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KEY FACTS
Terminology
Imaging
Pathology
Clinical Issues
TERMINOLOGY
Definitions
- Nephrogenic rests (NR): Metanephric blastema that persists after 36-weeks gestation; precursor to Wilms tumor
- Nephroblastomatosis: Presence of multiple or diffuse NR
- Intralobar NR: Occur anywhere in renal parenchyma
- Perilobar NR: Occur in peripheral cortex
Associated Syndromes
- Perilobar NR: Beckwith-Wiedemann syndrome, hemihypertrophy, Perlman syndrome, trisomy 18
- Intralobar NR: Drash syndrome, sporadic aniridia, WAGR syndrome
IMAGING
General Features
Ultrasonographic Findings
CT Findings
MR Findings
Nuclear Medicine Findings
Imaging Recommendations
Differentiation From Wilms Tumor
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
- Sandberg JK et al: Imaging characteristics of nephrogenic rests versus small Wilms tumors: a report from the Children's Oncology Group Study AREN03B2. AJR Am J Roentgenol. 214(5):987-94, 2020
- Chung EM et al: Renal tumors of childhood: radiologic-pathologic correlation part 1. the 1st decade: from the radiologic pathology archives. Radiographics. 36(2):499-522, 2016
- Littooij AS et al: Intra- and interobserver variability of whole-tumour apparent diffusion coefficient measurements in nephroblastoma: a pilot study. Pediatr Radiol. 45(11):1651-60, 2015
- Servaes S et al: Comparison of diagnostic performance of CT and MRI for abdominal staging of pediatric renal tumors: a report from the Children's Oncology Group. Pediatr Radiol. 45(2):166-72, 2015
- Uslu L et al: Value of 18F-FDG PET and PET/CT for evaluation of pediatric malignancies. J Nucl Med. 56(2):274-86, 2015
- Cox SG et al: Magnetic resonance imaging versus histopathology in Wilms tumor and nephroblastomatosis: 3 examples of noncorrelation. J Pediatr Hematol Oncol. 36(2):e81-4, 2014
- Stabouli S et al: Perilobar nephroblastomatosis: natural history and management. Case Rep Pediatr. 2014:756819, 2014
- Sethi AT et al: Best cases from the AFIP: Wilms tumor in the setting of bilateral nephroblastomatosis. Radiographics. 30(5):1421-5, 2010
Related Anatomy
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Pathology
Clinical Issues
TERMINOLOGY
Definitions
- Nephrogenic rests (NR): Metanephric blastema that persists after 36-weeks gestation; precursor to Wilms tumor
- Nephroblastomatosis: Presence of multiple or diffuse NR
- Intralobar NR: Occur anywhere in renal parenchyma
- Perilobar NR: Occur in peripheral cortex
Associated Syndromes
- Perilobar NR: Beckwith-Wiedemann syndrome, hemihypertrophy, Perlman syndrome, trisomy 18
- Intralobar NR: Drash syndrome, sporadic aniridia, WAGR syndrome
IMAGING
General Features
Ultrasonographic Findings
CT Findings
MR Findings
Nuclear Medicine Findings
Imaging Recommendations
Differentiation From Wilms Tumor
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
- Sandberg JK et al: Imaging characteristics of nephrogenic rests versus small Wilms tumors: a report from the Children's Oncology Group Study AREN03B2. AJR Am J Roentgenol. 214(5):987-94, 2020
- Chung EM et al: Renal tumors of childhood: radiologic-pathologic correlation part 1. the 1st decade: from the radiologic pathology archives. Radiographics. 36(2):499-522, 2016
- Littooij AS et al: Intra- and interobserver variability of whole-tumour apparent diffusion coefficient measurements in nephroblastoma: a pilot study. Pediatr Radiol. 45(11):1651-60, 2015
- Servaes S et al: Comparison of diagnostic performance of CT and MRI for abdominal staging of pediatric renal tumors: a report from the Children's Oncology Group. Pediatr Radiol. 45(2):166-72, 2015
- Uslu L et al: Value of 18F-FDG PET and PET/CT for evaluation of pediatric malignancies. J Nucl Med. 56(2):274-86, 2015
- Cox SG et al: Magnetic resonance imaging versus histopathology in Wilms tumor and nephroblastomatosis: 3 examples of noncorrelation. J Pediatr Hematol Oncol. 36(2):e81-4, 2014
- Stabouli S et al: Perilobar nephroblastomatosis: natural history and management. Case Rep Pediatr. 2014:756819, 2014
- Sethi AT et al: Best cases from the AFIP: Wilms tumor in the setting of bilateral nephroblastomatosis. Radiographics. 30(5):1421-5, 2010
