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Case study Renal block. Dr Willie Conradie May 2012 Diagnostic Radiology. 4yr old boy from L esotho. Weight loss Normal “Mile-stones” No chronic disease Resp : No coughing. No TB history GIT: No nausea/vomiting/ diarhoea . Uro : Normal frequency and colour - PowerPoint PPT Presentation
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Case study
Renal block
Dr Willie ConradieMay 2012
Diagnostic Radiology
4yr old boy from Lesotho
O Weight lossO Normal “Mile-stones”O No chronic disease
O Resp: No coughing. No TB historyO GIT: No nausea/vomiting/diarhoea.O Uro: Normal frequency and colour
O Exam: Right sided abdominal mass..
Nephroblastomavs
Neuroblastoma?
Nephroblastoma(Wilms’ tumor)
O IncidenceO Most common malignant abdominal
neoplasm in children (1-8 years)
O 3rd most common malignancy in children
O LeukemiaO CNS tumors
O 3rd most common renal mass in children
O HydronephrosisO Multi-cystic dysplastic kidney
O 7% of all childhood Ca
Wilms tumor
O Familial in 1-2%O M=FO More common in blacks
O Peak age at 3-4 yearsO 90% before 8 yearsO Rare in neonates and adults
O Undifferentiated metanephric blastema (persistant primitive renal parenchyma)
O “nephrogenic rests”O Multiple foci = Nephroblastomatosis
O Genetic:O Abnormal WT1 (11p13) - WAGR/DRASHO Abnormal WT2 (11p15) - Beckwith-Wiedemann
- Hemihypertrophy
Rule of 10’s:
• 10% unfavourable histology• 10% bilateral• 10% vascular invasion• 10% calcifications• 10% pulmonary metastases at
presentation• 10% metastasize to liver
O CTO Poorly enhancing massO Well-defined margins or pseudocapsuleO Local extension into perirenal fat and local lymph
nodes
Wilms tumor
Best diagnostic clue:• Large heterogeneous mass• Replacing/in kidney• Displacement of organs/vessels• Extending into renal vein and IVC• Calcifications less often seen
Wilms tumor
O MR FindingsO TlWl: Typically low signal intensity on TlO T2Wl: High signal on T2
O heterogeneous andO frequently containing blood products
O MRA: useful in determining vascular spread pre-operatively
O Nuclear medicineO Bone Scan:
O Metastatic disease to bone occurs very late;O Not routine
O PETO Increasing use in Wilms and all pediatric tumorsO Primarily has an adjunctive, problem solving role
O Differentiating scar tissue from residual active tumor
NeuroblastomaO Incidence
O 3rd most common malignant tumor in infancy O LeukemiaO CNS
O Most common tumor in first week of life
O Most common solid, extra-cranial malignant tumor in children
O 8-10% of all childhood Ca
O 15 % of cancer deaths in children
Neuroblastoma
O Mean age at diagnosis = 22 monthsO 36% are infantsO 89% < 5yearsO 98% <10 years
O M:V = 1.1:1
O Malignant tumor of primitive neural crest cellsO Location
O Adrenal glands/para-vertebral sympathetic ganglia (70%)
O Posterior mediastinum (20%)O Pelvis (2-5%)O Neck (1-5%)
O MRIO High on T2WI; Low on T1WI O Heterogeneous - related to calcification/hemorrhage/necrosisO Excellent for detecting extension of tumor into spinal canal.
Neuroblastoma
Best diagnostic clue:• Invasive heterogeneous suprarenal mass • Calcifications (85%)• Crosses midline• Rather surrounds and engulfs vasculature • Tendency to invade spinal canal (via
neuroforamina)• Metastasizes commonly to bone, liver and LN
Neuroblastoma
O Nuclear medicineO Bone Scan
O Technetium-99m MDPO Uptake seen in bony metastasis (both cortical and
marrow)O Calcified primary mass often also demonstrated
uptake (up to74% of cases)
O MIBGO Metaiodobenzylguanidine
O Avid uptake related to catecholamine productionO Excellent for following extent of disease in MIBG
avid tumorsO 30% of neuroblastoma are not MIBG avid
STAGE
• I- Limited to organ of origin• II- Regional spread not crossing midline• III- Extension across midline• IV- Metasteses• IVs- I or II with disease limited to liver, skin, BM
WITHOUT radiographic evidence of bone metasteses.
Neuroblastoma
References1. Siegel MJ, Chung EM. Wilms’ tumor and other
pediatric renal masses. Magn Reson Imaging Clin N Am 16 (2008):479-497.
2. Siegel MJ, AlokJaju. MR Imaging of Neuroblastic masses. Magn Reson Imaging Clin N Am 16 (2008):499-513.
3. Donnely et al. Diagnostic Imaging: Pediatrics. First edition. 5:50-53; 5:78-81.
4. Dahnert W. Radiology Review Manual. Sixth edition: 940-942; 992-993.