A 2-year-old child has an abdominal mass that crosses the midline. Ultrasound and CT show a large retroperitoneal mass with calcifications. Urine VMA and HVA are markedly elevated. This is neuroblastoma. The international staging system feature that most strongly predicts prognosis is:
- A MYCN amplification — single most powerful adverse biological prognostic marker ✓
- B Age at diagnosis — age <18 months at diagnosis is a favorable prognostic factor
- C Tumor size alone
- D Urine VMA level
Correct answer: A. MYCN amplification — single most powerful adverse biological prognostic marker
Explanation
MYCN (N-Myc proto-oncogene) amplification (>10 copies per diploid genome) is the single most powerful adverse prognostic marker in neuroblastoma, predicting aggressive behavior, rapid progression, and poor outcome regardless of stage. It is found in about 20% of neuroblastomas. Age <18 months is also favorable (higher likelihood of spontaneous maturation), and histology (Shimada classification) and segmental chromosomal aberrations contribute to risk stratification. But MYCN amplification is the landmark molecular prognostic factor.
Reference: Ghai Essential Pediatrics, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.