{"orpha":180261,"name":"Phyllodes tumor of the breast","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare fibroepithelial tumor of the breast characterized by a painless, circumscribed, firm mass potentially arising in any part of the breast, histologically showing a prominent intracanalicular growth pattern with leaf-like stromal fronds, capped by luminal epithelial and myoepithelial cell layers, accompanied by stromal hypercellularity. The tumor may be benign, borderline, or malignant.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-180261/","orphanet_url":"https://www.orpha.net/en/disease/detail/180261","names":{"fr":{"name":"Tumeur phyllode du sein","synonyms":["Tumeur phylloïde du sein"],"definition":"Tumeur fibroépithéliale rare du sein caractérisée par une masse indolore, circonscrite, ferme, pouvant affecter toute partie du sein et dont l'examen histologique révèle une croissance intracanaliculaire proéminente avec des frondes stromales en forme de feuilles, recouvertes des couches luminales de cellules épithéliales et myoépithéliales, accompagnées d'une hypercellularité stromale. La tumeur peut être bénigne, à la limite de la malignité ou maligne."},"de":{"name":"Phylloidestumor der Brust","definition":"Ein fibroepithelialer Tumor der Brust, der durch eine schmerzlose, umschriebene, feste Gewebeverdichtung gekennzeichnet ist, die an jeder beliebigen Stelle der Brust auftreten kann und histologisch ein ausgeprägtes intrakanalikuläres Wachstumsmuster mit blattartigen Stromafedern zeigt, die von luminalen Epithel- und Myoepithelzellschichten bedeckt sind, begleitet von einer stromalen Hyperzellularität. Der Tumor kann gutartig, grenzwertig oder maligne sein."},"es":{"name":"Tumor filoide de la mama","definition":"Es un tumor fibroepitelial poco frecuente de la mama caracterizado por una masa indolora, circunscrita y firme que puede surgir en cualquier parte de la mama. Histológicamente muestra un patrón de crecimiento intracanalicular prominente con frondas estromales foliáceas, rematadas por capas de células epiteliales y mioepiteliales luminales, acompañadas de hipercelularidad estromal. El tumor puede ser benigno, <i>borderline</i> o maligno."},"it":{"name":"Tumore filloide della mammella","definition":"Il tumore filloide è una neoplasia fibroepiteliale rara che rappresenta meno dell'1% di tutti i tumori della mammella e di solito esordisce nelle femmine adulte (35-55 anni). Può essere benigno o maligno e spesso si estrinseca con masse mobili circoscritte, che crescono rapidamente, talvolta in associazione con sintomi non specifici, come la dilatazione delle vene della cute, la retrazione del capezzolo, le ulcere cutanee, la linfadenopatia ascellare palpabile o la colorazione blu della cute."},"nl":{"name":"Phyllodestumor van borst","definition":"Een zeldzame fibro-epitheliale tumor van borst, gekenmerkt door een pijnloze, begrensde, solide massa die potentieel kan ontstaan in elk deel van de borst, en histologisch een prominent intracanaliculair groeipatroon vertoont met bladvormige stromale structuren begrensd door luminale epitheliale en myo-epitheliale cellagen, vergezeld van stromale hypercellulariteit. De tumor kan goedaardig, borderline of kwaadaardig zijn."},"pt":{"name":"Tumor filóide da mama","definition":"Tumor fibroepitelial raro da mama, caracterizado por uma massa firme, circunscrita e indolor, potencialmente surgindo em qualquer parte da mama, mostrando histologicamente um padrão de crescimento intracanalicular proeminente com projeções estromais semelhantes a folhas, cobertas por camadas de células epiteliais e mioepiteliais luminais, acompanhadas por hipercelularidade estromal. O tumor pode ser benigno, <i>borderline</i> ou maligno."},"pl":{"name":"Guz liściasty gruczołu piersiowego","synonyms":["Guz liściasty"]},"cs":{"name":"Fyloidní tumor prsu","synonyms":["Cystosarcoma phyllodes"]},"uk":{"name":"Філлодна пухлина молочної залози","definition":"A rare fibroepithelial tumor of the breast characterized by a painless, circumscribed, firm mass potentially arising in any part of the breast, histologically showing a prominent intracanalicular growth pattern with leaf-like stromal fronds, capped by luminal epithelial and myoepithelial cell layers, accompanied by stromal hypercellularity. The tumor may be benign, borderline, or malignant."},"zh":{"name":"乳房叶状肿瘤","synonyms":["乳房叶状囊性肉瘤"],"definition":"乳腺叶状肿瘤是一种罕见的纤维上皮肿瘤，占所有乳腺肿瘤的1%以下，其主要临床表现为边界清楚的活动性肿块，生长迅速，有时还伴有其他非特异性症状，如皮肤静脉曲张、乳头凹陷、皮肤溃疡、可触及的腋窝淋巴结肿大或皮肤呈蓝色，可分为以下3类：良性、交界性、恶性，常发生于成年女性（最常见于35-55岁之间）。"},"tr":{"name":"Memenin filloid tümörü","synonyms":["Memenin sistosarkom filodları"]}},"xrefs":[{"source":"ICD-10","code":"D48.6","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"2F30.3","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"827143668"},{"source":"MONDO","code":"15871","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D003557","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0238031","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"9514","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10011813","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":180253,"name":"Rare benign breast tumor","classifications":[202,205]},{"orpha":180257,"name":"Rare malignant breast tumor","classifications":[202,205]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adult"],"prevalence":[],"phenotypes":null}