¹⁸F‐FDG PET/CT as a predictor of hereditary head and neck paragangliomas

Abstract

Background Hereditary head and neck paragangliomas (HNPGLs) account for at least 35% of all HNPGLs, most commonly due to germline mutations in SDHx susceptibility genes. Several studies about sympathetic paragangliomas have shown that 18F-FDG PET/CT was not only able to detect and localize tumors, but also to characterize tumors (18F-FDG uptake being linked to SDHx mutations). However, the data concerning 18FFDG uptake specifically in HNPGLs have not been addressed. The aim of this study was to evaluate the relationship between 18F-FDG uptake and the SDHx mutation status in HNPGL patients. Methods 18F-FDG PET/CT from sixty HNPGL patients were evaluated. For all lesions, we measured the maximum standardized uptake values (SUVmax), and the uptake ratio defined as HNPGL SUVmax over pulmonary artery trunk SUVmean (SUVratio). Tumor sizes were assessed on radiological studies. Results Sixty patients (53.3% with SDHx mutations) were evaluated for a total of 106 HNPGLs. HNPGLs SUVmax and SUVratio were highly dispersed (1.2-30.5 and 1.0-17.0 respectively). The HNPGL 18F-FDG uptake was significantly higher in SDHx versus sporadic tumors on both univariate and multivariate analysis (p=0.002). We developed two models for calculating the probability of a germline SDHx mutation. The first one, based on a per-lesion analysis, had an accuracy of 75.5%. The second model, based on a per-patient analysis, had an accuracy of 80.0%. Conclusions 18F-FDG uptake in HNPGL is strongly dependent on patient genotype. Thus, the degree of 18F-FDG uptake in these tumors can be used clinically to help identify patients in whom SDHx mutations should be suspected.

Correlation Between In Vivo 18F-FDG PET and Immunohistochemical Markers of Glucose Uptake and Metabolism in Pheochromocytoma and Paraganglioma

“…18 F-FDG PET/CT is also useful for localizing benign PPGLs (7). Interestingly, 18 F-FDG uptake varies among PPGLs of different genotypes, with the highest standard uptake values (SUVs) being observed in SDH and VHL-related tumors (5,8). The precise mechanism behind these genotype-specific differences in 18 F-FDG uptake has not been elucidated.…”

15 YEARS OF PARAGANGLIOMA: Imaging and imaging-based treatment of pheochromocytoma and paraganglioma

“…123 I-MIBG scintigraphy has a sensitivity of 77-90% and specificity of 95-100% in the detection of PHEO; its sensitivity is lower for PGLs, especially for some that are hereditary (see below (Taieb et al 2009, 2014a). Several potential diagnoses should be considered in cases of highly 18 F-FDG-avid adrenal masses (Fig.…”

“…Several studies have found that 18 F-FDG PET/CT is excellent for SDHx germline mutations in comparison to other tumor types, regardless of tumor location . The performance and PGLs (Blanchet et al 2014a). By using an uptake ratio, it has even been shown that models could be established for predicting PHEO/PGL genotypes (Blanchet et al 2014a Ga-DOTA peptides Figure 5 Functional imaging findings in a case of extraadrenal sympathetic SDHBrelated PGL.…”

“…The performance and PGLs (Blanchet et al 2014a). By using an uptake ratio, it has even been shown that models could be established for predicting PHEO/PGL genotypes (Blanchet et al 2014a Ga-DOTA peptides Figure 5 Functional imaging findings in a case of extraadrenal sympathetic SDHBrelated PGL. Multimodality imaging with 123 I-MIBG (anterior and posterior views), SRS (three images), and 18 F-FDG PET/CT.…”

Genotype–phenotype Correlation in Children with Pheochromocytoma and Paraganglioma

“…18 The 18 F-FDG PET/CT can differentiate SDHx-related HNPGL from sporadic ones with 80% accuracy. 19 However, no such data are available in children.…”