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Case study Renal block

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

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Case study Renal block

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  1. Case studyRenal block Dr Willie Conradie May 2012 Diagnostic Radiology

  2. 4yr old boy from Lesotho • Weight loss • Normal “Mile-stones” • No chronic disease • Resp: No coughing. No TB history • GIT: No nausea/vomiting/diarhoea. • Uro: Normal frequency and colour • Exam: Right sided abdominal mass..

  3. NephroblastomavsNeuroblastoma?

  4. Nephroblastoma(Wilms’ tumor) • Incidence • Most common malignant abdominal neoplasm in children (1-8 years) • 3rd most common malignancy in children • Leukemia • CNS tumors • 3rd most common renal mass in children • Hydronephrosis • Multi-cystic dysplastic kidney • 7% of all childhood Ca

  5. Wilmstumor • Rule of 10’s: • 10% unfavourable histology • 10% bilateral • 10% vascular invasion • 10% calcifications • 10% pulmonary metastases at presentation • 10% metastasize to liver • Familial in 1-2% • M=F • More common in blacks • Peak age at 3-4 years • 90% before 8 years • Rare in neonates and adults • Undifferentiated metanephricblastema(persistant primitive renal parenchyma) • “nephrogenic rests” • Multiple foci = Nephroblastomatosis • Genetic: • Abnormal WT1 (11p13) - WAGR/DRASH • Abnormal WT2 (11p15) - Beckwith-Wiedemann - Hemihypertrophy

  6. Wilmstumor • Best diagnostic clue: • Large heterogeneousmass • Replacing/in kidney • Displacement of organs/vessels • Extending into renal vein and IVC • Calcifications less often seen • CT • Poorly enhancing mass • Well-defined margins or pseudocapsule • Local extension into perirenal fat and local lymph nodes

  7. Wilmstumor • MR Findings • TlWl: Typically low signal intensity on Tl • T2Wl: High signal on T2 • heterogeneous and • frequently containing blood products • MRA: useful in determining vascular spread pre-operatively • Nuclear medicine • Bone Scan: • Metastatic disease to bone occurs very late; • Not routine • PET • Increasing use in Wilms and all pediatrictumors • Primarily has an adjunctive, problem solving role • Differentiating scar tissue from residual active tumor

  8. Neuroblastoma • Incidence • 3rd most common malignant tumorin infancy • Leukemia • CNS • Most common tumor in first week of life • Most common solid, extra-cranial malignant tumor in children • 8-10% of all childhood Ca • 15 % of cancer deaths in children

  9. Neuroblastoma • Mean age at diagnosis = 22 months • 36% are infants • 89% < 5years • 98% <10 years • M:V = 1.1:1 • Malignant tumor of primitive neural crest cells • Location • Adrenal glands/para-vertebral sympathetic ganglia (70%) • Posterior mediastinum (20%) • Pelvis (2-5%) • Neck (1-5%)

  10. 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 • MRI • High on T2WI; Low on T1WI • Heterogeneous - related to calcification/hemorrhage/necrosis • Excellent for detecting extension of tumor into spinal canal.

  11. Neuroblastoma • Nuclear medicine • Bone Scan • Technetium-99m MDP • Uptake seen in bony metastasis (both cortical and marrow) • Calcified primary mass often also demonstrated uptake (up to74% of cases) • MIBG • Metaiodobenzylguanidine • Avid uptake related to catecholamine production • Excellent for following extent of disease in MIBG avid tumors • 30% of neuroblastoma are not MIBG avid

  12. Neuroblastoma • 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.

  13. References • Siegel MJ, Chung EM. Wilms’ tumor and other pediatric renal masses. MagnReson Imaging Clin N Am 16 (2008):479-497. • Siegel MJ, AlokJaju. MR Imaging of Neuroblastic masses.MagnReson Imaging Clin N Am 16 (2008):499-513. • Donnely et al. Diagnostic Imaging: Pediatrics. First edition. 5:50-53; 5:78-81. • Dahnert W. Radiology Review Manual. Sixth edition: 940-942; 992-993.

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