Imaging of Pediatric Renal Masses Jesse Courtier, MD Assistant Clinical Professor of Radiology UCSF Benioff Children’s Hopsital

Objectives I.Role of Dx Radiology in Rad Oncology II. Review Imaging of Common Pediatric Renal Masses III.Review Imaging Staging of Wilms Tumor

Objectives III.Review Imaging Staging of Wilms Tumor I.Role of Dx Radiology in Rad Oncology II. Review Imaging of Common Pediatric Renal Masses III.Review Imaging Staging of Wilms Tumor

Objectives III.Review Imaging Staging of Wilms Tumor I.Role of Dx Radiology in Rad Oncology II. Review Imaging of Common Pediatric Renal Masses III.Review Imaging Staging of Wilms Tumor

OUTLINE Introduction: Diagnostic Radiology role in Radiation Oncology Common Pediatric Renal Lesions Lesions Most Important to Rad Onc Imaging Staging of Wilms Tumor

OUTLINE Introduction: Diagnostic Radiology role in Radiation Oncology Common Pediatric Renal Lesions Lesions Most Important to Rad Onc Imaging Staging of Wilms Tumor

Dx Radiology Role In Rad Oncology Introduction Diagnostic Radiology & Radiation Oncology formerly integrated in the 1950’s Currently separated, both still cert by ABR Little specific training in Dx Radiology on Rad Therapy outside of I-131

How the Radiologist Can Help

Rad Oncology Workflow New Consult

I. Accurate Imaging Based Staging Rad Oncology Workflow New Consult I. Accurate Imaging Based Staging

I. Accurate Imaging Based Staging Rad Oncology Workflow New Consult Simulation I. Accurate Imaging Based Staging

I. Accurate Imaging Based Staging Rad Oncology Workflow New Consult Simulation Contouring I. Accurate Imaging Based Staging

Rad Oncology Workflow Contouring I. Accurate Imaging Based Staging New Consult Simulation Contouring I. Accurate Imaging Based Staging II. Aid with tumor Target/ normal tissue

Computer-ized Treatment Rad Oncology Workflow New Consult Simulation Contouring Computer-ized Treatment I. Accurate Imaging Based Staging II. Aid with tumor Target/ normal tissue

Computer-ized Treatment Rad Oncology Workflow New Consult Simulation Contouring Computer-ized Treatment 2nd check, QA testing I. Accurate Imaging Based Staging II. Aid with tumor Target/ normal tissue

Computer-ized Treatment Delivery of Tx Plan by Rad Onc Rad Oncology Workflow New Consult Simulation Contouring Computer-ized Treatment 2nd check, QA testing Delivery of Tx Plan by Rad Onc I. Accurate Imaging Based Staging II. Aid with tumor Target/ normal tissue

Rad Oncology Workflow New Consult 2nd check, QA testing Simulation Contouring Computer-ized Treatment 2nd check, QA testing Delivery of Tx Plan by Rad Onc Follow up care / response Ass-essment I. Accurate Imaging Based Staging II. Aid with tumor Target/ normal tissue III. Interpretation of Post Tx Imaging

Dx Radiology Role In Rad Oncology Introduction 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

OUTLINE Common Pediatric Renal Lesions Introduction: Diagnostic Radiology role in Radiation Oncology Common Pediatric Renal Lesions Lesions Most Important to Rad Onc Imaging Staging of Wilms Tumor

Pediatric Renal Tumors by Age 0-2 years

GU Neoplasms Case

GU Neoplasms Rhabdoid Tumor

Rhabdoid Tumor 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

Coronal Neonatal Head US image showing multiple echogenic foci in the basal ganglia

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

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)

Coronal post contrast T1 image of the brain showing multiple enhancing lesions (yellow arrows)

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)

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)

Rhabdoid Tumor: Pathology ➢ Large mass (<300 gm) ➢ Infiltrating Histology ➢ Mononuclear cells ➢ Eccentric nuclei and eosinophilic cytoplasm Photograph of a gross specimen shows a round, lobulated mass with a nonspecific appearance. A small amount of normal kidney (K) is noted at the edge of the specimen. (Reprinted, with permission, from reference 7.)

Rhabdoid Tumor Heterogeneous mass Subcapsular collection Can have synchronous brain mets

Mesoblastic Nephroma Most common renal neoplasm in pts <3mos Rarely occurs in >6mos Benign tumor US: heterog echogen CT: usu heterog low atten mass

Mesoblastic Nephroma

Mesoblastic Nephroma

GU Neoplasms Case

GU Neoplasms Nephroblastomatosis

Nephroblastomatosis GU Neoplasms "the presence of nephrogenic rests or nephrogenic blastema beyond 36 weeks gestation” Precursor to Wilms tumor

Nephroblastomatosis GU Neoplasms Two basic appearances: ➢ Confluent peripheral mass ➢ Focal cortical mass or masses

Nephroblastomatosis GU Neoplasms 2 types by location • Perilobar ➢ Peripheral cortex or columns of Bertin • Intralobar ➢ Deep cortex ➢ Greater risk of Wilms tumor

GU Neoplasms Nephroblastomatosis

GU Neoplasms Nephroblastomatosis

CASE

Multilocular Cystic Nephroma

Multilocular Cystic Renal Neoplasm GU Neoplasms Multilocular Cystic Renal Neoplasm Biphasic age distribution Boys 3 months to 2 years Women > 40 years Typically asymptomatic Can have pain & hematuria from prolapse into ureter

Multilocular Cystic Renal Neoplasm GU Neoplasms Multilocular Cystic Renal Neoplasm Composed of cysts & septa Encapsulated Mean diameter 7 to 10 cm

Cystic Masses GU Neoplasms < 5 years of age ➢ Multilocular cystic renal tumor ➢ Multicystic dysplastic kidney >5 years of age ➢ Simple renal cysts (rare)

Ossifying Renal Tumor of Infancy GU Neoplasms 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

Pediatric Renal Tumors by Age 0-2 years Rhabdoid Tumor Mesoblastic Nephroma Nephro-blastomatosis *Multilocular Cystic Nephroma

Pediatric Renal Tumors by Age 2-10 years

Wilms Tumor: Question Peds Renal Masses What stage is this tumor? IIA III IV V

Wilms Tumor: Question V Peds Renal Masses What stage is this tumor? IIA III IV V

Wilms Tumor Peds Renal Masses Epidemiology: 6%-7% of all childhood cancers Approximately 500 cases/year

Wilms Tumor Peds Renal Masses Associated syndromes: Aniridia WAGR syndrome (Wilms tumor, aniridia, genital abnormalities, retardation) (WT1 gene)

Wilms Tumor Peds Renal Masses Associated syndromes: Beckwith-Wiedemann syndrome & hemihypertrophy (WT2 gene) Drash syndrome (nephritis & male pseudohermaphrodism) WT 1 gene

Wilms Fig. 24.11a–c. Intraperitoneal Wilms’ tumor rupture in a 4-year-old girl presenting with painless right abdominal mass. Enhanced CT scan (a, c) with sagittal reconstruction (b) shows direct peritoneal extension (arrows, a, b) and Douglas’ recess peritoneal location (arrows, c)

Case

Lymphoma

Lymphoma Usu 2/2 direct spread from RP LN Primary Lymphoma rare Renal involvement more common on NHL (esp Burkitt’s)

Lymphoma Variable imaging findings Solitary or solid renal masses Most common pattern is multiple rounded masses

Clear Cell Sarcoma 4-5% of renal tumors in peds peak 1-4years reported male predom Usually unilat Imaging unable to diff from Wilm’s

Pediatric Renal Tumors by Age 2-10 years Wilms Tumor Non-Hodgkin’s Lymphoma Clear Cell Sarcoma

Pediatric Renal Tumors by Age 10+ years

Renal Cell Carcinoma

CASE

Renal Cell Carcinoma GU Neoplasms When seen in peds typically older, mean age 9 years Hematuria Commonly solid mass (as in adults)

Renal Cell Carcinoma GU Neoplasms Vascular invasion in to the renal veins or IVC not uncommmon

VHL: RCC Classic Lesions Hemangioblastoma Retinal Angioma (Hemangioblastoma) Pancreatic Cyst Renal Cysts and Ca Pheochromocytoma Epididymal Cystadenoma Endolymphatic sac tumor Fig. 4 RCC in a 10-year-old boy with known von Hippel Lindau syndrome (VHL). Contrast-enhanced CT axial image shows a heterogeneously enhancing mass in the right kidney, which was later confirmed to be RCC (white arrow). A simple cyst (black arrow) is present in the left kidney

Pediatric Renal Tumors by Age 10+ years Renal Cell Carcinoma Hodgkin’s Lymphoma Renal Medullary Carcinoma Angiomyolipoma

Pediatric Renal Tumors by Age 0-2 years 2-10 years 10+ years Rhabdoid Tumor Wilms Tumor Renal Cell Carcinoma Mesoblastic Nephroma Non-Hodgkin’s Lymphoma Hodgkin’s Lymphoma Nephro-blastomatosis Clear Cell Sarcoma Renal Medullary Carcinoma *Multilocular Cystic Nephroma Angiomyolipoma

Neuroblastoma GU Neoplasms 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.

International neuroblastoma staging system

OUTLINE Introduction: Diagnostic Radiology role in Radiation Oncology Common Pediatric Renal Lesions Lesions Most Important to Rad Onc Imaging Staging of Wilms Tumor

Pediatric Renal Tumors by Age 0-2 years 2-10 years 10+ years Rhabdoid Tumor Wilms Tumor Renal Cell Carcinoma Mesoblastic Nephroma Non-Hodgkin’s Lymphoma Hodgkin’s Lymphoma Nephro-blastomatosis Clear Cell Sarcoma Renal Medullary Carcinoma *Multilocular Cystic Nephroma Angiomyolipoma

Pediatric Renal Tumors by Age 0-2 years 2-10 years 10+ years Rhabdoid Tumor Wilms Tumor Renal Cell Carcinoma Mesoblastic Nephroma Non-Hodgkin’s Lymphoma Hodgkin’s Lymphoma Nephro-blastomatosis Clear Cell Sarcoma Renal Medullary Carcinoma *Multilocular Cystic Nephroma Angiomyolipoma

OUTLINE Imaging Staging of Wilms Tumor Introduction: Diagnostic Radiology role in Radiation Oncology Common Pediatic Renal Lesions Lesions Most Important to Rad Onc Imaging Staging of Wilms Tumor

Wilms: Staging Stage I Kidney Limited to the kidney and completely resectable Renal capsule intact Renal sinus may be infiltrated but not beyond hilum Kidney Mass Fig. 2 Axial T2-W fat-suppressed MR shows a large tumour with left renal origin. The mass was too large to be adequately imaged with US alone

Wilms: Staging Stage II Kidney Tumor infiltrates beyond kidney Completely resected Includes tumor with local spillage confined to flank Kidney Mass Fig. 2 Axial T2-W fat-suppressed MR shows a large tumour with left renal origin. The mass was too large to be adequately imaged with US alone

Wilms: Staging Stage III Kidney LN LN 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 mass aorta LN Fig. 2 Axial T2-W fat-suppressed MR shows a large tumour with left renal origin. The mass was too large to be adequately imaged with US alone LN

Wilms: Staging Stage IV Hemato-genous disease (added: lungs, lymph nodes, liver) M M M M M Fig. 2 Axial T2-W fat-suppressed MR shows a large tumour with left renal origin. The mass was too large to be adequately imaged with US alone Kidney mass

Wilms: Staging Stage V Kid Kid Bilateral disease; each side should be staged separately, since prognosis is dependent on the higher individual stage Kid Kid Mass Mass Fig. 2 Axial T2-W fat-suppressed MR shows a large tumour with left renal origin. The mass was too large to be adequately imaged with US alone

Summary: Renal Masses I. Neonate ➢ Mesoblastic nephroma II. 6 months to 5 years of age ➢ WILMS TUMOR Nephroblastomatosis Rhabdoid tumor Clear cell sarcoma

Peds Renal Masses: Unique Features Clinical Features Wilms Tumor Large Solid Mass, +/- vascular invasion Rhabdoid Tumor Subcapsular 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 Lymphoma Variable appearance, assoc LAD

Peds Renal Masses: Unique Features Clinical Features Wilms Tumor Large Solid Mass, +/- vascular invasion Rhabdoid Tumor Subcapsular 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 Lymphoma Variable appearance, assoc LAD

Peds Renal Masses: Unique Features Clinical Features Wilms Tumor Large Solid Mass, +/- vascular invasion Rhabdoid Tumor Subcapsular 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 Lymphoma Variable appearance, assoc LAD

Peds Renal Masses: Unique Features Clinical Features Wilms Tumor Large Solid Mass, +/- vascular invasion Rhabdoid Tumor Subcapsular 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 Lymphoma Variable appearance, assoc LAD

Peds Renal Masses: Unique Features Clinical Features Wilms Tumor Large Solid Mass, +/- vascular invasion Rhabdoid Tumor Subcapsular 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 Lymphoma Variable appearance, assoc LAD

Peds Renal Masses: Unique Features Clinical Features Wilms Tumor Large Solid Mass, +/- vascular invasion Rhabdoid Tumor Subcapsular 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 Lymphoma Variable appearance, assoc LAD

Peds Renal Masses: Unique Features Clinical Features Wilms Tumor Large Solid Mass, +/- vascular invasion Rhabdoid Tumor Subcapsular 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 Lymphoma Variable appearance, assoc LAD

Peds Renal Masses: Unique Features Clinical Features Wilms Tumor Large Solid Mass, +/- vascular invasion Rhabdoid Tumor Subcapsular 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 Lymphoma Variable appearance, assoc LAD

Tumor Prognosis: Summary Low Intermediate 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

OUTLINE Introduction: Diagnostic Radiology role in Radiation Oncology Common Pediatric Renal Lesions Lesions Most Important to Rad Onc Imaging Staging of Wilms Tumor

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