wilm’s tumour
DESCRIPTION
tumor wilmsTRANSCRIPT
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Wilm’s Tumor Oleh:
Dr.Nurbaiti, Sp. PA, M.Kes
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Pendahuluan Nephroblastoma, embryoma,
carcinosarcoma, adenosarcoma, ademyosarcoma.
>> anak-anak: 50% sebelum 3 th, 90% sebelum 6 th.
<< dewasa Dd/ mesoblastic nephroma
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Lanjutan ♂ = ♀ Oriental > kaukasoid > negroid Kanan=kiri Ekstrarenal: retroperitoneum,
saccrococcygeal region, testis, uterus, inguinal canal, mediastinum.
DD/ teratoma dengan predominan nephroblastic komponen
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Lanjutan Prekursor : Wilm’s-aniridia genital
abnormaly retardation syndrom (WAGR), omphalocele macroglossia, hemihypertrophy, Denys-Drash Synd.
Diduga prekursor: renal-genital malformations, cutaneus nevi, angioma, trisomy 18, Klippel-Trenaunay synd, neurofibromatosis, Bloom synd, cerebral gigantism
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Lanjutan Asosiasi: osteosarcoma, bothroid
rhabdomyosarcoma, retinoblastoma,hepatocellular carcinoma.
Genetik: kromosom 11p13 (WT1 gen) dan kromosom 11p15.5 (WT2)
Ab N: krom 1,8,12,16. mutasi p53 (5%)
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Klinis Massa di abdominal . Hematuria <<, nyeri<<, hypertensi<<. Protein uria ±
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Penunjang Pyelogram IV: massa intra renal yang
menghalangi pelvis. Kimia klinik (↑): hyaluronic acid,
acquired von Willebrand’s factor, inactive renin, erythropoietin serum
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Makroskopik >> lesi soliter,lobuler, batas tegas,
padat, lunak, kistik, nekrosis, hemorrhage, abu-abu.
7% multisentrik ∑ 550gr.
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Mikroskopik 3 komponen utama:1. Undiffetentiated blastema2. Mesenchymal (stroma) tissue3. Epitelial Salah satu komponen bisa lebih
dominan
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Epitelial, Blastema, Mesenkim
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Komponen Epitelial
Sel kecil-kecil, membentuk struk asinar, rosette like
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Epitelium
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Komponen Blastema
sangat selular, sel kecil bulat-oval, sitoplasma sedikit, << onkositoid, yang tumbuh difus, nodular, cord like, basaloid.
DD/ neuroblastoma
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Blastema
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Komponen Mesenchymal
Sel-sel spindel mirip fibroblast dan mirip otot.
Komponen epitelial: struktur menyerupai tubular, glomerular, rosette.
DD/ untuk dominan epitelial: multycystic nephroma dan renal cell carcinoma
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Mesenkim
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Sel anaplastik
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Lanjutan subtype: papillonodular Gross: penonjolan dari septa-septa
kedalam lumen
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Lanjutan Dapat ditemui adanya: sel musin, sel
bersilia, sel squamous, epitel transisional, sel endokrin, sel yg memproduksi renin, neuroepitelium, neuroblast, sel ganglion, neuroglia, sel lemak, kartilago, tulang kompak, sel hematopoietik, RCC
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IHC Blastema komponen : vimentin Epitelial komponen: keratin, EMA,
lectin. Mesenchymal komponen: myoglobin,
desmin, enolase, GFAP, S-100 protein.
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Sitologi Warning Dapat ditemui 3 unsur tumor
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Penyebaran Lokal: perirenal soft tissue, kel. Adrenal,
kolon, hati, vertebra, regio paraspinal kompresi spinal, vena renalis, pelvis renalis<<, ureter<<
KGB regional (15%) Metastase: paru, hati, peritoneum
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Terapi Tergantung stadium Operatif, kemoterapi, radioterapi
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Prognosis Sembuh pada 80-90% kasus Rekuren karena genetik dan terapi tidak adekwat. Parameter prognosis:1. Usia: semakin muda semakin baik2. Stadium 3. Ukuran 4. Anaplasia 5. Tubular differentiation ekstension6. Skeletal muscle differentiation7. Mucin production: buruk8. DNA ploidy
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Tumor Lain Pada Anak-anak
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Mesoblastic Nephroma / fetal / mesenchymal/ leiomyomatous hamartoma
>> (-) 6 bulan Gross: solid, batas tegas,kuning
keabuan, struktur seperti konde, hemoragik, nekrosis, kistik
Dekat hilus Mikroskopik: sel-sel spindel
hiperplastis, inti jelas, sitoplasma fibrilari asidofilik.
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Sel spindel
A
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Mitosis
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Sel plum
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Lanjutan IHC: vimentin, fibronectin, actin Genetik: trisomi 11
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Lanjutan Subtype: Cellular/ atypical mesoblastic
nephroma, mik: sangat selular, mitosis banyak, nekrosis, perdarahan.
Cenderung rekuren dan metastase
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Multicystic Nephroma/ multilocular cystic nephroma, multilocular cyst Jarang Gross: soliter, unilateral, batas tegas,
kistik, cairan serous Mikroskopik : kista dilapisi epitel
kolumnar-gepeng. Dd/ Lymphangioma
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KISTIK,LPF
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Epitel Gepeng
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Nephroblastomatosis dan Nephrogenic rest Jarang Mikroskopik mirip wilm’s tumor Kel konggenital : ditemukan
nephrogenic rest. mikron nephrogenic rest, masive
Nephroblastomatosis Mutasi gen WT1
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Intrarenal Neuroblastoma Primer atau sekunder lesi Dari adrenal atau jaringan sekitar
retroperitoneal Primer DD/ Wilm’s tumour Metastase : orbit, tulang, adrenal
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Clear Cell Sarcoma Bone metastasizing renal tumor. 4%% keganasan pada ginjal anak-anak Puncak pada tahun ke 2 kehidupan Dewasa <<, asosiasi dengan familial
colonic poliposis Gross : batas tegas, di medulla>>,
permukaan berwarna homogen abu-abu kecoklatan dan daerah-daerah mixoid.
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Lanjutan Mikroskopik: pola pertumbuhan difus,
sel kecil, inti bulat, anak inti sulit dilihat, sitoplasma jernih atau bervakuol, batas sel tidak jelas. Nuclear groove, mitosis jarang, stroma fibrovaskular, miksoid, kistik, fibrosis, hyalinisasi.
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LPF
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HPF
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Lanjutan Insuline like growth tumor Sangat agresiv, metastase ke skeletal
t.u skull, KGB regional, otak, paru, hati Angka mortalitas 70% kasus
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Rhabdoid Tumor Batita, median age 18 bulan Asosiasi dengan embryonal tumor pada
daerah fossa midline posterior, t.u medulloblastoma.
Gross: solid, lunak, batas tegas. Mikroskopik : tumbuh trabekular,
alveolar, diffuse, pada daerah medula Sel tumor bulat-oval, kadang spindel,
sitoplasma banyak eosinofilik.
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Massa Mengkilat
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Sitoplasma Eosinofilik
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Sel bulat, oval
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Lanjutan Sangat agresive Angka mortalitas 75% kasus
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Lain-lain Ossifiying renal tumour of infancy Embryonal sarcoma Nephrogenic adenofibroma Intrarenal teratoma
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TERIMA KASIH