Which one of the listed individuals is most likely to have a tumor that has a histologic appearance characterized by undifferentiated mesenchymal cells with immature tubules and aboive glomerular formation?
Correct Answer: An 8-month-old infant with an abdominal mass and normal urinary vanillylmandelic acid (VMA)
Description: Malignant tumors of the kidney in children are called nephroblastomas (Wilms tumor) and histologically reveal a combination of metanephric blastema, undifferentiated mesenchymal cells, and immature tubule or glomerular formation. Children present with an enlarging abdominal mass that, in contrast to adrenal neuroblastoma, is associated with normal urinary vanillylmandelic acid (VMA) levels. Deletions involving WT1, located on chromosome 11, are associated with the development of Wilms tumor (nephroblastoma). Several syndromes are associated with genetic deletions of WT1 that lead to an increased incidence of Wilms tumor. These include WAGR syndrome (characterized by aniridia, genital abnormalities, and mental retardation) and Denys-Drash syndrome (characterized by gonadal dysgenesis and renal failure). Deletions involving a second Wilms tumor gene (WT2) are associated with Beckwith-Wiedemann syndrome (characterized by hemihyperophy, renal medullary cysts, and adrenal cytomegaly). Reference: Robbins & Cotran Pathologic Basis of Disease, 9edition
Category:
Pathology
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