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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- 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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- 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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- 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 Pediatric Renal Lesions III. Lesions Most
Important to Rad Onc IV. Imaging Staging of Wilms Tumor
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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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- 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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- 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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- 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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- 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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- 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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- 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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- 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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- 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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- 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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- 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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