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

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About: Review of renal tumors associated with Birt-Hogg-Dube syndrome with focus on clinical and pathobiological aspects Goto Sponge NotDistinct Permalink

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Attributes	Values
rdf:type	skos:Concept http://linked.opendata.cz/ontology/domain/vavai/Vysledek
rdfs:seeAlso	http://dx.doi.org/10.5114/PJP.2014.43958
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 in an aggressive fashion. However, renal tumors including hybrid oncocytic tumor, chromophobe RCC and oncocytoma behave mostly in an indolent fashion. 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 in an aggressive fashion. However, renal tumors including hybrid oncocytic tumor, chromophobe RCC and oncocytoma behave mostly in an indolent fashion. (en)
Title	Review of renal tumors associated with Birt-Hogg-Dube syndrome with focus on clinical and pathobiological aspects Review of renal tumors associated with Birt-Hogg-Dube syndrome with focus on clinical and pathobiological aspects (en)
skos:prefLabel	Review of renal tumors associated with Birt-Hogg-Dube syndrome with focus on clinical and pathobiological aspects Review of renal tumors associated with Birt-Hogg-Dube syndrome with focus on clinical and pathobiological aspects (en)
skos:notation	RIV/00216208:11140/14:10271936!RIV15-MSM-11140___
http://linked.open...avai/riv/aktivita	I
http://linked.open...avai/riv/aktivity	I
http://linked.open...iv/cisloPeriodika	2
http://linked.open...vai/riv/dodaniDat	2015
http://linked.open...aciTvurceVysledku	Hora, Milan Michal, Michal Hes, Ondřej
http://linked.open.../riv/druhVysledku	J - Článek v odborném periodiku
http://linked.open...iv/duvernostUdaju	S - Úplné a pravdivé údaje nepodléhající ochraně podle zvláštních právních předpisů
http://linked.open...titaPredkladatele	Univerzita Karlova v Praze / Lékařská fakulta v Plzni
http://linked.open...dnocenehoVysledku	42615
http://linked.open...ai/riv/idVysledku	RIV/00216208:11140/14:10271936
http://linked.open...riv/jazykVysledku	eng - angličtina
http://linked.open.../riv/klicovaSlova	Birt-Hogg-Dube syndrome; renal tumor; review (en)
http://linked.open.../riv/klicoveSlovo	renal tumor review Birt-Hogg-Dube syndrome
http://linked.open...odStatuVydavatele	PL - Polská republika
http://linked.open...ontrolniKodProRIV	[AFC09835984C]
http://linked.open...i/riv/nazevZdroje	Polish Journal of Pathology
http://linked.open...in/vavai/riv/obor	FJ
http://linked.open...ichTvurcuVysledku	3 (xsd:int)
http://linked.open...cetTvurcuVysledku	11 (xsd:int)
http://linked.open...UplatneniVysledku	2014
http://linked.open...v/svazekPeriodika	65
http://linked.open...iv/tvurceVysledku	Hes, Ondřej Hora, Milan Kuroda, Naoto Michal, Michal Nagashima, Yoji Furuya, Mitsuko Gotohda, Hiroko Nakatani, Yukio Kawakami, Fumi Moritani, Suzuko Ota, Satoshi
http://linked.open...ain/vavai/riv/wos	000339365300002
issn	1233-9687
number of pages	7 (xsd:int)
http://bibframe.org/vocab/doi	10.5114/PJP.2014.43958
http://localhost/t...ganizacniJednotka	11140

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