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Presented by Pedram Argani, M.D. and prepared by Justin Poling, M.D.
Case 3: This is a 1 year old male with a renal mass.
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1. Question
Week 517: Case 3
This is a 1 year old male with a renal mass.images/jsp6252012/case3_2x.jpg
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images/jsp6252012/case3_10x_2.jpgCorrect
Answer: Wilms tumor with intralobar nephrogenic rests
Histology: This is a triphasic Wilms tumor with neoplastic blastemal cells, epithelial tubules, and stroma. The nuclei are relatively uniform and lack abnormal mitotic figures or markedly enlarged forms which would be diagnostic of anaplasia. At the edge of the Wilms tumor, there are primitive nephroblastic elements which intermingle with the native kidney. These are less cellular and have more prominent stroma. These are the typical features of an intralobar nephrogenic rests, which are typically situated at the periphery of a Wilms tumor.
Discussion: Anaplastic Wilms tumors would feature markedly enlarged nuclei with abnormal mitotic figures. Perilobar nephrogenic rests are typical subcapsular, and are lens shaped epithelial predominant lesions. They typically do not intermingle with the native kidney.
The presence of nephrogenic rests in a nephrectomy specimen containing Wilms tumor indicates an increased risk for subsequent development of Wilms tumor in the opposite kidney, which mandates increased radiographic surveillance of the opposite kidney.
Incorrect
Answer: Wilms tumor with intralobar nephrogenic rests
Histology: This is a triphasic Wilms tumor with neoplastic blastemal cells, epithelial tubules, and stroma. The nuclei are relatively uniform and lack abnormal mitotic figures or markedly enlarged forms which would be diagnostic of anaplasia. At the edge of the Wilms tumor, there are primitive nephroblastic elements which intermingle with the native kidney. These are less cellular and have more prominent stroma. These are the typical features of an intralobar nephrogenic rests, which are typically situated at the periphery of a Wilms tumor.
Discussion: Anaplastic Wilms tumors would feature markedly enlarged nuclei with abnormal mitotic figures. Perilobar nephrogenic rests are typical subcapsular, and are lens shaped epithelial predominant lesions. They typically do not intermingle with the native kidney.
The presence of nephrogenic rests in a nephrectomy specimen containing Wilms tumor indicates an increased risk for subsequent development of Wilms tumor in the opposite kidney, which mandates increased radiographic surveillance of the opposite kidney.
