Management of Pheochromocytomas and Paragangliomas: A Case-Based Review of Clinical Aspects and Perspectives

Abstract

Paraganglioma and pheochromocytoma are rare medical conditions. Thus, there are still a small number of studies, clinical trials, and evidence-based data in this field. This makes clinical decisions more difficult. In this study, we present a case report enriched with a short review of available essential clinical data, indicating the need for constant metoxycatecholamine level observation and a proper diagnostic imaging approach, especially in terms of ongoing pandemics. Our research also provides a summary of the molecular background of these diseases, indicating their future role in clinical management. We analyzed the ClinicalTrials.gov dataset in order to show future perspectives. In this paper, the use of the PET-CT before MRI or CT is proposed in specific cases during diagnosis processes contrary to the guidelines. PET-CT may be as effective as standard procedures and may provide a faster diagnosis, which is important in periods with more difficult access to health care, such as during the COVID-19 pandemic.

Keywords: neuroendocrine tumors; nuclear medicine; paraganglioma; pheochromocytoma; radiology.

Figure 1

The clinical symptoms of pheochromocytoma and paraganglioma.

Figure 2

(A) combined ⁶⁸GA-DOTATE PET and CT images showing a large, highly metabolically active mass in the skull; (B) CT scan showing a polycystic tumor compressing the left cerebellar tonsil; (C) combined MR and CT scan showing infiltration of the left sublingual nerve extending to the left internal carotid artery canal and obstructing the left internal jugular vein at the inferior bulb region.