Neuroblastoma

Question 1

What is Neuroblastoma

Answer 1

Cancer of immature nerve cells in various parts of body

Question 2

Classic Signs

Answer 2

Blueberry muffin sign: Non tender blue skin nodules
Racoon eyes: Orbital mets with proptosis
Pepper Synd: Massive liver involvement
Hutchinson Syd: Bone pain, skull masses, refusal to walk

Question 3

Median Age & Common Race

Answer 3

17-22 months (much younger than Wilms)
Caucasians (African American in Wilms)

Question 4

Pathognomic features

Answer 4

Calcifications >85%
Very ill patients
Never Mets to lungs

Question 5

Labs

Answer 5

Blood Catecholamines
Urine Catecholamines: VMA, HVA, E/NE, Dopamine
CBC, CMP, LDH, LFT

Question 6

H & P

Question 7

Imaging

Answer 7

CT CAP
MRI with contrast abdomen, liver , spine
MIBG > 90% pts have it; if -ve then PET-CT
MUGA/ECHO for chemotherapy

Question 8

Bad prognostic factor

Answer 8

MYCN amplification

Question 9

Biopsy

Answer 9

Biopsy if high risk

Question 10

Histopathology

Answer 10

Homer wright pseudorosettes in 20% cases

Question 11

Shimada Classification

Answer 11

Based on following factors
Schwann cell stroma (rich = better)
Age (<18mnths better)
degree of differentiation
Mitotic index
Nodular vs Diffuse pattern ( Diffuse is better)

Classified into favorable vs unfavorable

Question 12

Types of NB

Answer 12

1) Neuroblastoma
2) Ganglioneuroblastoma
3) Ganglioneuroma = schwann cell poor, rich, dominant

Question 13

Pre-OP Staging

Answer 13

Stage
L1: Localized
L2: Locally invasive
M: Mets except below MS
MS: Mets to skin, liver, bone marrow, age <18mnths

Question 14

Post-OP Staging

Answer 14

1: R0, R1, localized tumor, LN-ve
2A: R2
2B: R2 and or ipsilateral LN
3: Midline or contralateral primary or LN
4: Mets except below 4S
4S: Mets only to skin, liver, bone marrow, <12 months age

Question 15

Who are high risk patients

Answer 15

Any 3+ stage
Stage 4 >18 months
Stage 4 12-18 months
unfavorable histology
MYCN amplification

Question 16

Treatment Paradigm

Answer 16

MIBG scan + Biopsy
Induction Chemotherapy x 5 months
Max Safe resection
MIBG Scan
Myeloablative chemo + BMT
XRT by day 42 post BMT
Maintenace Chemotherapy

Question 17

Chemotherapy

Answer 17

CAPE TV

C: Cyclophosphamide
A: Adriamycin
P: Cisplatin
E: Etoposide
T: Topotecan
V: Vincristine

Question 18

Maintenance Chemotherapy

Answer 18

Cis-Retinoic acid
IL2
Anti GD2 therapies

Question 19

Surgery

Answer 19

Max Safe resection

Question 20

BMT

Answer 20

Myeloablative chemotherapy followed by BMT x 2 tandem
Usually chemo prior sometimes can use TBI

Question 21

Radiation

Question 22

Radiation Volumes

Answer 22

GTV1 = tumor volume = Post chemo pre sx
CTV 1 = GTV 1 + 1cm = 2160cGy in 12 fractions
Residual disease = Boost = 36Gy

Question 23

Toxicity

Answer 23

Growth disturbances
Endocrinopathies
Cardiac dysfunction
Bladder dysfunction
Secondary malignancy

Question 24

3 Yr OS

Answer 24

Low risk = 95%
Intermediate risk = 90%
High risk = 50-70%

Question 25

5Yr OS for M1

Answer 25

50%

Question 26

5 YR OS for High risk

Answer 26

EFS=70%