Review of renal tumors associated with Birt-Hogg-Dube syndrome with focus on clinical and pathobiological aspects

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11140%2F14%3A10271936" target="_blank" >RIV/00216208:11140/14:10271936 - isvavai.cz</a>

Alternative codes found

RIV/00669806:_____/14:10271936

Result on the web

<a href="http://dx.doi.org/10.5114/PJP.2014.43958" target="_blank" >http://dx.doi.org/10.5114/PJP.2014.43958</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.5114/PJP.2014.43958" target="_blank" >10.5114/PJP.2014.43958</a>

Result language

angličtina

Original language name

Review of renal tumors associated with Birt-Hogg-Dube syndrome with focus on clinical and pathobiological aspects

Original language description

Birt-Hogg-Dube syndrome (BHDS) is an autosomal dominant inherited disorder characterized by clinical features of skin lesions, pulmonary lesions and renal tumor. The gene responsible for this syndrome is located on chromosome 17p11.2 and designated as FLCN. In this article, we review renal tumors associated with BHDS with a focus on clinical and pathobiological aspects. Renal tumors often occur multifocally or bilaterally in the imaging analyses or gross examination. Histological examination of renal tumors includes a variety of subtypes such as hybrid oncocytic tumor, chromophobe renal cell carcinoma (RCC), oncocytoma, clear cell RCC and papillary RCC. The histologic discordance in multiple tumors seems to be characteristic of this syndrome. Oncocytosis is observed histologically in about half of the cases. Several investigations have elucidated that folliculin may be involved in the mammalian target of rapamycin (mTOR) pathway recently. Renal tumors composed of clear cells may behave

Czech name

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Czech description

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Type

J_x - Unclassified - Peer-reviewed scientific article (Jimp, Jsc and Jost)

CEP classification

FJ - Surgery including transplantology

OECD FORD branch

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Project

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Continuities

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Publication year

2014

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

Polish Journal of Pathology

ISSN

1233-9687

e-ISSN

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Volume of the periodical

65

Issue of the periodical within the volume

2

Country of publishing house

PL - POLAND

Number of pages

7

Pages from-to

93-99

UT code for WoS article

000339365300002

EID of the result in the Scopus database

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