tumora wilms
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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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