7 Neuroblastoma Flashcards

1
Q

What is the second most common solid tumor for peds?

A

Neuroblastoma

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2
Q

What is the most common primary tumor site for neuroblastoma? what are the next 2?

A

adrenal gland (50%) then abdomen, then thoracic.

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3
Q

Average age for neuroblastoma diagnosis?

A

2 years. most common for infants and newborns.

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4
Q

What are the 3 diseases in which there is an increased incidence of neuroblastoma?

A

Ondine’s curse (central hypoventilation), NF1, and hirschsprungs

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5
Q

Why is NBL ‘the silent tumor”?

A

Appear well usually, 60% already metastatic and can present with immediate life threatening emergencies due to tumor burden.

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6
Q

What is horner’s syndrome?

A

rare - damaged sympathetic nerve makes one eye droop, pupils constric and decrease in sweat production

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7
Q

What is ‘raccoon eyes’?

A

bruising and swelling around eyes, typical for advanced metastatic disease

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8
Q

TO diagnose NBL what needs to be in the urine?

A

increased HVA and VMA (95% of cases)

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9
Q

What are urine catecholamines

A

made by the brain and adrenal gland, in 90% of cases it is elevated wit hNBL

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10
Q

When diagnosing NBL, the bone marrow biopsy will be…

A

bilateral

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11
Q

With NBL, elevated ferritin and LDH mean…

A

advanced disease/poor outcome

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12
Q

Prognosis is best at what age

A

365 days or less

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13
Q

In general, what are the stages of NBL? 1-4

A

1,2A,2B - localized
3- unresectable, across midline
4- primary with distant mets
4S - very specific, les than a year, liver, skin and marrow only.

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14
Q

What is MYCN amplification?

A

MYCN is a gene that regulates cell growth- more than 10 copies is associated with a more aggressive tumor

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15
Q

How to treat NBL in patients under 3 months?

A

observe only. if a large tumor, may resect. excellent survival.

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16
Q

What is the 5 year survival for intermediate risk NBL?

A

8085%

17
Q

What is the treatment for NBL high risk patients?

A

chemo first to increase chance of GTR, then surgery, then HPCT and radiation after.

18
Q

What percentage of NBL patient relapse after high risk therapy?

A

50-70%

19
Q

5% of patients can have symptoms from the secretion of VIP (vasoactive intestinal polypeptide) by he tumor. What are the symptoms?

A

Intractable diarrhea, hypokalemia.