Diagnostic accuracy of imaging approaches for early tumor detection in children with Li-Fraumeni syndrome
Issued Date
2022-06-01
Resource Type
ISSN
03010449
eISSN
14321998
Scopus ID
2-s2.0-85127717141
Pubmed ID
35391548
Journal Title
Pediatric Radiology
Volume
52
Issue
7
Start Page
1283
End Page
1295
Rights Holder(s)
SCOPUS
Bibliographic Citation
Pediatric Radiology Vol.52 No.7 (2022) , 1283-1295
Suggested Citation
Tewattanarat N., Junhasavasdikul T., Panwar S., Joshi S.D., Abadeh A., Greer M.L.C., Goldenberg A., Zheng G., Villani A., Malkin D., Doria A.S. Diagnostic accuracy of imaging approaches for early tumor detection in children with Li-Fraumeni syndrome. Pediatric Radiology Vol.52 No.7 (2022) , 1283-1295. 1295. doi:10.1007/s00247-022-05296-9 Retrieved from: https://repository.li.mahidol.ac.th/handle/123456789/85841
Title
Diagnostic accuracy of imaging approaches for early tumor detection in children with Li-Fraumeni syndrome
Other Contributor(s)
Abstract
Background: The Toronto protocol for cancer surveillance in children with Li-Fraumeni syndrome has been adopted worldwide. Objective: To assess the diagnostic accuracy of the imaging used in this protocol. Materials and methods: We conducted a blinded retrospective review of imaging modalities in 31 pediatric patients. We compared imaging findings with the reference standards, which consisted of (1) histopathological diagnosis, (2) corresponding dedicated imaging or subsequent surveillance imaging or (3) clinical outcomes. We individually analyzed each modality’s diagnostic performance for cancer detection and assessed it on a per-study basis for chest and abdominal regional whole-body MRI (n=115 each), brain MRI (n=101) and abdominal/pelvic US (n=292), and on a per-lesion basis for skeleton/soft tissues on whole-body MRI (n=140). Results: Of 763 studies/lesions, approximately 80% had reference standards that identified 4 (0.7%) true-positive, 523 (85.3%) true-negative, 5 (0.8%) false-positive, 3 (0.5%) false-negative and 78 (12.7%) indeterminate results. There were 3 true-positives on whole-body MRI and 1 true-positive on brain MRI as well as 3 false-negatives on whole-body MRI. Sensitivities and specificities of tumor diagnosis using a worst-case scenario analysis were, respectively, 40.0% (95% confidence interval [CI]: 7.3%, 83.0%) and 38.2% (95% CI: 29.2%, 48.0%) for skeleton/soft tissues on whole-body MRI; sensitivity non-available and 97.8% (95% CI: 91.4%, 99.6%) for chest regional whole-body MRI; 100.0% (95% CI: 5.5%, 100.0%) and 96.8% (95% CI: 90.2%, 99.2%) for abdominal regional whole-body MRI; sensitivity non-available and 98.3% (95% CI: 95.3, 99.4) for abdominal/pelvic US; and 50.0% (95% CI: 2.7%, 97.3%) and 93.8% (95% CI: 85.6%, 97.7%) for brain MRI. Conclusion: Considerations for optimizing imaging protocol, defining criteria for abnormalities, developing a structured reporting system, and practicing consensus double-reading may enhance the diagnostic accuracy for tumor surveillance.