Neuroblastoma Flashcards
What is Neuroblastoma
Cancer of immature nerve cells in various parts of body
Classic Signs
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
Median Age & Common Race
17-22 months (much younger than Wilms)
Caucasians (African American in Wilms)
Pathognomic features
Calcifications >85%
Very ill patients
Never Mets to lungs
Labs
Blood Catecholamines
Urine Catecholamines: VMA, HVA, E/NE, Dopamine
CBC, CMP, LDH, LFT
H & P
Imaging
CT CAP
MRI with contrast abdomen, liver , spine
MIBG > 90% pts have it; if -ve then PET-CT
MUGA/ECHO for chemotherapy
Bad prognostic factor
MYCN amplification
Biopsy
Biopsy if high risk
Histopathology
Homer wright pseudorosettes in 20% cases
Shimada Classification
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
Types of NB
1) Neuroblastoma
2) Ganglioneuroblastoma
3) Ganglioneuroma = schwann cell poor, rich, dominant
Pre-OP Staging
Stage
L1: Localized
L2: Locally invasive
M: Mets except below MS
MS: Mets to skin, liver, bone marrow, age <18mnths
Post-OP Staging
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
Who are high risk patients
Any 3+ stage
Stage 4 >18 months
Stage 4 12-18 months
unfavorable histology
MYCN amplification
Treatment Paradigm
MIBG scan + Biopsy
Induction Chemotherapy x 5 months
Max Safe resection
MIBG Scan
Myeloablative chemo + BMT
XRT by day 42 post BMT
Maintenace Chemotherapy
Chemotherapy
CAPE TV
C: Cyclophosphamide
A: Adriamycin
P: Cisplatin
E: Etoposide
T: Topotecan
V: Vincristine
Maintenance Chemotherapy
Cis-Retinoic acid
IL2
Anti GD2 therapies
Surgery
Max Safe resection
BMT
Myeloablative chemotherapy followed by BMT x 2 tandem
Usually chemo prior sometimes can use TBI
Radiation
Radiation Volumes
GTV1 = tumor volume = Post chemo pre sx
CTV 1 = GTV 1 + 1cm = 2160cGy in 12 fractions
Residual disease = Boost = 36Gy
Toxicity
Growth disturbances
Endocrinopathies
Cardiac dysfunction
Bladder dysfunction
Secondary malignancy
3 Yr OS
Low risk = 95%
Intermediate risk = 90%
High risk = 50-70%
5Yr OS for M1
50%
5 YR OS for High risk
EFS=70%
