imaging tumor wilms
DESCRIPTION
IMAGING TUMOR WILMSTRANSCRIPT
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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
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18 FDG PET-CT uptake pd prox femur & os ischium27
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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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