10º Fichamento -  Câncer Renal  ( Heinnnn Fim )
Autor: Lorena Moura | Publicado: 2011-06-03T07:49:00-07:00

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CANCER RENALComentário de artigo publicado no Journal of UrologyLinehan WM, Walther MM, Zbar B. The genetic basis of cancer of the kidney. J Urol 2003;170:2163-72.
Comentarista: Stênio de Cássio Zequi
“O cancer de rim era considerado uma doença única. No entanto, são identificados distintos tipos de tumores de rim, causados por genes distintos. Cerca de 75% são carcinomas de células claras, 15% são papilíferos, 5% são cromofóbicos e 5% são oncocitomas.Há formas esporádicas e hereditárias de tumores renais. Cada tipo de síndrome hereditária é associada a um tipo histológico: 1) von Hippel-Lindau (VHL) é a forma hereditária do carcinoma de células claras; 2) carcinoma renal papilífero hereditário (HPRC) é a forma hereditária do carcinoma renal papilífero tipo 1; 3) a síndrome Birt Hogg Dubé (BHD) está associada ao carcinoma renal cromofóbico e ao oncocitoma; 4) a leiomiomatose renal (HLRCC) é a forma hereditária do carcinoma renal papilífero tipo 2.”
“Câncer renal papilífero: Câncer renal papilífero tipo 1 hereditário (HPRC)O HPRC é uma forma hereditária de carcinoma renal no qual indivíduos estão sob risco de tumores papilíferos tipo 1 bilaterais e multifocais. Os tumores renais nas famílias com HPRC aparecem entre a quarta e sexta décadas de vida. “‘Para identificar o gene responsável pelo HPRC foi realizada análise de ligação. O gene encontrado está localizado no cromossomo 7q31.1-34. Mutações missense foram encontradas no domínio da tirosina quinase do gene MET em famílias com HPRC. Esta tirosina quinase é um alto potencial para pequenas moléculas que bloqueiam a atividade da tirosina quinase do MET. “Leiomioma Cutâneo Múltiplo (MCL)/HLRCC“O HLRCC e o MCL representam uma doença complexa caracterizada pelo aparecimento de múltiplos leiomiomas cutâneos, leiomiomas&nbsp; e leiomiossarcomas uterinos e tumores de rim. Os tumores renais em pacientes com HLRCC são distintos dos tumores em pacientes com VHL, HPRC ou BHD. Os tumores renais no HLRCC são geralmente solitários papilíferos tipo 2. São tumores agressivos que apresentam metátases precocemente.”FONTE: ://www.gbeth.org.br/index.php?option=com_content&amp;view=article&amp;id=228&amp;catid=19&amp;Itemid10