TINJAUAN KEPUSTAKAAN

GAMBARAN IMAGING TUMOR WILMS

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PENDAHULUAN

tumor ganas ginjal paling sering pd anak

berasal dari sisa-sisa jaringan embrional

Nephroblastoma, Embrioma, Adenosarcoma, Adenomiosarcoma

pembedahan + radioterapi + chemoterapi survival rate mencapai 80-90 %.

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ANATOMI

ginjal kacang, retroperitoneal, V Th 12 -VL 3

dilindungi fascia renalis (membrane fibrous), capsul adipose dan capsul renalis trauma & infeksi

hilus ginjal : pembuluh darah, sistem limfatik, saraf & ureter

uk p : 10 12 cm, l: 5 7 cm & t:3 cm.

Berat ginjal laki 2 dws 150 gr & wanita 135 gr

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Pembentukan urine terjadi di nephron.

berisi lebih dari 1 juta nephron.

Nephron : glomerulus, tubulus renalis, dan Bowman's capsule.

Glomeruli : produksi urine dimulai.

Pembentukan urine di tub renalis, dialirkan dr cortex ke medulla )& kembali ke cortex.

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Fungsi Ginjal : meregulasi volume & konsentrasi cairan dlm tubuh melalui produksi urine.

Fungsi lainnya :

Detoksifikasi

absorbsi kalsium dgn produksi calcitrol

Menghasilkan erythropoietin

Sekresi renin

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Insidens

87 % dari tumor ginjal pada anak-anak

puncaknya pada umur 3-4 tahun

Tidak ada predileksi jenis kelamin

Unilateral 0,92 : 1, bilateral 0,60 : 1

Dx ditegakkan pd rata-rata usia 3,5 thn

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Gejala klinis

Umumnya asymptomatic abdominal mass, pd 80 % pdrt

Nyeri abdomen dan hematuria pd 25 % kasus.

Jarang : Infeksi traktus urinarius dan varicocele.

Hipertensi, gross hematuria dan demam pd 5-30 % penderita.

Perdarahan di dalam tumor hypotensi, anorexia, anemia dan demam.

Metastase paru gejala respiratorius (jarang)

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Pemeriksaan fisik

Pertama diperiksa : massa tumor dlm ukuran besar (kurang lebih 12 cm)

Kelainan dihub dgn syndrome WAGR dan Beckwith-Wiedemann sidrome ( aniridia, malformasi genitourinary & tanda 2 overgrowth)

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GAMBARAN RADIOLOGIS

Intravenous Urography

- Pelviocalyceal distorsi sampai displacement

Kalsifikasi :10 % kasus.

Nonfungsional kidney : 10-20 % kasus

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Intravenous urography pada menit k e 15

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USG Ginjal

diagnose awal dan follow up

Typical :

Massa solid well defined

dengan venous lakes

tumor trhombus vena cava inferior

aorta sering terdesak ,jarang terinfiltrasi

jarang lympheadenopathy

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Atypical

ruptur tumor

massa keluar dari kapsul ginjal

kalsifikasi

pembesaran lymphenode

encasement vascular

Doppler v. cava inferiorpendesakan /infiltrasi

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(A) Massa heterogen dengan ginjal terdesak ke superior(B) IVC dilatasi dan terisi tumor thrombus.

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(A) Massa tumor yg mendesak ginjal ke posterior(B) Post chemotherapy.

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A. Nodul di dekat collecting systemB. Doppler : hypovascular pd central nodule

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CT Scan

Massa solid slight hypodense dgn area necrotic/perdarahan /kistikheterogen enhancement

Fase corticomedulary

Distorsi parenkim, kadang pseudokapsul(+)

Infiltrasi vasculer & metastase hepar

Staging tumor & evaluasi kontralateral

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Penyebaran tumor

perluasan langsung melewati kapsul renalis organ sekitar

pembuluh darah melalui vena renalis atau vena cava inferior tumor thrombus

lymphe node perirenal, paracaval, paraortic, retroperitoneal atau retrocrural

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Screening

Faktor resiko tinggi

usia 6 bulan - 7 tahun

CT scan awal

USG serial tiap 3-6 bulan

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A. Massa ginjal kanan yg heterogen enhancement dan multiple kalsifikasi

B. Massa kistik ginjal kanan

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A. A claw sign B. Bilateral Wilms' tumours

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MRI

Sensitive patensi vena cava

vena cava inferior terinfiltrasi oleh tumor ?

Hipointese T1 hiperintense pada T2

Metastase lymphenode & tumor trombus

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A. Meluas ke vena renalisB. Meluas ke vena cava

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A. T1 : Nephroblastoma dgn tumour necrosis B. T2 : Nephroblastoma ginjal kiri

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Foto thorax

metastases ke paru.

lesi di paru-paru pd foto thorax diberikan terapi radiasi pada paru.

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PET/PET-CT

akurasi staging tumor

bedakan lesi benign dan malignant

respon awal pengobatan

18 F-FDG /PET-CT sensitif mendeteksi aktifitas metabolisme dari lesi

18 F-FDG /PET-CT dpt bedakan antara residual tumor dgn scar post operatif

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18 F-FDG-PET CT26

18 FDG PET-CT uptake pd prox femur & os ischium27

Metastase ke paru kanan

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II.8 TNM Klasifikasi Primary Tumor (T)

TX Primary tumor cannot be assessed

T0 No evidence of primary tumor

Ta Papillary noninvasive carcinoma (Figure 37.1)

Tis Carcinoma in situ

T1 Tumor invades subepithelial connective tissue (Figure 37.1)

T2 Tumor invades the muscularis (Figure 37.2)

T3 (For renal pelvis only) Tumor invades beyond muscularis into peripelvic

fat or the renal parenchyma (Figure 37.2)

T3 (For ureter only) Tumor invades beyond muscularis into periureteric fat

T4 Tumor invades adjacent organs, or through the kidney into the perinephric fat (Figures 37.3AC)

Regional Lymph Nodes (N)

NX Regional lymph nodes cannot be assessed

N0 No regional lymph node metastasis

N1 Metastasis in a single lymph node, 2cm or less in greatest dimension (Figure 37.4)

N2 Metastasis in a single lymph node, more than 2cm but not more than 5 cm in greatest dimension; or multiple lymph nodes, none more than 5 cm in greatest dimension (Figures 37.5A, B)

N3 Metastasis in a lymph node, more than 5 cm in greatest dimension (Figures 37.6A, B)

Distant Metastasis (M)

MX Distant metastasis cannot be assessed

M0 No distant metastasis

M1 Distant metastasis

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StagingThe current Children's Oncology Group (COG) staging for Wilms tumor is as follows:

Stage I: Tumor is limited to kidney and is completely resected. The renal capsule is intact. The tumor was not ruptured or biopsied prior to removal. The vessels of the renal sinus are not involved. No evidence of tumor is present at or beyond margins of resection.

Stage II: The tumor is completely resected, and no evidence of tumor at or beyond the margins of resection is noted. The tumor extends beyond the kidney (penetration of renal capsule, involvement of renal sinus).

Stage III: A residual nonhematogenous tumor is present following surgery and is confined to the abdomen. Positive lymph nodes in abdomen or pelvis are noted. Penetration through peritoneal surface is observed. Peritoneal implants are present. Gross or microscopic tumor remains postoperatively, including positive margins of resection. Tumor spillage is noted. The tumor is treated with preoperative chemotherapy. The rumor is removed in more than one piece.

Stage IV: Hematogenous metastases (eg, lung, liver, bone, brain) or lymph node metastases beyond abdomen or pelvis is noted.

Stage V: Bilateral renal involvement by tumor is present at diagnosis.

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DIAGNOSA BANDING

1. Neuroblastoma

a) IVP : ginjal terdesak ke inferior dan dropping lilypelviocalyceal

b) USG : massa extrarenal hyperechoic inhomogeneous ygmendesak ginjal

c) CT scan : massa solid dengan kalsifikasi , encasement vascular, dan heterogen enhancement

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A.lebih inhomogenB. terdesak ke posterior

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kalsifikasi , encasement vascular, & heterogen enhancement

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PERBEDAAN WILMS TUMOR & NEUROBLASTOMA

Karakteristik Wilms tumor Neuroblastoma

Usia

Origin (asal organ)

Lateral/bilateral

Kalsifikasi

Efek massa ke ginjal

Perluasan vascular

2-3 tahun

Ginjal

10 % bilateral

< 15 %

Efek massa dr dalam ginjal

Infiltrasi ke vena renalis

pada 5-10 % kasus

< 2 tahun

Syaraf di retroperitoneal

Hampir selalu unilateral

85 95 %

Pendesakan dari luar ginjal

Sering encasement

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2. Polykistik Kidney DiseaseBilateral, kista multiple dgn ukuran bervariasi

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3. RhabdomyosarcomaCT : massa solid dg nekrosis & kalsifikasi serta heterogeneous contrast enhancement

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KESIMPULAN

Klinis : asymptomatic abdominal mass, nyeri abdomen dan hematuria.

Penggunaan multiple modalitas screening, penegakkan dx, akurasi staging & follow up post terapi

DD : neuroblastoma, polycystic kidney & rhabdomyosarcoma

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GRATIASTERIMAKASIH

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