the (new) international neuroblastoma response criteria (inrc) · the international neuroblastoma...
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Neuroblastoma CTPM
• Co-Chairs: Rochelle Bagatell, Katherine Matthay, Julie Park, Dominique Valtuea-Couanet
• Executive Planning Committee: Penelope Brock, Frank Berthold, Susan Cohn, Wendy London, Andrew Pearson, Liz Scott, Nita Seibel, Wolf Lindwasser
• Date: April 12 – 13, 2012
Working Groups
• Primary Site: Kieran McHugh, Jed Nuchtern
• Metastatic Imaging: Ariane Boubacker, Kate Matthay
• Bone Marrow: Frank Berthold, Susan Burchill
• Ultra High Risk: John Maris, Gudrun Schleiermacher
• Phase 2: Julie Park, Dominique Valteau-Couanet
• expertise in oncology, pathology, nuclear medicine, radiology, surgery, biology and statistics (n=52)
INSS & INRG
INSS
• Stage I
• Stage II
• Stage III, crossing midline
• Stage IV
• Stage 4S
INRG
• L1 (non-invasive)
• L2 (infiltrative)
• Stage M
• Stage MS (age to 18 months)
MRI at diagnosis and routine FU
• No radiation
• Intraspinal
• Lymphadenopathy
• Marrow
• Chest wall
• Liver
• IDRFs
Pre-operative CT scan for Abdominal Neuroblastomas is Superior to MRI
for Safe Surgical Planning
SIOP Congress Kyoto 2018
K Burnand, G Barone, K McHugh, K Cross
Great Ormond Street Hospital for Children, London
Brodeur et al, JCO 1993
International Neuroblastoma Response Criteria (INRC)
developed to permit comparison of clinical trial results worldwide
Metastatic disease?
• MIBG, scoring systems
• FDG PET-CT if primary MIBG non-avid
• Bone marrow aspirates and trephines
• WBMRI?
12 anatomical segments
Extension score…..
SIOPEN method
The Curie scoring method
mIBG scoring
0 = no mIBG involvement
1 = 1 mIBG avid lesion
2 = > 1 mIBG avid lesion
3 = abnormal mIBG uptake in > 50% segment
INRC summary
• urinary catecholamine levels no longer recommended
• 4 samples - to assess bone marrow involvement (aspirates & trephines)
• % marrow infiltration calculated at all sample sites
• highest % tumour infiltration is used to assess response
• new category ‘minimal disease’ (0-5%)
Overall response
Response Definition
Complete Response (CR) CR at all involved sites
Partial Response (PR) PR in at least one site, with CR, PR, MD* (in bone marrow) at all
other sites. No progressive disease at any site.
Minor Response (MR) PR or CR in at least one site with SD at one or more sites. No
progressive disease.
Stable Disease (SD) SD in one site. No better than SD at any other site. No progressive
disease.
Progressive disease (PD) Progressive disease at any site.
MD* - minimal disease
The left one is the patient!
with permission of the parents
One of these twin girls was registered in
the HRNBL1 in 2005!