Presented by Dr. Pedram Argani and prepared by Dr. Katelynn Davis
This is a 4 year old boy with a renal tumor.
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1. Question
This is a 4 year old boy with a renal tumor.
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Answer: Wilms tumor with intralobar rest
Histologic Description: This is a blastemal rich Wilms tumor, composed of primitive cells with high nuclei cytoplasm ratio and abundant mitotic activity. Focally, tubular formations and stromal areas are present. At the edge of the tumor, where it interfaces with the native kidney, one can appreciate less cellular zones which have fat as a stromal component. These areas intermingle with native nephrons. This is characteristic of intralobar nephrogenic rest.
Differential Diagnosis: Perilobar nephrogenic rest are typically at the periphery of the lobe (most often subcapsular), and typically composed of primitive blastemal cells and tubules. Desmoplastic round cell tumor may label for WT1 and desmin like Wilms tumor; however, it would feature nests of primitive cells in desmoplastic stroma, and would not demonstrate tubule formations as seen in the current case.
Incorrect
Answer: Wilms tumor with intralobar rest
Histologic Description: This is a blastemal rich Wilms tumor, composed of primitive cells with high nuclei cytoplasm ratio and abundant mitotic activity. Focally, tubular formations and stromal areas are present. At the edge of the tumor, where it interfaces with the native kidney, one can appreciate less cellular zones which have fat as a stromal component. These areas intermingle with native nephrons. This is characteristic of intralobar nephrogenic rest.
Differential Diagnosis: Perilobar nephrogenic rest are typically at the periphery of the lobe (most often subcapsular), and typically composed of primitive blastemal cells and tubules. Desmoplastic round cell tumor may label for WT1 and desmin like Wilms tumor; however, it would feature nests of primitive cells in desmoplastic stroma, and would not demonstrate tubule formations as seen in the current case.
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