Pediatric Surgery NaT
Pediatric Renal Tumors
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Introduction
Wilms tumor is by far the most commonly encountered renal tumor of childhood. It is not until approximately age 15 years and older that renal cell carcinoma becomes the most commonly occurring renal tumor. Still, approximately 15% of the renal tumors that develop in children will be something other than Wilms tumor. Many of the principles guiding the surgical treatment of these tumors are similar to those employed for Wilms tumor. Nevertheless, there are several key differences and unique features among these tumors that the pediatric surgeon should become familiar with. A brief comparison of these tumors is provided in the table below.
Tumor | Average age at diagnosis | Prevalence of pediatric renal tumors | Unique presenting symptoms | Unique additional diagnostic workup* | Syndrome / Genetics | Important features |
three years (range zero years to adult) | 85% | painless abdominal mass | Beckwith-Wiedemann syndrome WAGR syndrome Denys-Drash syndrome Frasier syndrome Li-Fraumeni syndrome Any condition involving mutation of the WT1 gene | Radical nephroureterectomy with lymph node sampling and avoidage of tumor spillage is the surgical standard of care for unilateral tumors | ||
- Papillary - Trans- location associated - Renal medullary carcinoma - Oncocytic renal cell carcinoma following neuroblastoma | older than 15 years | 2% | hematuria | may carry Xp11 (TFE3)translocation von Hippel-Lindau tuberous sclerosis familial renal cell carcinoma | partial nephrectomy considered if negative margins achievable | |
11 months (range zero to four years) | 3.7% | fever, hematuria, advanced stage | brain CT or MRI | loss of function of SMARCB1/INI1/SNF5/BAF47 gene (chromosome 22q11.2) | 10 to 15% have concomitant central nrevous system lesions no survival without complete resection | |
Clear cell sarcoma of the kidney | 36 mo (range two months to 14 years) | 3.3% | 2:1 male to female ratio | PET or bone scan, consider brain CT or MRI | t(10;17) translocation and/or 14q deletion in some patients duplications in exon 15 of BCOR gene and /or a YWHAE-FAM22 gene fusion | spreads to lung, bone, brain and soft tissue brain frequent site of recurrent disease |
one to two months (90% present in infancy) | 2.2% | young age 2:1 male to female ratio | 12;15) (p13;q25) translocation/ETV6-NTRK3 fusion gene | overall excellent outcomes, but cellular variant can be aggressive incomplete resection also worsens prognosis | ||
Cystic nephroma | infants/young children or adult females | 2% | multilocular renal cysts | DICER1 mutation (pediatric cases) | benign | |
Ossifying renal tumor of infancy | less than one year | less than 1% | hematuria is universal | benign consider nephron sparing surgery | ||
* The standard workup includes magnetic resonance imaging (MRI) or computerized tomography (CT) of the abdomen and pelvis with attention to renal vein, inferior vena cava and contralateral kidney; chest CT; complete blood count, serum calcium, platelet count and coagulation profile (to asses for acquired von Willebrand disease) | ||||||
The purpose of the linked sections that follow is to familiarize surgeons with the distinguishing features and management principles associated with renal tumors of childhood, so that optimal surgical and multidisciplinary care can be provided when such patients are encountered.
see also Wilms Tumor, Renal Cell Carcinoma, Rhabdoid Tumor of the Kidney, Clear Cell Sarcoma of the Kidney, Mesoblastic Nephroma, Cystic Nephroma and Nephrectomy for Tumor.
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Introduction
Wilms tumor is by far the most commonly encountered renal tumor of childhood. It is not until approximately age 15 years and older that renal cell carcinoma becomes the most commonly occurring renal tumor. Still, approximately 15% of the renal tumors that develop in children will be something other than Wilms tumor. Many of the principles guiding the surgical treatment of these tumors are similar to those employed for Wilms tumor. Nevertheless, there are several key differences and unique features among these tumors that the pediatric surgeon should become familiar with. A brief comparison of these tumors is provided in the table below.
Tumor | Average age at diagnosis | Prevalence of pediatric renal tumors | Unique presenting symptoms | Unique additional diagnostic workup* | Syndrome / Genetics | Important features |
three years (range zero years to adult) | 85% | painless abdominal mass | Beckwith-Wiedemann syndrome WAGR syndrome Denys-Drash syndrome Frasier syndrome Li-Fraumeni syndrome Any condition involving mutation of the WT1 gene | Radical nephroureterectomy with lymph node sampling and avoidage of tumor spillage is the surgical standard of care for unilateral tumors | ||
- Papillary - Trans- location associated - Renal medullary carcinoma - Oncocytic renal cell carcinoma following neuroblastoma | older than 15 years | 2% | hematuria | may carry Xp11 (TFE3)translocation von Hippel-Lindau tuberous sclerosis familial renal cell carcinoma | partial nephrectomy considered if negative margins achievable | |
11 months (range zero to four years) | 3.7% | fever, hematuria, advanced stage | brain CT or MRI | loss of function of SMARCB1/INI1/SNF5/BAF47 gene (chromosome 22q11.2) | 10 to 15% have concomitant central nrevous system lesions no survival without complete resection | |
Clear cell sarcoma of the kidney | 36 mo (range two months to 14 years) | 3.3% | 2:1 male to female ratio | PET or bone scan, consider brain CT or MRI | t(10;17) translocation and/or 14q deletion in some patients duplications in exon 15 of BCOR gene and /or a YWHAE-FAM22 gene fusion | spreads to lung, bone, brain and soft tissue brain frequent site of recurrent disease |
one to two months (90% present in infancy) | 2.2% | young age 2:1 male to female ratio | 12;15) (p13;q25) translocation/ETV6-NTRK3 fusion gene | overall excellent outcomes, but cellular variant can be aggressive incomplete resection also worsens prognosis | ||
Cystic nephroma | infants/young children or adult females | 2% | multilocular renal cysts | DICER1 mutation (pediatric cases) | benign | |
Ossifying renal tumor of infancy | less than one year | less than 1% | hematuria is universal | benign consider nephron sparing surgery | ||
* The standard workup includes magnetic resonance imaging (MRI) or computerized tomography (CT) of the abdomen and pelvis with attention to renal vein, inferior vena cava and contralateral kidney; chest CT; complete blood count, serum calcium, platelet count and coagulation profile (to asses for acquired von Willebrand disease) | ||||||
The purpose of the linked sections that follow is to familiarize surgeons with the distinguishing features and management principles associated with renal tumors of childhood, so that optimal surgical and multidisciplinary care can be provided when such patients are encountered.
see also Wilms Tumor, Renal Cell Carcinoma, Rhabdoid Tumor of the Kidney, Clear Cell Sarcoma of the Kidney, Mesoblastic Nephroma, Cystic Nephroma and Nephrectomy for Tumor.
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