Jesse Courtier, MD Assistant Clinical Professor of Radiology UCSF Benioff Children’s Hopsital.

Transcript of Jesse Courtier, MD Assistant Clinical Professor of Radiology UCSF Benioff Children’s Hopsital.

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Jesse Courtier, MD Assistant Clinical Professor of Radiology UCSF Benioff Childrens Hopsital
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I. Introduction: Diagnostic Radiology role in Radiation Oncology II. Common Pediatric Renal Lesions III. Lesions Most Important to Rad Onc IV. Imaging Staging of Wilms Tumor
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Diagnostic Radiology & Radiation Oncology formerly integrated in the 1950s Currently separated, both still cert by ABR Little specific training in Dx Radiology on Rad Therapy outside of I-131 Introduction Dx Radiology Role In Rad Oncology
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I. Accurate Imaging Based Staging
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II. Aid with tumor Target/ normal tissue I. Accurate Imaging Based Staging
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II. Aid with tumor Target/ normal tissue
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I. Accurate Imaging Based Staging
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II. Aid with tumor Target/ normal tissue I. Accurate Imaging Based Staging
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II. Aid with tumor Target/ normal tissue I. Accurate Imaging Based Staging III. Interpretation of Post Tx Imaging
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Collaboration btwn Dx Rad and Rad Onc critical in complex contouring cases Help with delineation of the gross tumor volume, esp when abutting dose limiting normal structures Dx Radiology Role In Rad Oncology Introduction
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Pediatric Renal Tumors by Age 0-2 years
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GU Neoplasms
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2% of childhood renal neoplasms Arises from renal medulla Mean age 16 months (usually < 3yrs) Synchronous CNS lesions (10%) Metastases Primary neuro- ectodermal tumor, typically posterior fossa
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Coronal Neonatal Head US image showing multiple echogenic foci in the basal ganglia
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Axial T1 Post Contrast MR image of the abomen @ level of the kidneys: Large right renal mass (yellow arrows) and several smaller masses in the left kidney (white arrows
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Coronal T2 fat sat MR image of the chest/abd: Large mass infiltrates the right kidney (yellow arrows). Note normal size of left kidney (white arrow)
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Coronal post contrast T1 image of the brain showing multiple enhancing lesions (yellow arrows)
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More posterior Coronal T2 fat sat MR image of the chest in the soft tissues of the back shows multiple T2 bright nodules in the skin (yellow arrows)
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Large mass (
Most common renal neoplasm in pts 6mos Benign tumor US: heterog echogen CT: usu heterog low atten mass
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GU Neoplasms
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Nephroblastomatosis "the presence of nephrogenic rests or nephrogenic blastema beyond 36 weeks gestation Precursor to Wilms tumor GU Neoplasms
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Nephroblastomatosis Two basic appearances: Confluent peripheral mass Focal cortical mass or masses GU Neoplasms
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Nephroblastomatosis 2 types by location Perilobar Peripheral cortex or columns of Bertin Intralobar Deep cortex Greater risk of Wilms tumor GU Neoplasms
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Nephroblastomatosis GU Neoplasms
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Multilocular Cystic Renal Neoplasm GU Neoplasms Biphasic age distribution Boys 3 months to 2 years Women > 40 years Typically asymptomatic Can have pain & hematuria from prolapse into ureter
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Multilocular Cystic Renal Neoplasm GU Neoplasms Composed of cysts & septa Encapsulated Mean diameter 7 to 10 cm
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Cystic Masses GU Neoplasms < 5 years of age Multilocular cystic renal tumor Multicystic dysplastic kidney >5 years of age Simple renal cysts (rare)
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Ossifying Renal Tumor of Infancy Extremely rare ?arise from urothelium Reniform contour usually maintained May have calcifications in the collecting system May mimic a staghorn calculus GU Neoplasms
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Pediatric Renal Tumors by Age 0-2 years Rhabdoid Tumor Mesoblastic Nephroma Nephro- blastomatosis *Multilocular Cystic Nephroma
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Pediatric Renal Tumors by Age 2-10 years
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What stage is this tumor? A. IIA B. III C. IV D. V Peds Renal Masses Wilms Tumor: Question
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What stage is this tumor? A. IIA B. III C. IV D. V Peds Renal Masses Wilms Tumor: Question
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Wilms Tumor Peds Renal Masses Epidemiology: 6%-7% of all childhood cancers Approximately 500 cases/year
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Wilms Tumor Associated syndromes: Aniridia WAGR syndrome (Wilms tumor, aniridia, genital abnormalities, retardation) (WT1 gene) Peds Renal Masses
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Wilms Tumor Associated syndromes: Beckwith- Wiedemann syndrome & hemihypertrophy (WT2 gene) Drash syndrome (nephritis & male pseudohermaphro dism) WT 1 gene Peds Renal Masses
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Usu 2/2 direct spread from RP LN Primary Lymphoma rare Renal involvement more common on NHL (esp Burkitts)
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Variable imaging findings Solitary or solid renal masses Most common pattern is multiple rounded masses
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4-5% of renal tumors in peds peak 1-4years reported male predom Usually unilat Imaging unable to diff from Wilms
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Pediatric Renal Tumors by Age 2-10 years Wilms Tumor Non-Hodgkins Lymphoma Clear Cell Sarcoma
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Pediatric Renal Tumors by Age 10+ years
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Renal Cell Carcinoma
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CASE
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Renal Cell Carcinoma GU Neoplasms When seen in peds typically older, mean age 9 years Hematuria Commonly solid mass (as in adults)
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Renal Cell Carcinoma GU Neoplasms Vascular invasion in to the renal veins or IVC not uncommmon
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Classic Lesions Hemangioblastoma Retinal Angioma (Hemangioblastoma) Pancreatic Cyst Renal Cysts and Ca Pheochromocytoma Epididymal Cystadenoma Endolymphatic sac tumor
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Pediatric Renal Tumors by Age 10+ years Renal Cell Carcinoma Hodgkins Lymphoma Renal Medullary Carcinoma Angiomyolipoma
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Pediatric Renal Tumors by Age 0-2 years2-10 years10+ years Rhabdoid Tumor Wilms Tumor Renal Cell Carcinoma Mesoblastic Nephroma Non-Hodgkins Lymphoma Hodgkins Lymphoma Nephro- blastomatosis Clear Cell SarcomaRenal Medullary Carcinoma *Multilocular Cystic Nephroma Angiomyolipoma
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Neuroblastoma 2nd most common abdominal malignancy (after Wilms tumor) 10% of pediatric cancers 500-525 new cases/yr in the US Mean age ~ 2 yrs. 75% < 5 yrs. GU Neoplasms
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I. Introduction: Diagnostic Radiology role in Radiation Oncology II. Common Pediatic Renal Lesions III. Lesions Most Important to Rad Onc IV. Imaging Staging of Wilms Tumor
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Wilms: Staging Limited to the kidney and completely resectable Renal capsule intact Renal sinus may be infiltrated but not beyond hilum Kidney Mass Stage I
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Wilms: Staging Tumor infiltrates beyond kidney Completely resected Includes tumor with local spillage confined to flank Kidney Mass Stage II
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Wilms: Staging Residual tumor confined to abdomen, non- hematogenous; includes : (a) positive abdominal nodes (b) diffuse peritoneal contamination by direct growth, implants, or spillage (c) positive margins (d) residual nonresected tumor Kidney Stage III mass LN aorta LN
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Wilms: Staging Hemato- genous disease (added: lungs, lymph nodes, liver) Kidney Stage IV mass M M M M M
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Wilms: Staging Bilateral disease; each side should be staged separately, since prognosis is dependent on the higher individual stage Kid Stage V Mass Kid Mass
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I. Neonate Mesoblastic nephroma II. 6 months to 5 years of age WILMS TUMOR Nephroblastomatosis Rhabdoid tumor Clear cell sarcoma
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Peds Renal Masses: Unique Features Renal MassClinical Features Wilms TumorLarge Solid Mass, +/- vascular invasion Rhabdoid TumorSubcapsular Fluid, Brain Mets Renal Cell Carcinoma In Peds, look for assoc VHL Nephroblasto- matosis Multiple supcapsular solid masses *Multilocular Cystic Nephroma Multicystic mass, little solid tissue, invagination into renal pelvis LymphomaVariable appearance, assoc LAD
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Tumor Prognosis: Summary LowIntermediate High Mesoblastic Nephroma Wilms Tumor (non-anaplastic types) Renal Cell Carcinoma Multilocular Cystic Nephroma Wilms (anaplastic) Angiomyolipoma Rhabdoid Wilms (Highly Epithelial type) Clear Cell, Renal Medullary
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Jesse Courtier, MD Assistant Clinical Professor of Radiology UCSF Benioff Children’s Hopsital.

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Transcript of Jesse Courtier, MD Assistant Clinical Professor of Radiology UCSF Benioff Children’s Hopsital.