Supplementary Materials for “Real-World Experience of 40 Children with Pheochromocytoma/Paraganglioma: Algorithm–Practice Concordance in Resource-Limited Settings”

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Supplementary Figure 1. Anterior and left lateral maximum intensity projection (MIP) images from (A–B) the baseline 68Ga-DOTATATE PET/CT study, the baseline 18F-FDG PET/CT study (C–D), and post-operative 18F-FDG PET/CT study performed dueto biochemical recurrence following right adrenalectomy are presented (E–F). These sequential imaging studies allow assessment of somatostatin receptor expression (A–B) and glucose metabolism in adrenal lesions (C–F).(A–B) On the baseline 68Ga-DOTATATE PET/CT, the right adrenal mass (thick blue arrow) demonstrates mild somatostatin receptortype-2 expression, with uptake comparable to liver reference activity (SUVmax: 9.30). Although this finding indicates relatively lowreceptor density, it remains compatible with neuroendocrine tumor physiology.(C–D) The subsequent baseline 18F-FDG PET/CT study shows markedly increased FDG uptake in the right adrenal lesion (thickgreen arrow) (SUVmax: 13.06). Compared to the DOTA-TATE study, this high level of glucose metabolism suggests a metabolic–receptor mismatch in the biological behavior of the mass.(A–D) As an additional finding—much more prominent on FDG imaging—both baseline PET/CT studies demonstrate symmetric,intense uptake in the cervical, thoracic, abdominal, and pelvic adipose tissues, including the region of the left adrenal gland (thinbrown arrows). This pattern is consistent with brown adipose tissue (BAT) activation secondary to excessive catecholamine secretionand is characterized by diffuse, physiologic BAT distribution.(E–F) After the post-right adrenalectomy 18F-FDG PET/CT performed due to biochemical persistence, the previously observedbrown adipose tissue activation has completely resolved, consistent with removal of the catecholamine-secreting source. However,this study shows intense focal FDG uptake in the left adrenal gland (thick red arrow) (SUVmax: 15.99). This increased uptake raisedsuspicion for bilateral pheochromocytoma involving the contralateral adrenal gland as well.Supplementary Video 1. Preoperative (A−B) and postoperative (C) functionalimaging of the patientSupplementary Figure 2. Axial images from the 18F-FDG PET/CT study performed due to biochemical persistence following rightadrenalectomy (A–B) demonstrate a nodular lesion in the left adrenal gland, measuring approximately 1.5 cm in diameter, showingintense FDG uptake (thick red arrow). Metallic surgical clips are visualized in the right adrenalectomy bed (thick yellow arrow), and no pathologic FDG uptake is observed at the operative site. Because the right adrenal lesion on baseline studies had shown FDG avidity but low somatostatin receptor expression, direct imaging with 18F-FDG PET/CT was preferred.Supplementary Figure 3. Magnitude of elevation of plasma-free and 24-hour urinary metanephrine/normetanephrines expressed as multiples of ULN.Abbreviations: ULN: upper limit of normal, 24UF MN: 24-hour urinary metanephrine, 24UF NMN: 24-hour urinary normetanephrine, PF MN: plasma-free metanephrine, PF NMN: plasma-free normetanephrine.ULN values were defined according to age- and assay-specific reference ranges. Each dot represents an individual patient. The results were expressed as means±SE.Supplementary Table 1. Genetic Testing Strategies and Molecular Findings in the PPGL Study Cohort.Supplementary Table 2. Magnitude of elevation of plasma-free and 24-hour urinary normetanephrine/metanephrinesexpressed as multiples of ULN.Abbreviations: na: not available, ULN: upper limit of normal ULN values were defined according to age- and assay-specific reference ranges.Patients without detectable catecholamine elevation are highlighted in yellow.Patients with borderline elevation (<2× ULN) catecholamine elevation are highlighted in green.

Ce résumé expose les affirmations des auteurs. BNTIC ne l’interprète pas comme une validation indépendante des résultats.