Imaging of Adrenal and Extra-Adrenal Paraganglioma

Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine neoplasms with variable degrees of heritability and molecular heterogeneity. Over the past 2 decades, advances in cross‑sectional and functional imaging have transformed the diagnosis of PPGL, its risk stratification, and patient‑tailored treatment planning. Computed tomography (CT) and magnetic resonance imaging form the basis of anatomic mapping, whereas molecular imaging with newer agents such as somatostatin‑receptor (SSTR) analog positron emission tomography/computed tomography (PET/CT), ¹⁸F‑fluoro‑L‑dihydroxyphenylalanine (¹⁸F‑FDOPA), ¹⁸F‑fluorodeoxyglucose (¹⁸F‑FDG), and radio iodinated metaiodobenzylguanidine (¹²³I/¹³¹I‑MIBG) enables phenotype‑directed staging and therapy selection. Contemporary consensus places somatostatin‑receptor analogues such as ⁶⁸Ga-DOTATATE and ⁶⁴Cu-DOTATATE at the center of head‑and‑neck paraganglioma, SDHx‑mutated disease, sporadic or hereditary head‑and‑neck paragangliomas, and metastatic PPGLs, while ¹⁸F‑FDOPA is increasingly favored for adrenal pheochromocytoma (PCC); and ¹⁸F‑FDG complements both by detecting aggressive biology. MIBG now is only considered for inoperable or metastatic PPGLs under consideration for ¹³¹I‑MIBG therapy.