Aggressive Pursuit of No Evidence of Disease Status in Hepatoblastoma Improves Survival: An Observational Study.

BACKGROUND: The utility of repeated surgical interventions in hepatoblastoma to achieve no evidence of disease (NED) is not well-defined. We examined the effect of aggressive pursuit of NED status on event-free (EFS) and overall survival (OS) in hepatoblastoma with subgroup analysis of high-risk patients. METHODS: Hospital records were queried for patients with hepatoblastoma from 2005 to 2021. Primary outcomes were OS and EFS stratified by risk and NED status. Group comparisons were performed using univariate analysis and simple logistic regression. Survival differences were compared with log-rank tests. RESULTS: Fifty consecutive patients with hepatoblastoma were treated. Forty-one (82%) were rendered NED. NED was inversely correlated with 5-year mortality (OR 0.006; CI 0.001-0.056; P < .01). Ten-year OS (P < .01) and EFS (P < .01) were improved by achieving NED. Ten-year OS was similar between 24 high-risk and 26 not high-risk patients when NED was attained (P = .83). Fourteen high-risk patients underwent a median of 2.5 pulmonary metastasectomies, 7 for unilateral disease, and 7 for bilateral, with a median of 4.5 nodules resected. Five high-risk patients relapsed, and three were salvaged. CONCLUSIONS: NED status is necessary for survival in hepatoblastoma. Repeated pulmonary metastasectomy and/or complex local control strategies to obtain NED can achieve long-term survival in high-risk patients. LEVEL OF EVIDENCE: Level III - Treatment Study - Retrospective Comparative Study.

Automated T(2) * measurements using supplementary field mapping to assess cardiac iron content.

PURPOSE: To develop and evaluate an algorithm that automatically identifies high-susceptibility areas and excludes them from T(2) * measurements in the left ventricle (LV) for myocardial iron measurements. MATERIALS AND METHODS: An autoregressive moving average (ARMA) model was implemented on multigradient echo scans of 24 patients (age range 3-45 years, 10 male/14 female). Voxels with relatively high susceptibility (>3 Hz/mm) were flagged and deselected from the T(2) * calculations for iron quantification. The mean, standard deviation, and coefficient of variation (CoV) of the ARMA-defined region were compared to the CoV of four distinct regions of the LV and the entire LV using a Student's t-test (α = 0.05). RESULTS: The CoV of T(2) * values obtained by the ARMA method are comparable with that in the interventricular septum (IS), where susceptibility was the lowest (CoV = 0.31). The ARMA method provides a greater area (51.9 ± 13.7% of the LV) to measure T(2) * than that using the IS alone (21.1 ± 3.4%, P < 0.0001). Areas where low susceptibility are measured corroborate with areas reported in previous studies that investigated T(2) * variations throughout the LV. CONCLUSION: An automated method to measure T(2) * relaxation in the LV with minimal effects from susceptibility has been developed. Variability is reduced while covering more regions for cardiac T2 * calculation.

PET-CT in pediatric Langerhans cell histiocytosis.

Our objective was to assess the utility of PET-CT in five pediatric patients with Langerhans cell histiocytosis (LCH) who underwent PET-CT imaging for clinical staging and determination of lesion activity at various stages of treatment and follow-up. PET-CT combines the anatomic detail of CT and the physiologic activity of (18)F-FDG imaging. We conclude that PET-CT information is clinically useful to evaluate disease activity and response to therapy and provides information that cannot be obtained from technetium 99m methylene diphosphonate bone scans or radiographs.