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Statements

Subject Item
n2:RIV%2F00669806%3A_____%2F11%3A10105904%21RIV12-MZ0-00669806
rdf:type
skos:Concept n4:Vysledek
rdfs:seeAlso
http://journals.lww.com/ajsp/Abstract/2011/02000/Pure_Epithelioid_PEComas__So_Called_Epithelioid.1.aspx
dcterms:description
Epithelioid angiomyolipomas (perivascular epithelioid cell tumors) of the kidney are defined as potentially malignant mesenchymal lesions that are closely related to classic angiomyolipoma. Although approximately 120 cases are published, mostly as case reports with variably used diagnostic criteria, the pathologic prognostic predictors of outcome are unknown. We analyzed the clinicopathologic parameters in a large series of 41 cases of pure epithelioid angiomyolipomas of the kidney, which we designate as pure (monotypic) epithelioid PEComas to contrast them from classic angiomyolipomas that are regarded by some as PEComas. We use the terminology %22pure%22 to separate these cases from those that may have variable epithelioid components. The mean age of the patients was 40.7 years (range, 14 to 68 y). The male-to-female ratio was 1:1. Seventy-nine percent of patients were symptomatic at presentation with metastatic disease at onset in 12 cases. Follow-up and/or disease progression information were available for 33 of 41 cases (mean, 44.5 mo and median, 24.5 mo; range, 4 to 240); 9 patients had a history of associated tuberous sclerosis. Recurrence and metastasis were seen in 17% and 49% of patients; 33% of patients died of disease. Epithelioid angiomyolipomas (perivascular epithelioid cell tumors) of the kidney are defined as potentially malignant mesenchymal lesions that are closely related to classic angiomyolipoma. Although approximately 120 cases are published, mostly as case reports with variably used diagnostic criteria, the pathologic prognostic predictors of outcome are unknown. We analyzed the clinicopathologic parameters in a large series of 41 cases of pure epithelioid angiomyolipomas of the kidney, which we designate as pure (monotypic) epithelioid PEComas to contrast them from classic angiomyolipomas that are regarded by some as PEComas. We use the terminology %22pure%22 to separate these cases from those that may have variable epithelioid components. The mean age of the patients was 40.7 years (range, 14 to 68 y). The male-to-female ratio was 1:1. Seventy-nine percent of patients were symptomatic at presentation with metastatic disease at onset in 12 cases. Follow-up and/or disease progression information were available for 33 of 41 cases (mean, 44.5 mo and median, 24.5 mo; range, 4 to 240); 9 patients had a history of associated tuberous sclerosis. Recurrence and metastasis were seen in 17% and 49% of patients; 33% of patients died of disease.
dcterms:title
Pure epithelioid PEComas (so-called epithelioid angiomyolipoma) of the kidney: A clinicopathologic study of 41 cases: detailed assessment of morphology and risk stratification Pure epithelioid PEComas (so-called epithelioid angiomyolipoma) of the kidney: A clinicopathologic study of 41 cases: detailed assessment of morphology and risk stratification
skos:prefLabel
Pure epithelioid PEComas (so-called epithelioid angiomyolipoma) of the kidney: A clinicopathologic study of 41 cases: detailed assessment of morphology and risk stratification Pure epithelioid PEComas (so-called epithelioid angiomyolipoma) of the kidney: A clinicopathologic study of 41 cases: detailed assessment of morphology and risk stratification
skos:notation
RIV/00669806:_____/11:10105904!RIV12-MZ0-00669806
n4:predkladatel
n5:ico%3A00669806
n3:aktivita
n14:V
n3:aktivity
V
n3:cisloPeriodika
2
n3:dodaniDat
n18:2012
n3:domaciTvurceVysledku
n17:9342710
n3:druhVysledku
n8:J
n3:duvernostUdaju
n12:S
n3:entitaPredkladatele
n13:predkladatel
n3:idSjednocenehoVysledku
225284
n3:idVysledku
RIV/00669806:_____/11:10105904
n3:jazykVysledku
n6:eng
n3:klicovaSlova
outcome; morphology; tuberous sclerosis complex; prognosis; angiomyolipoma; epithelioid angiomyolipoma; pure PEComa; PEComa; kidney
n3:klicoveSlovo
n10:prognosis n10:epithelioid%20angiomyolipoma n10:outcome n10:tuberous%20sclerosis%20complex n10:angiomyolipoma n10:kidney n10:PEComa n10:pure%20PEComa n10:morphology
n3:kodStatuVydavatele
US - Spojené státy americké
n3:kontrolniKodProRIV
[A076277F2C40]
n3:nazevZdroje
American Journal of Surgical Pathology
n3:obor
n19:FP
n3:pocetDomacichTvurcuVysledku
1
n3:pocetTvurcuVysledku
20
n3:rokUplatneniVysledku
n18:2011
n3:svazekPeriodika
35
n3:tvurceVysledku
Pea, Maurizio Gown, Allen M. Rogatko, Andre Amin, Mitual B. Fletcher, Christopher D. Kim, Hyung Vankalakunti, Mahesha Berel, Dror Švec, Alexandr Pan, Chin-Chan Ro, Jae Y. Amin, Mahul B. Hwang, Il S. Hes, Ondřej Martignoni, Guido Gupta, Ruta Sato, Katsuaki Bonetti, Franco Kida, Masatoshi Nese, Nalan
n3:wos
000286581700001
s:issn
0147-5185
s:numberOfPages
16
n11:doi
10.1097/PAS.0b013e318206f2a9