Review Question - QID 105543

The boy in this vignette most likely has a neuroblastoma, which presents classically under age four. A work up would have involved a CT scan of the abdomen showing calcifications and hemorrhages of the adrenal medulla.

Neuroblastomas are the most common tumor of the adrenal medulla in children, though they can occur anywhere along the sympathetic chain. Oncogenesis proceeds through the amplification of the N-myc oncogene, a nuclear transcription factor. Associated conditions at presentation include opsoclonus-myoclonus syndrome (myoclonic jerks and chaotic eye jerks). These tumors commonly have metastases to bone and skin.

Angstman et al. discuss the diagnosis of neuroblastoma. They report that it accounts for 8 percent of all pediatric tumors and 11 percent of all deaths from cancer in children. Symptoms typically appear before four years of age and rarely after 14 years of age.

Yalcin et al. performed a systematic meta-analysis regarding the treatment of neuroblastoma. They report that myeloablative therapy seems to improve event-free survival. With this in mind, no definitive conclusions can be made regarding adverse effects and quality of life, although possible higher levels of adverse effects should be suspected.

Illustration A shows a CT scan revealing calcifications and hemorrhages of the adrenal gland, as would be expected with a neuroblastoma.

Incorrect Answers:
Answer 1: Neuroblastomas, unlike pheochromocytomas in adults, do not present with symptoms of adrenergic hyperactivity.
Answer 3: Neuroblastomas typically present with elevated urine and serum catecholamines.
Answer 4: Neuroblastomas can present with opsoclonus-myoclonus syndrome, not absence seizures.
Answer 5: Neuroblastomas typically present as an abdominal mass, not a pelvic mass.