The aim of our study was to retrospectively evaluate whether maximum standardized uptake value (SUV max ), total lesion gylcolysis (TLG), or change therein using 18 F-FDG PET/CT performed before and after initial chemotherapy were indicators of patient outcome. Methods: Thirty-one consecutive patients who underwent 18 F-FDG PET/CT before and after chemotherapy, followed by tumor resection, were retrospectively reviewed. Univariate Cox regression was used to analyze for relationships between covariates of interest (SUV max before and after chemotherapy, change in SUV max , TLG before and after chemotherapy, change in TLG, and tumor necrosis) and progression-free and overall survival. Logistic regression was used to evaluate tumor necrosis. Results: High SUV max before and after chemotherapy (P 5 0.008 and P 5 0.009, respectively) was associated with worse progression-free survival. The cut point for SUV max before chemotherapy was greater than 15 g/mL* (P 5 0.015), and after chemotherapy it was greater than 5 g/mL* (P 5 0.006), as measured at our institution and using lean body mass. Increase in TLG after chemotherapy was associated with worse progressionfree survival (P 5 0.016). High SUV max after chemotherapy was associated with poor overall survival (P 5 0.035). The cut point was above the median of 3.3 g/mL* (P 5 0.043). High TLG before chemotherapy was associated with poor overall survival (P 5 0.021). Good overall and progression-free survival was associated with a tumor necrosis greater than 90% (P 5 0.018 and 0.08, respectively). A tumor necrosis greater than 90% was most strongly associated with a decrease in SUV max (P 5 0.015). Conclusion: 18 F-FDG PET/CT can be used as a prognostic indicator for progression-free survival, overall survival, and tumor necrosis in osteosarcoma.
HIPEC may be an effective therapy for children and young adults with DSRCT. Patients with DSRCT require complete cytoreduction before HIPEC to optimize outcome. Patients with DSRCT and disease outside the abdomen at the time of surgery do not benefit from HIPEC.
Clinicians and radiologists should be aware of the radiographic manifestations of HLH, which are nonspecific and overlap with infectious, inflammatory and neoplastic disorders. Findings in the chest (similar to acute respiratory distress syndrome) and abdomen may progress rapidly and then regress with institution of appropriate anti-HLH therapy. CNS findings may be progressive. In some infants, initial imaging findings may mimic nonaccidental trauma.
Cytoreductive surgery and HIPEC with a programmatic approach for patients 3-21 years of age is unique. The best outcome was experienced by patients with desmoplastic small round cell tumor and those with complete cytoreduction. Complete cytoreduction for patients without disease outside the abdominal cavity at the time of surgery affords the best outcome.
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