Pediatrics: Adrenal

Question 1

What is the most common extra-cranial solid childhood malignancy?

Answer 1

Neuroblastoma

Question 2

Neuroblastoma is most common in what age group?

Answer 2

Very young kids (can be born with it and 95% of cases occur before age 10)

Question 3

What are the most common locations for a neuroblastoma?

Answer 3

• Adrenal (35%)
• Retroperitoneal (30%)
• Posterior mediastinum (20%)
• Neck (5%)

Question 4

What imaging findings should you look for that would increase the stage of a neuroblastoma?

Answer 4

• Crossing the midline
• Contralateral positive nodes

Note: Both of these make it stage 3.

Question 5

Stage 4S neuroblastoma

Answer 5

A “special” stage of neuroblastoma that occurs in kids less than 1 year old where metastases are confined to the skin, liver and bone MARROW. It has an excellent prognosis.

Note: Mets to the bone MARROW not cortical bone.

Question 6

Stage 4S neuroblastoma has metastases confined to the…

Answer 6

• Skin
• Liver
• Bone marrow (NOT cortex)

Note: This has an excellent prognosis.

Question 7

Neuroblastoma are associated with

Answer 7

• NF-1
• Hirschsprungs
• DiGeorge
• Beckwith-Wiedemann

Note: Most are sporadic.

Question 8

Opsomyoclonus (dancing eyes, dancing feet)

Answer 8

A paraneoplastic syndrome associated with neuroblastoma

Question 9

Raccoon eyes in a pt with neuroblastoma…

Answer 9

Think orbital neuroblastoma metastases

Question 10

What is the best study to look for neuroblastoma mets?

Answer 10

MIBG nuclear imaging

Note: This is better than conventional bone scans.

Question 11

What type of bone mets are common in neuroblastoma?

Answer 11

Lytic bone mets, especially near metaphyses

Note: Sclerotic mets are rare.

Question 12

What lab abnormality is almost always present with neuroblastoma?

Answer 12

Elevated urine catecholamines (95%)

Question 13

Classic imaging appearance of neuroblastoma

Answer 13

Poorly marginated adrenal mass that cases vessels (doesn’t invade) and is almost always calcified (90%)

Question 14

What is the most common cause of an adrenal mass in a neonate?

Answer 14

Neonatal adrenal hemorrhage

Question 15

Neonatal adrenal hemorrhage is associated with…

Answer 15

Scrotal hemorrhage

Question 16

Which side is neonatal adrenal hemorrhage more common on?

Answer 16

The right (due to compression of the right adrenal gland between the liver and right kidney)

Question 17

How can you differentiate adrenal hemorrhage from neuroblastoma?

Answer 17

Ultrasound (adrenal hemorrhage should be hypoechoic and avascular)

Note: Can also differentiate on MRI (adrenal hemorrhage should be T2 hypointense and neuroblastoma T2 hyperintense).

Question 18

Left-sided neonatal adrenal hemorrhage…

Answer 18

Think renal vein thrombus (the left adrenal vein drains into the left renal vein)

Note: Neonatal adrenal hemorrhage is much more common on the right due to compression from the liver/kidney.

Question 19

Risk factor for neonatal adrenal hemorrhage

Answer 19

• Birth trauma
• Stress
• Maternal diabetes (due to increased risk for renal vein thrombosis)

Question 20

Next step: neonate hospitalized for unrelated cause is found to have an adrenal mass…

Answer 20

Suspect neonatal adrenal hemorrhage and get follow-up ultrasounds to ensure resolution (and exclude neuroblastoma)

Question 21

How can adrenal insufficiency help you narrow down an adrenal mass differential?

Answer 21

It can’t really: neuroblastoma, adrenal hemorrhage, and many other things can cause adrenal insufficiency