title: Whole-body magnetic resonance imaging (WB-MRI) as surveillance for subsequent malignancies in survivors of hereditary retinoblastoma: a pilot study.
abstract: BACKGROUND
  Individuals with hereditary retinoblastoma (RB) are at very high risk of developing subsequent malignant neoplasms (SMNs) of which osteosarcoma (OS) is one of the most common. We hypothesized that annual surveillance using whole-body magnetic resonance imaging (WB-MRI) in asymptomatic survivors of hereditary RB would detect SMN of the bone and soft tissues at an early stage.
  
  
  METHODS
  Retrospective review of the results of a WB-MRI screening program in hereditary RB survivors from February 2008 to August 2012. The primary outcome was to determine the sensitivity and specificity of WB-MRI in detecting SMNs.
  
  
  RESULTS
  Twenty-five patients had at least one WB-MRI performed (range: 1-5). First WB-MRI was performed at a median age of 16 years (range: 8-25 years). WB-MRI detected new osseous abnormalities suspicious for malignancy in five patients: two were diagnosed with localized high-grade OS of the extremity and three were found to have benign osseous abnormalities after dedicated imaging (n = 5/5) and/or biopsy (n = 3/5). One patient was diagnosed with secondary OS 3 months after a normal screening WB-MRI exam. Among a total of 41 WB-MRI screening tests performed in survivors of hereditary RB, the sensitivity of detecting SMN was 66.7% and the specificity was 92.1%.
  
  
  CONCLUSIONS
  Preliminary results suggest that annual WB-MRI surveillance detects SMN in survivors of hereditary RB, but with modest sensitivity. Further study is needed to assess the performance of annual surveillance WB-MRIs and whether this modality decreases SMN-related mortality in RB survivors.
authors.affiliation: Department of Pediatrics, Memorial Sloan-Kettering Cancer Center, New York, New York.
authors.forename: Danielle Novetsky
authors.initials: DN
authors.lastname: Friedman
authors.forename: Eric
authors.initials: E
authors.lastname: Lis
authors.forename: Charles A
authors.initials: CA
authors.lastname: Sklar
authors.forename: Kevin C
authors.initials: KC
authors.lastname: Oeffinger
authors.forename: Marina
authors.initials: M
authors.lastname: Reppucci
authors.forename: Megan Harlan
authors.initials: MH
authors.lastname: Fleischut
authors.forename: Jasmine H
authors.initials: JH
authors.lastname: Francis
authors.forename: Brian
authors.initials: B
authors.lastname: Marr
authors.forename: David H
authors.initials: DH
authors.lastname: Abramson
authors.forename: Ira J
authors.initials: IJ
authors.lastname: Dunkel
mesh_terms: D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D008297:Male; D016609:Neoplasms, Second Primary / Q000000981:diagnostic imaging* / Q000628:therapy; D012516:Osteosarcoma / Q000000981:diagnostic imaging* / Q000628:therapy; D011859:Radiography; D012175:Retinoblastoma / Q000000981:diagnostic imaging* / Q000628:therapy; D012189:Retrospective Studies
keywords: pediatric oncology; retinoblastoma; screening; survivors; whole-body MRI