Neuroblastoma

Question 1

Image defined risk factors(11)

Answer 1

Ipsilateral tumours in two body compartments
Encroachment on the base of the skull
Compression of trachea or main bronchi
Encasing brachial plexus
Spinal cord encroachment >1/3 or obliteration of leptomeninges at that region or abN SC signal
Encasement of major vessels
Infiltration of adjacent organs
Involvement beyond the renal pedicle
Extension passed the sciatic notch
Involvement of T9-T12 vertebrae
Infiltrating the ligaments of the liver

Question 2

INRGSS high risk classification

Answer 2

Any MYC amplified tumor
Patients with metastatic disease > 18 months
Infants with MS disease And 11q abberation

Question 3

INRGSS low risk classification

Answer 3

L1 tumors not MYC amplified
L2 tumors in patients < 18 months not MYC amplified or with 11q alteration
L2 tumors in patients > 18 mo with favourable histology, MYC NA and no 11q abberation
MS in patients < 18 months not MYC amplified or with 11q alteration
M tumors in patients < 18 months with hyper diploids and MYC NA

Question 4

Syndromes associated with NBL

Answer 4

Familial:
ALK
PHOX2B- congenital central hypoventation syndrome-

Others:
NF-1
Noonan
Li fraumeni
Beck width Weidmann

Question 5

INRG intermediate risk

Answer 5

L2 tumors in > 18 mo without MYC or 11q abberation
L2 tumors in those < 18 mon with 11 q abberation
M stage in those < 18 mo with diploid tumors