Baseline Surveillance in Li-Fraumeni Syndrome Using Whole-Body Magnetic Resonance Imaging: A Meta-analysis. - PubMed - NCBI
JAMA Oncol. 2017 Aug 3. doi: 10.1001/jamaoncol.2017.1968. [Epub ahead of print]
Baseline Surveillance in Li-Fraumeni Syndrome Using Whole-Body Magnetic Resonance Imaging: A Meta-analysis.
Ballinger ML1,
Best A2,
Mai PL3,
Khincha PP3,
Loud JT3,
Peters JA3,
Achatz MI3,4,
Chojniak R4,
Balieiro da Costa A5,
Santiago KM6,
Garber J7,
O'Neill AF8,
Eeles RA9,
Evans DG10,
Bleiker E11,
Sonke GS12,
Ruijs M13,
Loo C14,
Schiffman J15,
Naumer A15,
Kohlmann W15,
Strong LC16,
Bojadzieva J16,
Malkin D17,18,
Rednam SP19,
Stoffel EM20,
Koeppe E20,
Weitzel JN21,
Slavin TP21,
Nehoray B21,
Robson M22,
Walsh M23,
Manelli L24,
Villani A17,
Thomas DM1,
Savage SA2.
Abstract
IMPORTANCE:
Guidelines for clinical management in Li-Fraumeni syndrome, a multiple-organ cancer predisposition condition, are limited. Whole-body magnetic resonance imaging (WBMRI) may play a role in surveillance of this high-risk population. OBJECTIVE:
To assess the clinical utility of WBMRI in germline TP53 mutation carriers at baseline. DATA SOURCES:
Clinical and research surveillance cohorts were identified through the Li-Fraumeni Exploration Research Consortium. STUDY SELECTION:
Cohorts that incorporated WBMRI for individuals with germline TP53 mutations from January 1, 2004, through October 1, 2016, were included. DATA EXTRACTION AND SYNTHESIS:
Data were extracted by investigators from each cohort independently and synthesized by 2 investigators. Random-effects meta-analysis methods were used to estimate proportions. MAIN OUTCOMES AND MEASURES:
The proportions of participants at baseline in whom a lesion was detected that required follow-up and in whom a new primary malignant neoplasm was detected. RESULTS:
A total of 578 participants (376 female [65.1%] and 202 male [34.9%]; mean [SD] age, 33.2 [17.1] years) from 13 cohorts in 6 countries were included in the analysis. Two hundred twenty-five lesions requiring clinical follow-up were detected by WBMRI in 173 participants. Sixty-one lesions were diagnosed in 54 individuals as benign or malignant neoplasms. Overall, 42 cancers were identified in 39 individuals, with 35 new localized cancers treated with curative intent. The overall estimated detection rate for new, localized primary cancers was 7% (95% CI, 5%-9%). CONCLUSIONS AND RELEVANCE:
These data suggest clinical utility of baseline WBMRI in TP53 germline mutation carriers and may form an integral part of baseline clinical risk management in this high-risk population.
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