What is the second most common solid tumor for peds?
What is the most common primary tumor site for neuroblastoma? what are the next 2?
adrenal gland (50%) then abdomen, then thoracic.
Average age for neuroblastoma diagnosis?
2 years. most common for infants and newborns.
What are the 3 diseases in which there is an increased incidence of neuroblastoma?
Ondine’s curse (central hypoventilation), NF1, and hirschsprungs
Why is NBL ‘the silent tumor”?
Appear well usually, 60% already metastatic and can present with immediate life threatening emergencies due to tumor burden.
What is horner’s syndrome?
rare - damaged sympathetic nerve makes one eye droop, pupils constric and decrease in sweat production
What is ‘raccoon eyes’?
bruising and swelling around eyes, typical for advanced metastatic disease
TO diagnose NBL what needs to be in the urine?
increased HVA and VMA (95% of cases)
What are urine catecholamines
made by the brain and adrenal gland, in 90% of cases it is elevated wit hNBL
When diagnosing NBL, the bone marrow biopsy will be…
With NBL, elevated ferritin and LDH mean…
advanced disease/poor outcome
Prognosis is best at what age
365 days or less
In general, what are the stages of NBL? 1-4
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.
What is MYCN amplification?
MYCN is a gene that regulates cell growth- more than 10 copies is associated with a more aggressive tumor
How to treat NBL in patients under 3 months?
observe only. if a large tumor, may resect. excellent survival.
What is the 5 year survival for intermediate risk NBL?
What is the treatment for NBL high risk patients?
chemo first to increase chance of GTR, then surgery, then HPCT and radiation after.
What percentage of NBL patient relapse after high risk therapy?
5% of patients can have symptoms from the secretion of VIP (vasoactive intestinal polypeptide) by he tumor. What are the symptoms?
Intractable diarrhea, hypokalemia.