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Pathology · USMLE Step 1

USMLE Pathology: Pediatric Tumors

Classic pediatric tumor morphology (Wilms tumor, neuroblastoma, retinoblastoma) distinct from the congenital-anomaly focus of the Embryology decks.

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What is another name for Wilms tumor?
Nephroblastoma
What is the most common renal malignancy of childhood?
Wilms tumor (nephroblastoma)
What is the peak age of onset for Wilms tumor?
2 to 5 years old
What embryologic tissue does Wilms tumor arise from?
Metanephric blastema (nephrogenic blastema)
What are the three classic histologic components of Wilms tumor?
Blastemal, stromal, and epithelial components (triphasic)
Mutation of which gene, located on chromosome 11p13, causes Wilms tumor?
WT1
What does the acronym WAGR syndrome stand for?
Wilms tumor, Aniridia, Genitourinary anomalies, mental Retardation
What is the classic triad of Denys-Drash syndrome?
Nephropathy, Wilms tumor, and male pseudohermaphroditism
Which overgrowth syndrome, linked to chromosome 11p15, increases the risk of Wilms tumor?
Beckwith-Wiedemann syndrome
How does a Wilms tumor classically present on abdominal exam?
A large, unilateral abdominal or flank mass that does not cross the midline
What histologic feature of Wilms tumor indicates an unfavorable prognosis?
Anaplasia
What is the most common extracranial solid tumor of childhood?
Neuroblastoma
What is the most common malignant tumor diagnosed in infants under 1 year old?
Neuroblastoma
What embryologic cell type gives rise to neuroblastoma?
Neural crest cells
What is the most common primary site of neuroblastoma?
Adrenal medulla

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