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Retinoblastoma imaging update
H.J. Brisse, Paris, France
European
Retinoblastoma
Imaging
Collaboration
New trends
Treatment
Increasing use of conservative approaches:
• Increasing use of local chemotherapy (IA/IV)
• Dramatic decreased use of radiation therapy
 Decreased availability of pathological information
Imaging
• MRI is the reference techniques
• Major role in disease staging
• Increasing role in patients’ follow-up / screening
Institut Curie - HJ Brisse – Oncology & Nuclear Medicine – Retinoblastoma Update
IPR - Chicago – May 2016
Staging
Numerous RB staging systems
• Reese-Ellsworth (1969, eye preservation / RTh)
• ICIR (2005 International Classification for Intra-Ocular Retinoblastoma, COG,
eye preservation / ChemoTh)
• IRSS (2006, International Retinoblastoma Staging System)
• TNM (American Joint Committee on Cancer)
8th AJCC Cancer Staging Manuel
• Suitable for : eye preservation, metastatic risk, survival
• HR-MRI recommended for « cTNM »:
• Extra-scleral or optic nerve invasion (cT4a)
• Distant metastases, CNS metastases (cM1a, cM1b)
Institut Curie - HJ Brisse – Oncology & Nuclear Medicine – Retinoblastoma Update
IPR - Chicago – May 2016
macroscopic invasion
cT4b
cT4a
cT4a
ON prechiasmatic
ON orbital portion
Extra-scleral
Institut Curie - HJ Brisse – Oncology & Nuclear Medicine – Retinoblastoma Update
IPR - Chicago – May 2016
MRI and local staging
Pathological risk factors
• Massive choroidal involvement (pT3a)
• Retrolaminar optic nerve involvement & free margins (pT3b)
• Orbital invasion (pT4)
Choroid
PLON
Institut Curie - HJ Brisse – Oncology & Nuclear Medicine – Retinoblastoma Update
Lamina
cribrosa
IPR - Chicago – May 2016
Need for high-resolution MRI
Imaging guidelines
• General anaesthesia
• Orbits:
• 1.5 T & surface coil / 3T & head coil
• SW = 2 mm, Pixel < 0.5 x 0.5 mm
• T2 (SE, CISS)
• T1 SE before / after Gd (+/- FS)
• Axial & sagittal
• Brain:
• T2 axial or sagittal
• T1 Gad
de Graaf P et al. Guidelines for imaging retinoblastoma. Ped Rad 2012
Sirin S et al. High-resolution MRI using orbit surface coils Neuroradiology 2015
Galluzzi P et al. Is CT Still Useful in the Study Protocol of Retinoblastoma? AJNR 2009
Rodjan F et al. Detection of calcifications in retinoblastoma using gradient-echo MR . AJNR 2015
Institut Curie - HJ Brisse – Oncology & Nuclear Medicine – Retinoblastoma Update
IPR - Chicago – May 2016
HR-MRI and microscopic ON invasion
1.4 mm
1.7 mm
MR accuracy / pathology ?
• High specificity (~ 95%) and NPV (~ 90%) for post-laminar invasion
• Still low sensitivity (~ 59%) and PPV (~ 50%)
• Strong association with tumor volume / size ( < 14.4 mm or > 19.2 mm)
Brisse HJ et al. Assessment of early-stage optic nerve invasion in retinoblastoma. Eur Radiol 2015
de Jong MC et al. Meta-analysis. Ophthalmology 2014
De Jong MC et al. Tumor Size and PLONI. Radiology 2015
Galluzzi P et al. MRI helps depict clinically undetectable risk factors. The neuroradiology journal 2015
Institut Curie - HJ Brisse – Oncology & Nuclear Medicine – Retinoblastoma Update
IPR - Chicago – May 2016
Tricks
Bulging lamina cribrosa
« double dots »
Brisse HJ et al. Assessment of early-stage optic nerve invasion in retinoblastoma. Eur Radiol 2015
Institut Curie - HJ Brisse – Oncology & Nuclear Medicine – Retinoblastoma Update
IPR - Chicago – May 2016
HR-MRI and choroidal invasion
Massive choroid invasion
Se ~ 74%, Sp ~ 72%
Superficial choroid invasion ...
de Jong MC et al. Diagnostic performance of magnetic resonance imaging and computed tomography for advanced
retinoblastoma: a systematic review and meta-analysis. Ophthalmology. 2014
Institut Curie - HJ Brisse – Oncology & Nuclear Medicine – Retinoblastoma Update
IPR - Chicago – May 2016
Imaging follow-up
during conservative treatment
FU by ocular fundus examination
Diagnosis
After intra-arterial chemoth
Strabism / myositis
ON non-specific enhancement
Institut Curie - HJ Brisse – Oncology & Nuclear Medicine – Retinoblastoma Update
IPR - Chicago – May 2016
Screening (1) : Trilateral RB
Pineal (or supra-sellar) PNET
Low incidence (~3.5% among hereditary RB)
Variable MR pattern (cystic / solid)
MR screening at diagnosis recommended
Normal pineal size charts recently published
Close FU of pineal cysts recommended
diagnosis
14 months later
Rodjan F et al. Trilateral retinoblastoma: neuroimaging characteristics and value of routine brain screening on admission. J Neurooncol 2012
de Jong MC et al. Trilateral retinoblastoma: a systematic review and meta-analysis. Lancet Oncol 2014
de Jong MC et al. The Incidence of Trilateral Retinoblastoma: A Systematic Review and Meta-Analysis. Am J Ophth 2015
Galluzzi P et al. MRI-based assessment of the pineal gland in a large population of children aged 0-5 years and comparison with
pineoblastoma: part I, the solid gland. Neuroradiology 2016
Sirin S et al. MRI-based assessment of the pineal gland in a large population of children aged 0-5 years and comparison with pineoblastoma:
part II, the cystic gland. Neuroradiology 2016
Institut Curie - HJ Brisse – Oncology & Nuclear Medicine – Retinoblastoma Update
IPR - Chicago – May 2016
Screening (2) : Second primary tumors
Bone & Soft tissue sarcomas
Survivors of hereditary RB (RB1 mutation)
EBRT: major risk factor
Benefit of imaging screening ?
Pilot study / WBMRI in hereditary RB survivors
Currently assessed in Europe for head & neck
after EBRT for hereditary RB
Rodjan F et al. Second cranio-facial malignancies in hereditary retinoblastoma survivors previously treated with radiation therapy: clinic
and radiologic characteristics and survival outcomes. Eur J Cancer 2013
Friedman DN et al. Whole-body magnetic resonance imaging (WB-MRI) as surveillance for subsequent malignancies in survivors of
hereditary retinoblastoma: a pilot study. Pediatr Blood Cancer 2014
Institut Curie - HJ Brisse – Oncology & Nuclear Medicine – Retinoblastoma Update
IPR - Chicago – May 2016
Sept 2009
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Jonas Castelijns, Pim De Graaf (Amsterdam, The Netherlands)
Paolo Galluzi (Sienna, Italy)
Sophia Göricke & Selma Sirin (Essen, Germany)
Philippe Maeder (Lausanne, Switzerland)
Hervé Brisse (Paris, France)
Institut Curie - HJ Brisse – Oncology & Nuclear Medicine – Retinoblastoma Update
European
Retinoblastoma
Imaging
Collaboration
IPR - Chicago – May 2016