Part 11: Peds Tumor- Neuroblastoma

What is neuroblastoma?

Originates from neural crest cells that normally develop into the adrenal medulla

def. triangular shaped body that covers the superior surface of each kidney

term. adrenal glands

Neuroblastoma epidemiology

• 2nd most common pediatric solid tumor 👑

• Median Age: 2

• Rare Over Age: 7 (like Wilms)

Where does Neuroblastoma occur?

anywhere along the sympathetic nerve chain, but usually on the adrenal glands

Etiology for neuroblastoma

1. Linked to abnormal maturation of fetal neural crest cells

2. hereditary

3. 1-3% incidence at autopsy of infants that died from other causes, so may seem to spontaneously regress

Presentation of neuroblastoma (symptoms)

1. Abdominal Mass

2. Pain

3. Malaise (lethargy)

4. Horner’s Syndrome

Horner’s Syndrome

1. ptosis

2. miosis

3. anhidrosis

Diagnosis of Neuroblastoma

1. Urinalysis (from diapers) - detects byproducts of excess hormone production

2. Hypotension (high blood pressure)

Neuroblastoma treatment

1. surgery

2. RTT

3. Chemo

1. Surgery= best hope for cure

2. RTT for

   1. Adjunct: late stage (3&4)

   2. Palliation

3. Chemo: Adjunct for all stages: multi-agent chemo

Dose for RTT to treat chemo when it is used as :

1. Adjunct for late stages:

2. Palliation

1. Adjunct for late stages: 20-30 Gy to tumor bed

2. Palliation: 1000 cGy

fyi: 2 adrenal glands

Why is multi-agent chemo used for neuroblastoma?

to reduce the risk of late cancer production

5 year survival for neuroblastoma

• before age of 1y: 70%

• after age of 1y: 30%

What is the most important prognostic factor for neuroblastoma?

Age

patients over 18 months have a 70% chance of having mets

True or False: Neuroblastoma is the most common pediatric solid tumor

False

1st: Brain

2nd: neuroblastoma