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Presented by Dr. Pedram Argani and prepared by Dr. Monica Butcher
This is a 3 year old female with a renal mass.
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1. Question
This is a 3 year old female with a renal mass.
Diagnosis:
Correct
Answer: C
Histologic Description: The main neoplasm is composed of primitive small round blue cells which mold to each other. The nuclei are hyperchromatic, and there is abundant mitotic activity. There is no evidence of atypical mitotic activity or marked nuclear pleomorphism. Adjacent to the tumor are primitive tubules which lack significant mitotic activity. These are distributed at the periphery of the renal lobe, in this case underneath the renal capsule. These are the typical features of perilobar nephrogenic rest in the setting of a blastema rich Wilms tumor.
Differential Diagnosis: Anaplastic Wilms tumor would demonstrate pleomorphism and atypical mitotic figures. Clear cell sarcoma of the kidney would demonstrate uniform cells with open or grooved chromatin separated by regular branching fibrovascular septa. The nuclei are typically spaced apart by mucopolysaccharide and do not mold each other. Intralobar nephrogenic rests are stromal rich lesions associated with Wilms tumor. They typical permeate between native nephrons, and are composed of predominantly stroma including fat, unlike the current case which forwards the nests are composed of primitive epithelial cells.
The identification of nephrogenic rests in a nephrectomy for Wilms tumor changes the follow-up for the patient. Identification of rests in the resected kidney indicates that rests are almost certainly present in the contralateral kidney, so that kidney must be closely followed by more frequent ultrasound examinations to detect Wilms tumors at an early stage. This would allow nephron-preserving approaches to be undertaken to prevent the child from ending up on dialysis.
Incorrect
Answer: C
Histologic Description: The main neoplasm is composed of primitive small round blue cells which mold to each other. The nuclei are hyperchromatic, and there is abundant mitotic activity. There is no evidence of atypical mitotic activity or marked nuclear pleomorphism. Adjacent to the tumor are primitive tubules which lack significant mitotic activity. These are distributed at the periphery of the renal lobe, in this case underneath the renal capsule. These are the typical features of perilobar nephrogenic rest in the setting of a blastema rich Wilms tumor.
Differential Diagnosis: Anaplastic Wilms tumor would demonstrate pleomorphism and atypical mitotic figures. Clear cell sarcoma of the kidney would demonstrate uniform cells with open or grooved chromatin separated by regular branching fibrovascular septa. The nuclei are typically spaced apart by mucopolysaccharide and do not mold each other. Intralobar nephrogenic rests are stromal rich lesions associated with Wilms tumor. They typical permeate between native nephrons, and are composed of predominantly stroma including fat, unlike the current case which forwards the nests are composed of primitive epithelial cells.
The identification of nephrogenic rests in a nephrectomy for Wilms tumor changes the follow-up for the patient. Identification of rests in the resected kidney indicates that rests are almost certainly present in the contralateral kidney, so that kidney must be closely followed by more frequent ultrasound examinations to detect Wilms tumors at an early stage. This would allow nephron-preserving approaches to be undertaken to prevent the child from ending up on dialysis.
