'atteinte primaire des ganglions lymphatiques ou de la rate est moins fréquente. Les symptômes systémiques sont généralement absents. L'évolution clinique est très variable."},"de":{"name":"Dendritisches Zell-Sarkom, andernorts nicht klassifiziert","definition":"Ein seltener dendritischer Zelltumor, der durch eine Neoplasie gekennzeichnet ist, die aus spindelförmigen bis eiförmigen Zellen besteht und phänotypische Merkmale aufweist, die denen normaler unbestimmter Zellen ähneln. Die Tumorzellen exprimieren durchweg S100-Protein und CD1a, während Langerin, spezifische B- und T-Zell-Marker, CD30, der histiozytäre Marker CD163 und die follikulären dendritischen Zellmarker CD21, CD23 und CD35 negativ sind. Bei der ultrastrukturellen Untersuchung sind keine Birbeck-Granula zu finden. Die Patienten zeigen typischerweise multiple Papeln, Knötchen oder Plaques auf der Haut. Ein primärer Lymphknoten- oder Milzbefall ist weniger häufig. Systemische Symptome sind in der Regel nicht vorhanden. Der klinische Verlauf ist sehr variabel."},"es":{"name":"Sárcoma de células dendriticas sin otra especificación","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 de las células indeterminadas normales. Las células tumorales expresan constitutivamente la proteína S100 y CD1a, mientras que son negativas para langerina, los marcadores específicos de las células B y T, CD30, el marcador histiocítico CD163 y los marcadores de células foliculares dendríticas CD21, CD23 y CD35. El examen ultraestructural no muestra gránulos de Birbeck. Por lo general, los pacientes presentan múltiples pápulas, nódulos o placas en la piel. La afectación primaria de los ganglios linfáticos o esplénica es menos frecuente. Los síntomas sistémicos suelen estar ausentes. El curso clínico es altamente variable."},"it":{"name":"Sarcoma a cellule dendritiche non atrimenti specificato","definition":"Si tratta di una neoplasia rara a cellule dendritiche, composta da cellule fusiformi o ovoidali con caratteristiche fenotipiche simili a quelle delle cellule indeterminate normali. Le cellule tumorali esprimono sempre la proteina S100 e CD1a, mentre la langerina, i marcatori specifici delle cellule B e T, CD30, il marcatore istiocitario CD163 e i marcatori delle cellule dendritiche follicolari CD21, CD23 e CD35 sono negativi. L'esame ultrastrutturale non evidenzia granuli di Birbeck. Di solito i pazienti presentano papule, noduli o placche cutanee multiple. È raro l'interessamento primario dei linfonodi o della milza. In genere i pazienti non presentano sintomi sistemici. Il decorso clinico è molto variabile."},"nl":{"name":"Dendritische celsarcoom niet anders gespecificeerd","definition":"Een zeldzame tumor van dendritische cel, gekarakteriseerd door een neoplasma bestaande uit spoelvormige tot ovoïde cellen met fenotypische kenmerken gelijkend op die van normale onbepaalde cellen. De tumorcellen brengen voortdurend S100-proteïne en CD1a tot expressie, terwijl ze negatief zijn voor langerine, specifieke merkers van B- en T-cellen, CD30, de histiocytische merker CD163, en de merkers van folliculaire dendritische cellen CD21, CD23 en CD35. Birbeck-korrels zijn afwezig bij ultrastructureel onderzoek. Patiënten presenteren zich doorgaans met multipele papels, noduli, of plaques van huid. Primaire betrokkenheid van lymfeklieren of milt komt minder vaak voor. Systemische symptomen zijn meestal afwezig Het klinische verloop is zeer variabel."},"pt":{"name":"Sarcoma de células dendríticas, não especificado"},"pl":{"name":"Mięsak z komórek dendrytycznych nie sklasyfikowany w inny sposób"},"cs":{"name":"Sarkom z dendritických buněk jinak nespecifikovaný"},"uk":{"name":"Дендритноклітинна саркома, не специфікована","definition":"A rare dendritic cell tumor characterized by a neoplasm composed of spindled to ovoid cells with phenotypic features similar to those of normal indeterminate cells. The tumor cells consistently express S100 protein and CD1a, while langerin, specific B- and T-cell markers, CD30, the histiocytic marker CD163, and the follicular dendritic cell markers CD21, CD23, and CD35 are negative. Birbeck granules are absent on ultrastructural examination. Patients typically present with multiple papules, nodules, or plaques of the skin. Primary lymph node or splenic involvement is less common. Systemic symptoms are usually absent. The clinical course is highly variable."},"zh":{"name":"未分类树突细胞肉瘤"},"tr":{"name":"Dendritik hücre sarkomu, başka türlü tanımlanmamış"}},"xrefs":[{"source":"ICD-10","code":"C96.4","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2B31.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"4254301"},{"source":"ICD-11","code":"XH8Q19","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1559082259"},{"source":"MONDO","code":"0019482","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"19482","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"4380","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1301364","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":[],"prevalence":[],"phenotypes":null}