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Case study Renal block Dr Willie Conradie May 2012 Diagnostic Radiology
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4yr old boy from Lesotho O Weight loss O Normal “Mile-stones” O No chronic disease O Resp:No coughing. No TB history O GIT: No nausea/vomiting/diarhoea. O Uro: Normal frequency and colour O Exam:Right sided abdominal mass..
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Nephroblastoma vs Neuroblastoma?
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Nephroblastoma (Wilms’ tumor) O Incidence O Most common malignant abdominal neoplasm in children (1-8 years) O 3 rd most common malignancy in children O Leukemia O CNS tumors O 3 rd most common renal mass in children O Hydronephrosis O Multi-cystic dysplastic kidney O 7% of all childhood Ca
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Wilms tumor O Familial in 1-2% O M=F O More common in blacks O Peak age at 3-4 years O 90% before 8 years O 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/DRASH O 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
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O CT O Poorly enhancing mass O Well-defined margins or pseudocapsule O 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
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Wilms tumor O MR Findings O TlWl: Typically low signal intensity on Tl O T2Wl: High signal on T2 O heterogeneous and O frequently containing blood products O MRA: useful in determining vascular spread pre-operatively O Nuclear medicine O Bone Scan: O Metastatic disease to bone occurs very late; O Not routine O PET O Increasing use in Wilms and all pediatric tumors O Primarily has an adjunctive, problem solving role O Differentiating scar tissue from residual active tumor
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Neuroblastoma O Incidence O 3 rd most common malignant tumor in infancy O Leukemia O 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
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Neuroblastoma O Mean age at diagnosis = 22 months O 36% are infants O 89% < 5years O 98% <10 years O M:V = 1.1:1 O Malignant tumor of primitive neural crest cells O Location O Adrenal glands/para-vertebral sympathetic ganglia (70%) O Posterior mediastinum (20%) O Pelvis (2-5%) O Neck (1-5%)
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O MRI O High on T2WI;Low on T1WI O Heterogeneous - related to calcification/hemorrhage/necrosis O 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
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Neuroblastoma O Nuclear medicine O Bone Scan O Technetium-99m MDP O Uptake seen in bony metastasis (both cortical and marrow) O Calcified primary mass often also demonstrated uptake (up to74% of cases) O MIBG O Metaiodobenzylguanidine O Avid uptake related to catecholamine production O Excellent for following extent of disease in MIBG avid tumors O 30% of neuroblastoma are not MIBG avid
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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
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References 1. 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.
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