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Kidney tumor - cysts, children, adult benign

Childhood neoplasms

Renal cell carcinoma in children


Reviewers: Mandolin Ziadie, M.D. (see Reviewers page)
Revised: 13 January 2012, last major update January 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

Epidemiology
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● 4% of renal tumors in children (Pediatr Blood Cancer 2009;52:237)
● 1/3 associated with either tuberous sclerosis, neuroblastoma, teratoma with chemotherapy, Saethre-Chotzen syndrome, chronic renal failure or related diseases in family members (Cancer 2006;107:2906)
● 22% papillary, 20-46% translocation-associated (Am J Clin Pathol 2006;126:349, Histopathology 2008;53:533), 15% clear cell, 8% Wilms’ associated, 5% collecting duct, 5% chromophobe and 24% unclassified (Am J Surg Pathol 2004;28:1117)

Case reports
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● 3 year old girl (Saudi J Kidney Dis Transpl 2009;20:124)

Treatment
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● Excision (Pediatr Surg Int 2007;23:327)
● Retroperitoneal lymph node dissection recommended (Pediatr Blood Cancer 2008;51:475)

Micro description
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● Pediatric papillary RCC shares histologic, immunohistochemical and molecular features with adult papillary RCC
● Xp11.2 translocation RCC shows cells with abundant clear or granular eosinophilic cytoplasm arranged in papilla or nests
● t(6;11) RCC is composed of epithelioid cells with abundant cytoplasm, well-defined borders and small round nuclei arranged in nests / tubules

Positive stains
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● Xp11.2 translocation RCC: TFE3 (nuclear)
● t(6;11) RCC: TFEB (nuclear)

Negative stains
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● Xp11.22 and t(6;11) RCC: cytokeratin, CAM5.2 and EMA

Additional references
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Am J Surg Pathol 1999;23:795

End of Kidney tumor - cysts, children, adult benign > Childhood neoplasms > Renal cell carcinoma in children


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