The utility of 68Ga-DOTATATE PET/CT in localizing primary/metastatic pheochromocytoma and paraganglioma in children and adolescents - a single-center experience

Objectives: Pediatric pheochromocytoma and paraganglioma (PPGL) are rare tumors with limited data on the diagnostic performance of Ga-68-DOTA(0)-Tyr(3)-octreotate positron emission tomography-computed tomography (Ga-68-DOTATATE PET/CT). We have described our experience of Ga-68-DOTATATE PET/CT in overall and von Hippel Lindau (VHL)-associated pediatric PPGL and compared its sensitivity with that of I-131-meta-iodobenzyl-guanidine (I-131-MIBG), F-18-fluorodeoxyglucose PET/CT (F-18-FDG PET/CT), and contrast-enhanced CT (CECT). Methods: Retrospective evaluation of consecutive PPGL patients (age: <= 20 years), who had undergone at least one functional imaging [I-131-MIBG, F-18-FDG PET/CT, and/or Ga-68-DOTATATE PET/CT], was done. Composite of anatomical and all the performed functional imaging scans, image comparator (IC), was considered as the gold standard for sensitivity analysis. Results: In a cohort of 32 patients (16 males, age at diagnosis: 16.4 +/- 2.68 years), lesion-wise sensitivity of Ga-68-DOTATATE PET/CT (95%) was higher than that of both F-18-FDG-PET/CT (80%, p=0.027) and I-131-MIBG (65%, p=0.0004) for overall lesions, than that of F-18-FDG-PET/CT (100 vs. 67%, p=0.017) for primary PPG, and than that of I-131-MIBG (93 vs. 42%, p=0.0001) for metastases. In the VHL (n=14), subgroup, Ga-68-DOTATATE PET/CT had higher lesion-wise sensitivity (100%) compared to F-18-FDG PET/CT (74%, p=0.045) and I-131-MIBG (64%, p=0.0145). Conclusions: In our pediatric PPGL cohort, overall lesion-wise sensitivity of Ga-68-DOTATATE PET/CT was higher than that of F-18-FDG PET/CT and I-131-MIBG scintigraphy. Hence, we recommend Ga-68-DOTATATE PET/CT as the preferred modality in pediatric PPGL. Ga-68-DOTATATE PET/CT may evolve as a preferred imaging modality for disease surveillance in VHL.