Back to Pediatric Renal Home Page

REVIEW OF PEDIATRIC RENAL NEOPLASMS
John Hicks
Texas Children's Hospital, Houston, TX

RENAL CELL CARCINOMA

INDEX

Renal Cell Carcinoma: Epidemiology
Pediatric Renal Cell Carcinoma 2% of All Cases

Mean Age 9.8 Years

Gender Ratio 1.0M : 1.0F

African-American Race:
Pediatric 21%
Adults 6%

Bilateral More Frequent in Pediatric Cases
von Hippel-Lindau Syndrome (33 to 50%)

Associated with Tuberous Sclerosis,
Autosomal Dominant Polycystic Kidney Disease

 

Renal Cell Carcinoma: Symptoms and Signs
Presenting Symptoms:
Palpable Mass 24% (by parents) -- 64% (by physician)
Nausea/Vomiting, Malaise 44%
Weight Loss, Lethargy
Hematuria 40%
Pain 32%
Hypertension 6%
Urologic Abnormality 7%
Mass, Hematuria, Pain 8-14%

Detected by Imaging 97%
Tumor Size 6cm
Calcifications 24%
Renal Cell Carcinoma: Pathology;  
Light microscopy (A) and Ultrastructure (B) of the Clear Cell Variant
renal cell carcinoma - lm renal cell carcinoma - em
Figure 5A: Relatively large epithelial cells with clear cytoplasmic arranged in nests separated by fibrous septa.  Figure 5B: Tumor cells arranged into glandular formation with cellular attachments characteristic for epithelial differentiation.

 

Renal Cell Carcinoma: Prognosis
Improved Prognosis:
Symptoms <1 week or >6 months
Pseudocapsule
Clear Cell-Trabecular Pattern
Stage at Diagnosis (5 year survival)
-Tumor Extending Through Capsule: 80%
-Regional Lymph Nodes Involved: 75%
Functioning Kidney

Poor Prognosis:
Granular Cell/Papillary Pattern
Stage at Diagnosis (5 year survival)
-Vascular Invasion: 0 to 30%
-Distant Metastases: 10%
Non-functioning Kidney (11% survival)
Rupture of Kidney

Metastatic Disease:
Regional Lymph Nodes
Lung, Bone, Liver

Management: Primarily Surgical
Renal Cell Carcinoma: Cytogenetics
Cytogenetics:
Non-papillary Renal Cell Carcinoma
-3p and 14q deletions
-5q rearrangements
Papillary Renal Adenomas
-Trisomy 7 and 17
-Loss of Y
Papillary Renal Cell Carcinomas
-Trisomy 16, 20, 12
Chromophobe Renal Cell Carcinoma
-Diverse, Allelic Loss
-Rearrangement in Mitochondrial DNA

von Hippel-Lindau Syndrome:
3p deletion
VHL, tumor suppressor gene (3p25)
-Mutations or Deletions in both
-Syndromatic and Sporadic Cancers


INDEX
Nephroblastoma (Wilms tumor)
Congenital Mesoblastic Nephroma
Clear Cell Sarcoma
Malignant Rhabdoid Tumor
Angiomyolipoma
Ossifying Tumor of Infantile Kidney

Top of page

SUP Logo