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Dr Shashank R Joshi, Mumbai 18 December 2021

Computed tomography (CT) with arterial enhancement is the first-choice imaging modality. Magnetic resonance imaging (MRI) is recommended in patients with metastatic pheochromocytoma and paraganglioma (PPGL), for detection of the skull base and neck paragangliomas, in patients with surgical clips that cause artifacts when using CT, in patients with an allergy to CT contrast, and in patients in whom radiation exposure should be limited (children, pregnant women, patients with known germline mutations and those with recent excessive radiation exposure).

¹⁸F-FDOPA or ⁶⁸Ga-DOTATATE scanning is the preferred functional modality in patients with primary solitary or metastatic disease.
¹²³I-MIBG scintigraphy as a functional imaging modality in patients with metastatic PPGL, detected by other imaging modalities when radiotherapy using ¹³¹I-MIBG is planned.
All patients with a hormonally functional PPGL should undergo preoperative blockade with adrenoceptor blockers followed by β-adrenoceptor blockade as the first choice to prevent perioperative CV complications for 7 to 14 days.
Minimally invasive adrenalectomy is recommended for most adrenal PPGLs and open resection for large or invasive PPGLs to ensure complete resection and avoid local recurrence.
Multidisciplinary teams at centers with appropriate experience to ensure favorable outcomes should treat all patients with PPGL.

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