This disease is described in a story by Lorrie Moore with the same title as this case. Here is a typical presentation. A four year old African American girl presents with abdominal pain and hematuria. On exam, you find that she is hypertensive and you palpate a firm, nontender, smooth abdominal mass that does not cross the midline. Further workup involves removing a kidney. The pathology is shown below:
You notice the gross specimen shows infiltration by a fleshy tan mass with a pseudocapsule surrounded by areas of hemorrhage and necrosis. The histology shows three cell types.
Challenge: What's the diagnosis here?
Related Questions:
1. What three cell types are seen on histology?
Images shown under fair use.
You notice the gross specimen shows infiltration by a fleshy tan mass with a pseudocapsule surrounded by areas of hemorrhage and necrosis. The histology shows three cell types.
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wilm's tumor?
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This is Wilms’ tumor, the most common childhood renal malignancy and the fourth most common childhood cancer. It is associated with several congenital syndromes, WAGR (aniridia, GU abnormalities, mental retardation, Wilms’ tumor) and Denys-Drash syndrome (renal disease, male pseudohermaphroditism, Wilms’ tumor), and Beckwith-Wiedemann syndrome (increased childhood tumors). The genes involved are WT1 and WT2. Pathogenesis involves abnormal renal development with metanephric blastema formation without tubular or glomerular differentiation. Most are solitary, but bilateral involvement is possible. Histologically, the tumor has blastemal undifferentiated cells, stromal cells, and epithelial cells.
Sources: UpToDate; pathology.washington.edu
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