Genotype-dependent Brown Adipose Tissue Activation in Patients with Pheochromocytoma and Paraganglioma.

T. Puar, A. van Berkel, M. Gotthardt, B. Havekes, A. Hermus, J. Lenders, W. vanMarken Lichtenbelt, Y. Xu, B. Brans and H. Timmers




Patients with pheochromocytomas and paragangliomas (PGLs) may have brown adipose tissue (BAT) activation induced by catecholamine excess. (18)F-fluorodeoxyglucose ((18)F-FDG) PET/CT can be used for the localization of both PGLs and BAT. It is unknown whether BAT is specifically affected by altered cellular energy metabolism in patients with SDHx and VHL-related PGLs.To determine endocrine and paracrine effects of catecholamine excess on BAT activation in patients with PGLS as detected by (18)F-FDG PET/CT, taking into account genetic variation.Patients with PGLs who were fully genetically characterized underwent pre-surgical (18)F-FDG PET/CT imaging for tumor localization and to quantify BAT activation.Single Dutch tertiary referral centre.73 patients, age 52.4 ± 15.4 yr, BMI 25.2 ± 4.1 kg/m(2), mean ± SD, were grouped into sporadic, cluster 1 (SDHx, VHL) and cluster 2 (RET, NF1, MAX) mutations.(18)F-FDG mean standard uptake values (SUVmean) were assessed in predefined BAT locations, including perirenal fat.21/73 (28.8\%) patients exhibited BAT activation. BAT activation was absent in all six patients with non-secreting PGLs. No difference in (18)F-FDG uptake by perirenal fat on the side of the pheochromocytoma and the contralateral side was observed (SUVmean 0.80 vs. 0.78 respectively, P=0.42). The prevalence of BAT activation did not differ between sporadic (28.9\%), cluster 1 (40.0\%) and cluster 2 patients (15.4\%), P=0.36.Patients with PGLs exhibit a high prevalence of BAT activation on (18)F-FDG PET/CT. This is likely due to systemic catecholamine excess. BAT activation is not associated with specific germline mutations.

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