Performances of Functional and Anatomic Imaging Modalities in Succinate Dehydrogenase A-Related Metastatic Pheochromocytoma and Paraganglioma

Simple Summary Pheochromocytoma and paraganglioma (PPGL) are rare neuroendocrine cancers which carry the risk of metastatic disease. Pathogenic variants in the succinate dehydrogenase subunit A gene (SDHA) have been shown to cause metastatic disease, occurring in various regions of the body. Imaging is an early and vital step in the diagnosis and clinical care of these patients. The study here identifies which imaging modality among positron emission tomography (PET), computed tomography (CT), and magnetic resonance imaging (MRI) performs better in localizing metastatic PPGL lesions related to SDHA. The study identified that Ga-68-DOTATATE PET/CT performed best at overall lesion detection; however, F-18-FDG PET/CT performed better in certain anatomic regions of the body. A combined approach with Ga-68-DOTATATE and F-18-FDG would optimize care and guide clinicians in selecting the appropriate interventions and therapies. The study identifies the importance of positron emission tomographic (PET) and anatomic imaging modalities and their individual performances in detecting succinate dehydrogenase A (SDHA)-related metastatic pheochromocytoma and paraganglioma (PPGL). The detection rates of PET modalities-Ga-68-DOTATATE, F-18-FDG, and F-18-FDOPA-along with the combination of computed tomography (CT) and magnetic resonance imaging (MRI) are compared in a cohort of 11 patients with metastatic PPGL in the setting of a germline SDHA mutation. The imaging detection performances were evaluated at three levels: overall lesions, anatomic regions, and a patient-by-patient basis. Ga-68-DOTATATE PET demonstrated a lesion-based detection rate of 88.6% [95% confidence interval (CI), 84.3-92.5%], while F-18-FDG, F-18-FDOPA, and CT/MRI showed detection rates of 82.9% (CI, 78.0-87.1%), 39.8% (CI, 30.2-50.2%), and 58.2% (CI, 52.0-64.1%), respectively. The study found that Ga-68-DOTATATE best detects lesions in a subset of patients with SDHA-related metastatic PPGL. However, F-18-FDG did detect more lesions in the liver, mediastinum, and abdomen/pelvis anatomic regions, showing the importance of a combined approach using both PET modalities in evaluating SDHA-related PPGL.