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

Kód výsledku v 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>

Nalezeny alternativní kódy

RIV/00669806:_____/14:10271936

Výsledek na webu

<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>

Jazyk výsledku

angličtina

Název v původním jazyce

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

Popis výsledku v původním jazyce

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

Název v anglickém jazyce

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

Popis výsledku anglicky

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

Druh

J_x - Nezařazeno - Článek v odborném periodiku (Jimp, Jsc a Jost)

CEP obor

FJ - Chirurgie včetně transplantologie

OECD FORD obor

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Projekt

—

Návaznosti

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Rok uplatnění

2014

Kód důvěrnosti údajů

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

Název periodika

Polish Journal of Pathology

ISSN

1233-9687

e-ISSN

—

Svazek periodika

65

Číslo periodika v rámci svazku

2

Stát vydavatele periodika

PL - Polská republika

Počet stran výsledku

7

Strana od-do

93-99

Kód UT WoS článku

000339365300002

EID výsledku v databázi Scopus

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