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Question 356 · LEK · Neuroblastoma

A 4-year-old child with an abdominal lump on an ultrasound scan is admitted into the Pediatric Emergency Department. The lump is localized in the plan view of the right kidney, size: 8x7x7 cm. It is not encapsulated and infiltrates the adjacent structures. There are metastases to the local lymph nodes. A physical examination has revealed edema of the temporozygomatic region with bilaterally visible periorbital hematomas. Indicate the true statement(s) regarding the above clinical scenario (diagnosis and prognosis):

1) one of the steps to ensure proper diagnostic workup is to order a 24-hour urine collection to carry out tests e.g. for metanephrines and vanillylmandelic acid;
2) a biopsy of the lump is contraindicated due to worsening of the prognosis;
3) the presence of the N-MYC amplification in the tumor tissues improves the prognosis;
4) the disease stage as described above is referred to as IVS;
5) the patient’s prognosis is good (a 5-year survival in >70% of cases).
The correct answer is:
  1. A. only 1.
  2. B. 1,2.
  3. C. 1,2,3.
  4. D. only 2.
  5. E. all the above.
Hint

Periorbital ecchymoses with a suprarenal mass point to a specific tumour; consider age-dependent staging and molecular prognostic markers.

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