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Pediatric Renal Tumors

Pediatric Renal Tumors is a topic covered in the Pediatric Surgery NaT.

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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.

Pediatric Renal Tumors

Tumor

Average age at diagnosis

Prevalence of pediatric renal tumors

Unique presenting symptoms

Unique additional diagnostic workup*

Syndrome / Genetics

Important features

Wilms tumor

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

Renal cell carcinoma

- 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

Rhabdoid tumor of the kidney

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

Congenital mesoblastic nephroma

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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Last updated: October 24, 2018

Citation

Weil, Brent R, and Marcus M Malek. "Pediatric Renal Tumors." Pediatric Surgery NaT, American Pediatric Surgical Association, 2018. APSA Webapp, www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829660/all/Pediatric_Renal_Tumors.
Weil BR, Malek MM. Pediatric Renal Tumors. In: Waldhausen J, Powell D, Hirschl R, eds. Pediatric Surgery NaT. American Pediatric Surgical Association; 2018. https://www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829660/all/Pediatric_Renal_Tumors. Accessed July 17, 2019.
Weil, B. R., & Malek, M. M. (2018). Pediatric Renal Tumors. In Waldhausen, J., Powell, D., & Hirschl, R. (Eds.), Pediatric Surgery NaT. Available from https://www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829660/all/Pediatric_Renal_Tumors
Weil BR, Malek MM. Pediatric Renal Tumors [Internet]. In: Waldhausen J, Powell D, Hirschl R, editors. Pediatric Surgery NaT. American Pediatric Surgical Association; 2018. [cited 2019 July 17]. Available from: https://www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829660/all/Pediatric_Renal_Tumors.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Pediatric Renal Tumors ID - 829660 A1 - Weil,Brent,MD AU - Malek,Marcus,M.D. ED - Waldhausen,John, ED - Powell,David, ED - Hirschl,Ron, Y1 - 2018/10/24/ BT - Pediatric Surgery NaT UR - https://www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829660/all/Pediatric_Renal_Tumors PB - American Pediatric Surgical Association DB - APSA Webapp DP - Unbound Medicine ER -