{"orpha":498228,"name":"Phyllodes tumor of the prostate","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Cystic epithelial-stromal tumors of the prostate","Cystosarcoma phyllodes of the prostate","Phyllodes type of atypical prostatic hyperplasia"],"definition":"A rare urogenital tumor characterized by stromal and epithelial components forming cysts lined by hyperplastic epithelium in a cellular or sarcomatoid stroma. The tumors may be clinically benign or malignant and tend to recur after transurethral resection. Metastatic spread is to lungs, bone, and liver. Patients may present with obstructive voiding symptoms, dysuria, hematuria, urinary retention, or a palpable abdominal mass. The prostate is palpably enlarged but feels soft and spongy.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-498228/","orphanet_url":"https://www.orpha.net/en/disease/detail/498228","names":{"fr":{"name":"Tumeur phyllode de la prostate","synonyms":["Cystosarcome phyllode de la prostate","Cystosarcome phylloïde de la prostate","Tumeur phylloïde de la prostate"],"definition":"Tumeur urogénitale rare caractérisée par des kystes à composante stromale et épithéliale, formant des kystes tapissés d'un épithélium hyperplasique dans un stroma cellulaire ou sarcomatoïde. Sur le plan clinique, les tumeurs peuvent être bénignes ou malignes et avoir tendance à récidiver après une résection transurétrale. Les poumons, les os et le foie sont les cibles des métastases. Les patients peuvent présenter des symptômes de troubles mictionnels obstructifs, une dysurie, une hématurie, une rétention urinaire. La palpation permet d'identifier une masse abdominale et une augmentation de la taille de la prostate dont l'aspect au toucher est plutôt mou et spongieux."},"de":{"name":"Phylloidtumor der Prostata","synonyms":["Cystosarcoma phyllodes der Prostata"],"definition":"Ein seltener urogenitaler Tumor, der durch stromale und epitheliale Komponenten gekennzeichnet ist, die Zysten bilden, die von einem hyperplastischen Epithel in einem zellulären oder sarkomatoiden Stroma ausgekleidet sind. Die Tumoren können klinisch benigne oder maligne sein und neigen nach transurethraler Resektion zum Rezidiv. Metastasen können sich in Lunge, Knochen und Leber ausbreiten. Die Patienten können sich mit obstruktiven Miktionssymptomen, Dysurie, Hämaturie, Harnverhalt oder tastbaren Gewebeverdichtungen im Bauchraum vorstellen. Die Prostata ist tastbar vergrößert, fühlt sich aber weich und schwammig an."},"es":{"name":"Tumor filoides de la próstata","synonyms":["Cistosarcoma filoides de la próstata"],"definition":"Es un tumor urogenital poco frecuente caracterizado por componentes estromales y epiteliales formando quistes revestidos por epitelio hiperplásico en un estroma celular o sarcomatoide. Los tumores pueden ser clínicamente benignos o malignos y suelen recurrir después de la resección transuretral. La diseminación metastásica suele afectar a los pulmones, los huesos y el hígado. Los pacientes pueden presentarse con síntomas de micción obstructiva, disuria, hematuria, retención urinaria o una masa abdominal palpable. La próstata aumenta de tamaño de forma palpable, notándose blanda y esponjosa."},"it":{"name":"Tumore filloide della prostata","definition":"Si tratta di un tumore urogenitale raro, caratterizzato da componenti stromali ed epiteliali che formano cisti rivestite da epitelio iperplastico in uno stroma cellulare o sarcomatoide. Dal punto di vista clinico, i tumori possono essere benigni o maligni, e tendono a recidivare dopo la resezione transuretrale. Le metastasi si localizzano nei polmoni, nelle ossa e nel fegato. I pazienti possono presentare ostruzione minzionale, disuria, ematuria, ritenzione urinaria o una massa addominale palpabile. La prostata risulta ingrossata alla palpazione, ed è morbida e spugnosa."},"nl":{"name":"Phyllodestumor van prostaat","synonyms":["Atypische hyperplasie van prostaat, phyllodes-type","Cystische epitheliale-stromale tumoren van prostaat","Cystosarcoma phyllodes van prostaat"],"definition":"Een zeldzame urogenitale tumor, gekarakteriseerd door stromale en epitheliale componenten die cysten bekleed met hyperplastisch epitheel vormen in een cellulair of sarcomatoïd stroma. De tumoren kunnen klinisch goed- of kwaadaardig zijn, en hebben de neiging terug te keren na transurethrale excisie. Er treedt metastase op naar long, bot en lever. Patiënten kunnen zich presenteren met symptomen van obstructie van mictie, dysurie, hematurie, urineretentie, of een palpabele abdominale massa. De prostaat is voelbaar vergroot, maar voelt zacht en sponzig aan."},"pt":{"name":"Tumor filóide da próstata"},"pl":{"name":"Guz liściasty prostaty","synonyms":["Cystic epithelial-stromal tumors of the prostate","Cystosarcoma phyllodes of the prostate","Phyllodes type of atypical prostatic hyperplasia"]},"cs":{"name":"Phyllodes tumor prostaty","synonyms":["Cystic epithelial-stromal tumors of the prostate","Cystosarcoma phyllodes of the prostate","Phyllodes type of atypical prostatic hyperplasia"]},"uk":{"name":"Фіброзна пухлина передміхурової залози","synonyms":["Кістозні епітеліально-стромальні пухлини передміхурової залози","Фіброаденома передміхурової залози","Аденома передміхурової залози за типом Філлодеса"],"definition":"A rare urogenital tumor characterized by stromal and epithelial components forming cysts lined by hyperplastic epithelium in a cellular or sarcomatoid stroma. The tumors may be clinically benign or malignant and tend to recur after transurethral resection. Metastatic spread is to lungs, bone, and liver. Patients may present with obstructive voiding symptoms, dysuria, hematuria, urinary retention, or a palpable abdominal mass. The prostate is palpably enlarged but feels soft and spongy."},"zh":{"name":"前列腺叶状肿瘤","synonyms":["叶状不典型前列腺增生"]},"tr":{"name":"Prostatın filloid tümörü","synonyms":["Prostatın sistosarkom filodları","Prostatın kistik epitel-stromal tümörleri","Atipik prostat hiperplazisinin filloid tipi"]}},"xrefs":[{"source":"ICD-10","code":"C61","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C82.Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0021102","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1335409","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"9404","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":98058,"name":"Rare urinary tract tumor","classifications":[185,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult","Elderly"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":90.0,"area":"Worldwide","status":"Validated","source":"25210561[PMID]"}],"phenotypes":null}