Lisa J. States

In children with cancer predisposition syndromes, familiarity with syndrome-specific protocols and characteristic tumor imaging features, adoption of child-centered imaging strategies, and a coordinated multidisciplinary approach are essential to optimize outcomes and minimize screening-related harm.

Synovial sarcoma is the most common nonrhabdomyosarcoma soft tissue sarcoma in children and adolescents, typically associated with more favorable outcomes compared with adults. However, the role of ¹⁸ F-fluorodeoxyglucose ( ¹⁸ F-FDG) PET imaging in pediatric synovial sarcoma is underexplored. This study investigates the prognostic value of pretreatment maximum standardized uptake value (SUVmax) on ¹⁸ F-FDG PET imaging in 15 pediatric patients with synovial sarcoma who underwent PET/CT or PET/MRI and MRI. SUVmax of the primary tumors were analyzed for their association with overall survival (OS) and progression-free survival (PFS). The median SUVmax was 4.64 (range: 1.66 to 12.46). Although higher SUVmax tended to be associated with poorer OS and PFS, no statistically significant associations were found. MRI findings also showed no significant association with outcomes. Our result highlights that tumors with low SUVmax can still exhibit aggressive behavior. High-grade tumors and those larger than 5 cm were associated with higher SUVmax, and were more frequently located in axial regions. These findings suggest integrating SUVmax with tumor grade, tumor size and location may enhance risk stratification and support more individualized treatment strategies in pediatric synovial sarcoma.

Pediatric neuroendocrine tumors (NETs) and neuroblastomas are rare malignancies with poor outcomes when metastatic. Limited treatment options are currently available for pediatric NETs. Recently, radioligand therapy (RLT) consisting of a radionuclide attached to a ligand, such as [¹⁷⁷Lu]Lu-DOTA-TATE, has been approved for the treatment of NETs in adolescents aged ≥12 years. Although long-term safety of RLT in adolescents and other pediatric patients needs to be further investigated, data from large adult studies and early pediatric studies suggest feasibility and low toxicity. Future research is needed to assess potential combinations of RLTs with conventional chemotherapy and radiation sensitizers in order to optimize the treatment for pediatric patients with NETs. This review highlights the current status and future directions for RLTs as theranostics for pediatric patients with NETs and neuroblastomas.

Supplementary Table 1 shows non-malignant findings identified on WBMRI screening in our patient cohort with corresponding patient IDs to cancer diagnoses identified on WBMRI screening.

Supplementary Table 2 outlines prior studies of initial WBMRI screening in LFS cohorts both in pediatric and adult patient populations including length of the study, number of patients with findings, and number of cancers detected.

PET/MRI is a highly versatile and effective imaging modality in pediatric populations that requires dedicated study preparation, optimized imaging protocols, and structured interpretation to reach its full potential.

Perspectives and Understanding of Radiation Risks in CPS: A Practitioner Survey

Children with cancer predisposition syndromes have an increased risk of developing certain cancers. Surveillance imaging plays an increasingly important role in the management of these children. The approach to imaging must account for several factors, including age of the child, onset and frequency of imaging, whether sedation is needed, and the types of tumors associated with a particular syndrome. Consideration must also be given to the potential risks and benefits associated with a given imaging technique. Whole-body MRI offers many advantages, including a comprehensive examination without ionizing radiation. Other techniques, such as CT, involve low doses of ionizing radiation but may be appropriate depending on the clinical circumstance. This article offers an overview of available imaging techniques along with potential strategies to guide the discussion with patients and families when deciding on the most appropriate surveillance imaging approach.