Background: Anatomical imaging with CT or MRI is regarded as first-choice modality for tumour localization in patients with pheochromocytoma and paraganglioma (PPGL). Functional imaging with 123I-labeled metaiodobenzylguanidine (123I-MIBG) is widely used, but the added diagnostic value is unclear. We investigated the impact of 123I-MIBG scintigraphy on diagnosis and treatment of PPGL compared to anatomical imaging alone.
Methods: In this retrospective international multicenter study we evaluated 340 patients with PPGLs (236 unilateral adrenal, 18 bilateral adrenal, 48 extra-adrenal, 12 multifocal, 26 metastatic) from seven centres. All patients underwent both CT and/or MRI and 123I-MIBG scintigraphy. Clinical data were obtained and local imaging reports were analysed centrally by two blinded independent observers. For each patient, tentative diagnoses based on CT/MRI alone, 123I-MIBG alone, CT/MRI plus 123I-MIBG were compared with the actual diagnosis as recorded in registries of the local centers. Results were analysed in relation to tumour location and size, biochemical phenotype, and genotype.
Results: Diagnoses based on only CT/MRI and CT/MRI plus 123I-MIBG showed close concordance with actual diagnoses of 89 and 88.2% respectively (non-significant). In 3 out of 6 patients with false-negative results on CT/MRI, the diagnosis was corrected by 123I-MIBG (1 unilateral, 2 extra-adrenal). In 1 out of 30 patients with false-positive results on CT/MRI, diagnosis was corrected by 123I-MIBG. 123I-MIBG yielded false-positive lesions in 8 patients (7 unilateral, 1 extra-adrenal). Overall, 123I-MIBG resulted in an appropriate change in management for 4 patients (1%) and an inappropriate change for 9 patients (3%) (non-significant).
Conclusion: The addition of 123I-MIBG scintigraphy to CT/MRI for localization of PPGL does not lead to more correct than incorrect changes in the diagnoses and treatment, even when 123I-MIBG scintigraphy is restricted to patients at risk for metastatic disease. However 123I-MIBG scintigraphy remains necessary for all patients with metastatic PPGL who qualify for 131I-MIBG radiotherapy.