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

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

3. 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

4. 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

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

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

7. 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

8. How the Radiologist Can Help

10. Rad Oncology Workflow
New Consult

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

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

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

14. 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

15. 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

16. 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

17. 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

18. 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

19. 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

20. 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

21. Pediatric Renal Tumors by Age
0-2 years

22. GU Neoplasms
Case

23. GU Neoplasms
Rhabdoid Tumor

24. 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

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

29. Axial T1 Post Contrast MR image of the level of the kidneys: Large right renal mass (yellow arrows) and several smaller masses in the left kidney (white arrows

30. 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)

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

32. 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)

33. 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)

34. 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.)

35. Rhabdoid Tumor Heterogeneous mass Subcapsular collection
Can have synchronous brain mets

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

37. Mesoblastic Nephroma

38. Mesoblastic Nephroma

39. GU Neoplasms
Case

40. GU Neoplasms
Nephroblastomatosis

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

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

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

44. GU Neoplasms
Nephroblastomatosis

45. GU Neoplasms
Nephroblastomatosis

46. CASE

47. Multilocular Cystic Nephroma

48. 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

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

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

51. 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

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

53. Pediatric Renal Tumors by Age
2-10 years

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

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

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

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

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

61. 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)

62. Case

63. Lymphoma

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

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

68. 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

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

70. Pediatric Renal Tumors by Age
10+ years

71. Renal Cell Carcinoma

72. CASE

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

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

84. 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

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

86. 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

87. Neuroblastoma GU Neoplasms
2nd most common abdominal malignancy (after Wilms tumor)
10% of pediatric cancers
new cases/yr in the US
Mean age ~ 2 yrs.
75% < 5 yrs.

88. International neuroblastoma staging system

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

90. 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

91. 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

92. 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

93. 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

94. 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

95. 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

96. 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

97. 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

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

99. 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

100. 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

101. 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

102. 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

103. 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

104. 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

105. 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

106. 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

107. 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

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

109. Thank you!