{"orpha":199260,"name":"Calcifying aponeurotic fibroma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Juvenile aponeurotic fibromatosis","Keasby tumor"],"definition":"A rare, superficial fibromatosis characterized by non-malignant, locally invading, fibrosing tumour of differentiated fibroblasts, slowly growing subcutaneously, occurring predominantly distally on the extremities, especially the hands and feet. Histologic examination shows a multinodular pattern with large areas of calcification and fibrosis, and the presence of elongated spindle cells with hyperchromatic plump vesicular nuclei interspersed within fine bands of collagen.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-199260/","orphanet_url":"https://www.orpha.net/en/disease/detail/199260","names":{"fr":{"name":"Fibrome aponévrotique calcifié","synonyms":["Fibromatose aponévrotique juvénile","Tumeur de Keasby"],"definition":"Fibromatose superficielle rare caractérisée par une tumeur fibrosante bénigne et localement invasive des fibroblastes différenciés, de progression sous-cutanée lente et siégeant principalement sur la partie distale des extrémités (notamment les mains et les pieds). L'examen histologique met en évidence un aspect multinodulaire avec de vastes zones de calcification et de fibrose, et révèle la présence de cellules fusiformes allongées dotées de noyaux vésiculaires hyperchromatiques, dispersées à l'intérieur de petites bandes de collagènes."},"de":{"name":"Fibrom, aponeurotisches kalzifizierendes","synonyms":["Fibromatose, aponeurotische juvenile","Keasby-Tumor"],"definition":"Eine seltene, oberflächliche Fibromatose, die durch einen nicht malignen, lokal eindringenden, fibrosierenden Tumor aus differenzierten Fibroblasten gekennzeichnet ist, der langsam subkutan wächst und vorwiegend distal an den Extremitäten (insbesondere an Händen und Füßen) auftritt. Die histologische Untersuchung zeigt ein multinoduläres Muster mit großen Bereichen von Verkalkung und Fibrose und das Vorhandensein von länglichen Spindelzellen mit hyperchromatischen, plump vesikulären Kernen, die von feinen Kollagenbändern durchsetzt sind."},"es":{"name":"Fibroma aponeurótico calcificado","synonyms":["Fibromatosis aponeurótica juvenil","Tumor de Keasby"],"definition":"Es una fibromatosis superficial poco frecuente caracterizada por un tumor fibroso benigno compuesto por fibroblastos diferenciados, localmente invasivo, de crecimiento lento y subcutáneo que se presenta predominantemente en la parte distal de las extremidades, especialmente en las manos y los pies. El examen histológico revela un patrón de crecimiento multinodular con grandes áreas de calcificación y fibrosis, y presencia de células fusiformes alargadas con núcleos vesiculares grandes e hipercromáticos dispuestos entre finas bandas de colágeno."},"it":{"name":"Fibroma aponeurotico calcificante","synonyms":["Fibromatosi aponeurotica giovanile","Tumore di Keasby"],"definition":"È una fibromatosi superficiale rara, caratterizzata da una neoplasia fibrosante benigna, localmente invasiva, composta da fibroblasti differenziati, a crescita sottocutanea lenta, localizzata prevalentemente sulla porzione distale degli arti, in particolare sulle mani e sui piedi. L'esame istologico evidenzia un aspetto multinodulare con ampie aree di calcificazione e fibrosi, e cellule fusiformi allungate con ampi nuclei vescicolari ipercromatici sparsi all'interno di sottili bande di collagene."},"nl":{"name":"Calcificerend aponeurotisch fibroom","synonyms":["Juveniele aponeurotische fibromatose","Keasby-tumor"],"definition":"Een zeldzame, oppervlakkige fibromatose gekarakteriseerd door een niet-maligne, lokaal invasieve, fibroserende tumor van gedifferentieerde fibroblasten, die traag onderhuids groeit en voornamelijk distaal op de ledematen voorkomt, vooral de handen en voeten. Histologisch onderzoek toont een multinodulair patroon met grote delen met calcificatie en fibrose, en de aanwezigheid van langwerpige spoelcellen met hyperchromatische, grote, vesiculaire kernen tussen fijne collageenbanden."},"pt":{"name":"Fibroma aponevrótico calcificado"},"pl":{"name":"Zwapniały włókniak rozcięgna","synonyms":["Guz Keasby'ego","Młodzieńcza włókniakowatość rozcięgna"]},"cs":{"name":"Kalcifikující aponeurotický fibrom","synonyms":["Juvenilní aponeurotický fibrom","Keasbyův nádor"]},"uk":{"name":"Кальцинуюча апоневротична фіброма","synonyms":["Ювенільний апоневротичний фіброматоз","Пухлина Кізбі"],"definition":"A rare, superficial fibromatosis characterized by non-malignant, locally invading, fibrosing tumour of differentiated fibroblasts, slowly growing subcutaneously, occurring predominantly distally on the extremities, especially the hands and feet. Histologic examination shows a multinodular pattern with large areas of calcification and fibrosis, and the presence of elongated spindle cells with hyperchromatic plump vesicular nuclei interspersed within fine bands of collagen."},"zh":{"name":"钙化性腱膜纤维瘤","synonyms":["青少年腱膜纤维瘤病"],"definition":"钙化性腱膜纤维瘤是一种罕见的浅表性纤维瘤病变，其主要临床表现为分化的成纤维细胞的非恶性纤维瘤，局部浸润，皮下生长缓慢，主要发生于四肢远端，尤其是手和脚。组织学表现为大面积钙化和纤维化的多结节样病变，可见细长的梭形细胞，胞核深染、饱满，分布于胶原细条带内。"},"tr":{"name":"Kalsifiye aponevrotik fibrom","synonyms":["Keasby tümörü","jüvenil aponevrotik fibromatoz"]}},"xrefs":[{"source":"ICD-10","code":"M72.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"EE61","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0016038","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"C000625499","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0553647","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":199257,"name":"Superficial fibromatosis","classifications":[187,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}