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Wilms Tumor Overview
Wilms Tumor Overview
Physical examination:
• Firm, nontender, smooth mass that rarely crosses the midline and
generally does not move with respiration. (versus neuroblastoma or
splenomegaly)
• Look for associated anomalies, such as aniridia, hemihypertrophy, and
genitourinary anomalies.
Role of imaging
• Establish the presence of a renal tumor
(vs. hydronephrosis or multicystic kidney
disease)
• Size and extent of the tumor
• Intravascular tumor thrombosis (Doppler
U/S)
• Evaluate contralateral kidney
• Presence and function
• Tumor involvement
• Nephrogenic rests
• Lung metastases
• Guides management decisions
• Surgical approach
• Preoperative chemotherapy
Treatment
Europe:
• Preoperative neoadjuvant chemotherapy
• Postoperative chemotherapy based on pathologic
response
United States:
• Primary nephrectomy
• Postoperative chemotherapy based on diagnosis and
stage
• Central Pathology Review (“center of excellence”)
• COG registry into Tumor Classification and Banking
protocol
• Recut slides, pathology report, frozen tissue (normal
and tumor), paraffin block, other samples
Therapeutic protocols:
1. Low- and standard-risk FHWT
2. High-risk FHWT
3. Bilateral Wilms tumor, unilateral Wilms tumor with high risk
for renal failure, and nephroblastomatosis
4. High-risk renal tumors (CCSK, MRT, anaplastic Wilms
Tumor, renal cell carcinoma)
Staging of pediatric renal tumors
In the gross room
• A solitary well circumscribed
or lobular mass with gray to
pink variegated appearance.
• Cystic changes (PPE! And stand back! )
• Necrosis and hemorrhage
are common
• Location in kidney
• Nodules – number and size
• Friable – drag artifact
Faria P, Beckwith JB, Mishra K, et al. Focal Versus Diffuse Anaplasia in Wilms Tumor – New Definitions With Prognostic
Significance. A Report from the National Wilms Tumor Study Group. Am J Surg Path 1996:20(8);909-920.
Nephrogenic rests
• Abnormally persistent foci of
embryonal cells that are capable
of developing into
nephroblastomas
• Perilobar
• Intralobar
• Combined
WT
Mesoblastic nephroma
Rhabdoid tumor
• Neuroblastoma
• Synovial sarcoma
• Primitive neuroectodermal tumor
• Rhabdomyosarcoma
• Perlman EJ. Pediatric Renal Tumors: Practical Updates for the Pathologist. Pediatric and
Developmental Pathology 2005;8:320–338
• Faria P, Beckwith JB, Mishra K, et al. Focal Versus Diffuse Anaplasia in Wilms Tumor – New
Definitions With Prognostic Significance. A Report from the National Wilms Tumor Study
Group. Am J Surg Path 1996:20(8);909-920.
• Tumors of the Kidney, Bladder, and Related Urinary Structures. AFIP Atlas of Tumor Pathology
– 4th Series
• Lester SC. Manual of Surgical Pathology pp. China:Elsevier; 2006. pp. 380-385.
• Bernstein L, Linet M, Smith MA, Olshan AF. RENAL TUMORS National Cancer Institute 79 SEER Pediatric Monograph IN:
Ries LAG, Smith MA, Gurney JG, Linet M, Tamra T, Young JL, Bunin GR (eds). Cancer Incidence and Survival among
Children and Adolescents: United States SEER Program 1975-1995, National Cancer Institute, SEER Program. NIH Pub. No.
99-4649. Bethesda, MD, 1999.
• Beckwith JB. Wilms’ Tumor and Other Renal Tumors of Childhood: a selective review from the National Wilms’ Tumor Study
Pathology Center. Human Pathology. 1983:14(6);481-492.
• Zuppan CW, Beckwith B, Luckey DW. Anaplasia in Unilateral Wilms’ tumor: a report from the National Wilms’ Tumor Study
Pathology Center. Human Pathology. 1988:19(10);1199-1209.