Tumor de wilm's
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NefroblastomaTumor de WilmsDr. Luis Humberto Cruz ContrerasAnatomía patológicaHospital GeneralMorelia, Mich
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Carl Max Wilhem Wilms (1867-1918)
Médico cirujano y patólogo ALEMAN• Universidad de Bonn• Muere tras salvar la vida a un soldado con edema de laringe por
Difteria
Estudio extensamente los tumores renales• Propuso que las células tumorales derivan del embrión• Monografía “Die Mischgeschwülste der Niere”, los tumores
renales mixtos• Epónimo Tumor de Wilms
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NefroblastomaTumor de Wilms
500 casos anualmente en USA
80% de tumores renales en niños
Edad de presentación : 2 a 4 años
Bilaterales 5%
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Tumor de Wilms PATOGÉNESIS
RESTOS NEFROGÉNICOS
• Peri-lobares (Debajo de la cápsula)• Intra-lobares• NEFROBLASTOMATOSIS: restos nefrogénicos multifocales y/o difusos (también tumor de Wilms multifocal, y
bilateral)• <1% en niños sanos, hasta 41% en riñones con Wilms
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Restos nefrogénicos perilobares
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Restos nefrogénicos y Tumor de Wilms
1. Blastema 2. Epitelio
3. Mesenquima
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Restos nefrogénicos intralobares
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Restos nefrogénicos perilobares (epitelio)
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Restos nefrogénicos perilobares (epitelio)
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Patogénesis molecular
Esporádicos (90%), Asociados a síndromes (5%)
• Sx Denys-Drash• Sx WAGR, (Wilms tumor, Aniridia, Genitourinary abnormalities, Retardation)• Beckwith-Wiedeman (Bety, BMW)
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Tumor de WilmsPatogénesis molecular
WT1 (cromosoma 11p13)
• Wilms tumor gene 1• Factor de transcripción de ADN, regula factores de crecimiento (IGF1) (PDGF)• Supresor tumoral
WT2 (11p15.5)
• LOH
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Tumor de WilmsMacroscopía
Tumor más grande que el riñón donde se origina
• > 5 cm
Solido, Suave, blanco-gris
• Se parece al tejido cerebral
Hemorragia, Necrosis y quistes
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Tumor de Wilms
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Tumor de Wilms
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Tumor de Wilms TRIFÁSICOUn clásico….
1. Blastema: cél. Pequeñas, sin
citoplasma, con mitosis, núcleo hipercromático
2. Epitelio, tubos, quistes con epitelio cilíndrico o cúbico
3. Mesenquima, estroma mixoide o fusocelular. Pueden diferenciarse a otros tejidos blandos
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Tumor de Wilms TRIFÁSICOUn clásico….
1. Blastema 2. Epitelio
3. Mesenquima
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Tumor de Wilms TRIFÁSICOotro con todo….
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T. Wilms con predominio del blastema (y algo de mesénquima)
Patrones del blastema:•Serpinginoso•Nodular•Difuso
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T. Wilms con predominio del blastema (y algo de mesénquima)
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T. Wilms solo epitelial…
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T. De Wilms con todo y algo más…
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WT1 ….. IHQNuclear
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Como se clasifican, dividenLo importante…
SIN ANAPLASIA (favorable)
CON ANAPLASIA (NO favorable)•6% de los casos
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Anaplasia en el tumor de WilmsANAPLASIA : núcleo grande hipercromático, 3 veces el tamaño
nuclear del blastema
• Mitosis atípicas multipolares
Factor predictivo de mal pronóstico
• MALA RESPUESTA AL TX• Muestreo EXTENSO• ADECUADO : 1 CAPSULA / CM
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T. Wilms – TratamientoComo las frutas se madura
El epitelio presenta aspecto tubular maduro.. Y el mesénquima ahora es tejido adiposo y fibroso maduro
SIN EMBARGO, aún hay anaplasia…
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T. Wilms – con cambios Post-Tx
El epitelio presenta configuración glomerular
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TratamientoTumor de Wilms
Quimioterapia
Radioterapia
Cirugía
Supervivencia a 10 años 90% Con histología favorable