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TUMORA WILMS

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TUMORA WILMS1899 Wilms

sinonime: nefroblastom, embriom, adenomiosarcom embrionar

30% dintre tumorile maligne ale copilului

incidena maxim la 2 5 anirar peste 15 ani relativ frecvent la n.n. i sugar

bilateral: 5 10% din cazuri

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ETIOPATOGENIEtumor embrionar -blastemul metanefrogendeleia braului scurt cromozom 11, b. 13malformaii asociate: - aniridie

- sindromul omfalocel - macroglosie- hamartoame, hemangioame, nevi pigmentari- alte malformaii congenitale urinare

- predispoziie congenital 30%

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ANATOMIE PATOLOGIC sferic ovoid , neted boselat

fragil, cu pseudocapsul vascularizat

mare (d=10,15,20cm), 500-1500g

alb glbuie, aspect cerebriform,zone de necroz sau hemoragice

invazie de vecintate rapid (d.d., colon, splin, ficat)

adenopatie loco- regional

invazie venoas prin trombi neoplazici

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A.P. HISTOLOGIE

celule epiteliale de tip blastomatos cu grade diferite dedifereniere

strom mixomatoas (fibroblati, f. musculare netede istriate, esut nervos etc.)

Clasificare histologic White i Bodian: 1. reniform, 2. tubular, 3. mezenchimal, 4. papilar,5. local blastomatos, 6. mixt, 7. masiv blastomatos.

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ASPECTE CLINICETriada casic: - tumor 50%

- durere 25%- hematurie 15%

Tulburri digestive: diaree, vrsturi, grea. abdomen acut - inflamator

- traumaticSemne urinare: hematurie capricioas

infecie urinar varicocel

Hipertensiune arterial

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EXAMENUL CLINIC

NU M ATINGE

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CLASIFiCAREA STADIAL

st. I tu. limitat la rinichi, capsul intact, adenopatii( -),metastaze(-)

st.II: tu. extins regional, capsul spart, rezecabil total, adenopatii(+), metastaze(-)

st.III: tu. st.II necrozat, rupt, biopsiat

st. IV: tumor cu metstaze hematogene

st. V : tumor bilateral , simultan sau succesiv

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IMAGISTICA-ECOGRAFIA

Neinvaziv Orienteaz rapid diagnosticulEvideniaz: - extinderea tu.- metastazele- rinichiul contra-

lateral- +/- calcificri

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IMAGISTICA-ECOGRAFIA

Evideniaz:

-prezena -extinderea

trombozei neoplazice

La nivelul v. renale, V.C.I. sau cord

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IMAGISTICA-UROGRAFIA

regina investigaiilor urologice arbore caliceal dezorientatstarea rinichiului opusnormal tumor pediculat

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IMAGISTICA-C.T./ R.M.N.

extindere local

stadializareadenopatiimetastaze

urmrire dup chemoterapie

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I M A G I S T I C A

ARTERIOGRAFIA- nu se efectueaz de rutin (servicii calificate)

VENOGRAFIA- extinderea intravenoas

a tumorii

RADIOGRAFII OSOASE

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INVESTIGAII DE

LABORATOR grup sanguin, hemogram, V.S.H.

dozri de markeri biologici: eritropoietina, alfa fetoproteina,fosfataza alcalin, V.M.A. i H.V.A.

Sumar de urin: hematurie microscopic (+) catecolamine (-)

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DIAGNOSTIC DIFERENIAL AFECIUNI BENIGNE

Hidronefroz Rinichi polichistic

R. n potcoav HamartommezenchimalHematomperirenalTeratomretroperitoneal

Chist hidatichepatic

Splenomegalie

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DIAGNOSTIC DIFERENIAL AFECIUNI MALIGNE

neuroblastomul

feocromocitomul

hepatoblastomul

limfosarcomul

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TRATAMENT

chirurgical : - rezecia tumorii - stadializare

- histologie- tu. Wilms bilateral!!!

radioterapie : - pre / postoperator(protocol)

chimioterapie : - cur primar/secundarecu: actinomicina D, vincristin doxorubicin, cytoxan

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