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Presented by Dr. Pedram Argani and prepared by Dr. Yembur Ahmad
This case talks about bilateral renal tumors in a pediatric patient.
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1. Question
A young child presents with bilateral renal tumors after therapy. The tumor illustrated is the largest one. What is the diagnosis?
Correct
Answer: C
Histologic Description: This is a post-therapy specimen, so there are large areas of regression consisting of macrophages and scarring. There are also areas of differentiated epithelium at the periphery of the tumor. Other areas show the usual proliferating blastemal elements that are non-anaplastic. However, there is well-defined nodule of blastemal cells with markedly enlarged nuclei showing hyperchromasia, along with atypical mitotic figures. This focus was well delineated and confined to the kidney, which justifies the diagnosis of focal anaplasia.
Differential Diagnosis: The presence of enlarged hyperchromatic nuclei demonstrating atypical mitotic figures supports the diagnosis of anaplasia (unfavorable histology). Focal anaplasia must be well delineated and confined to the kidney, as seen in the current case. If not, anaplasia is considered diffuse by default. Desmoplastic small round cell tumor is immunoreactive for WT1, desmin and keratin like the current Wilms tumor; however, it typically demonstrates desmoplastic stroma and nuclei that have coarser chromatin than the delicate chromatin of a blastemal Wilms tumor nucleus.Incorrect
Answer: C
Histologic Description: This is a post-therapy specimen, so there are large areas of regression consisting of macrophages and scarring. There are also areas of differentiated epithelium at the periphery of the tumor. Other areas show the usual proliferating blastemal elements that are non-anaplastic. However, there is well-defined nodule of blastemal cells with markedly enlarged nuclei showing hyperchromasia, along with atypical mitotic figures. This focus was well delineated and confined to the kidney, which justifies the diagnosis of focal anaplasia.
Differential Diagnosis: The presence of enlarged hyperchromatic nuclei demonstrating atypical mitotic figures supports the diagnosis of anaplasia (unfavorable histology). Focal anaplasia must be well delineated and confined to the kidney, as seen in the current case. If not, anaplasia is considered diffuse by default. Desmoplastic small round cell tumor is immunoreactive for WT1, desmin and keratin like the current Wilms tumor; however, it typically demonstrates desmoplastic stroma and nuclei that have coarser chromatin than the delicate chromatin of a blastemal Wilms tumor nucleus.