Whole‐body magnetic resonance imaging as surveillance for subsequent malignancies in preadolescent, adolescent, and young adult survivors of germline retinoblastoma: An update

Abstract

Background: Germline retinoblastoma (Rb) survivors are at lifelong risk for developing subsequent malignancies (SMNs). Optimal surveillance modalities are needed to detect SMN at an early stage in this high-risk cohort. We investigated the use of rapid whole-body magnetic resonance imaging (WB-MRI) as a noninvasive screening modality in this cohort.Procedure: WB-MRI was performed in asymptomatic preadolescent, adolescent, or young adult survivors of germline Rb from February 1, 2008 to December 31, 2018 at a tertiary cancer center.We calculated sensitivity and specificity of WB-MRI and rate of false-positive findings requiring additional evaluation. Results:Overall, 110 WB-MRI were performed in 47 germline Rb survivors (51% female; median age at initial WB-MRI: 15.5 years [range 8-25.3]). Patients received 1-10 annual WB-MRI examinations (median: two). Thirteen patients had an abnormal WB-MRI; three findings were deemed to be likely benign and were not evaluated further. Ten patients required dedicated imaging and three required biopsy; two patients were diagnosed with localized high-grade osteosarcoma, while the other eight had benign findings. One patient was diagnosed with secondary osteosarcoma 3 months after normal WB-MRI. In total, there were 96 true negatives, 11 false positives, two true positives, and one false negative. The sensitivity of WB-MRI in this cohort was 66.7% (95% confidence interval [CI], 14.2-96.0) and the specificity was 89.7% (95% CI, 83.6-93.7). Conclusions:Based on our 10-year experience, surveillance WB-MRI appears to have limited utility as a surveillance modality for SMN in germline Rb survivors. Alternate screening modalities should be investigated.

Incidence and Mortality of Second Primary Cancers in Danish Patients With Retinoblastoma, 1943-2013

“… 6 From the results of a recent pilot study, whole-body magnetic resonance imaging appeared to have limited sensitivity (67%) in detecting SPCs in survivors of heritable retinoblastoma, and any association with mortality, as well as whether the benefits outweigh the potential risks (eg, emotional stress and cost), are still unclear. 36 …”

“…However, annual oncologic workup is now routine at several retinoblastoma centers, and whole-body magnetic resonance imaging at regular intervals has been suggested . From the results of a recent pilot study, whole-body magnetic resonance imaging appeared to have limited sensitivity (67%) in detecting SPCs in survivors of heritable retinoblastoma, and any association with mortality, as well as whether the benefits outweigh the potential risks (eg, emotional stress and cost), are still unclear …”

Does the Clinical Presentation of Secondary Osteosarcoma in Patients Who Survive Retinoblastoma Differ From That of Conventional Osteosarcoma and How Do We Detect Them?

“…Other than bone scan, whole-body MRI can be considered an alternative modality for regular surveillance. This modality is known to show modest sensitivity for detecting secondary malignancy [11,12]. Regarding the interval of imaging, cost-effectiveness and concerns about radiation exposure should be considered [2].…”

Bildgebung des Retinoblastoms

“…Weitere wichtige Indikationen zur MRT-Bildgebung von Kindern mit Retinoblastom sind Verlaufskontrollen des intrakraniellen Befunds bei hereditärem Retinoblastom. Ob ein systematisches MRT-Ganzkörper-Screening auf Sekundärmalignome bei Vorliegen eines Tumorprädispositionssyndroms erfolgen sollte, wird aktuell noch kontrovers diskutiert [ 20 ]; bei klinischen Symptomen sollte jedoch stets eine gezielte MRT-Abklärung der Region erfolgen, um Zweitmalignome nicht zu verpassen (Abb. 6 ).…”