Management of pheochromocytomas and paragangliomas: Review of current diagnosis and treatment options

Abstract

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Tumor metabolism in pheochromocytomas: clinical and therapeutic implications

“…The recommended targets for blood pressure are 130/80 mmHg (1 mmHg = 0.133 kPa), while the heart rate should be between 60–80 beats per minute [ 2 , 88 ]. Administering α-adrenergic receptor blockers for 10–14 days before surgery, adequate volume repletion, and a high salt diet significantly reduce postoperative mortality rates [ 89 – 92 ]. The objective of preoperative medical management is to control hypertension and tachycardia while also addressing volume depletion caused by catecholamines [ 91 ].…”

“…This is discussed further in the systemic therapy. When there are no tumor-related symptoms and the tumor has a low metastatic potential, close monitoring may also be considered [ 89 , 121 ].…”

“…In order to alleviate symptoms associated with advanced disease and excessive catecholamine production by chromaffin cells, cytoreductive surgery is advised with or without adjuvant radiotherapy [ 89 , 122 , 123 ]. Radiotherapy can be considered for palliative treatment to provide symptomatic relief.…”

“…Instead, they are often used to manage disease progression after chemotherapy or radionuclide therapy, or in clinical trials [ 2 , 123 , 125 ]. CVD chemotherapy, temozolomide [with or without poly(ADP-ribose) polymerase (PARP) or PARP inhibitors], PRRT, and hypoxia-inducible factor 2α (HIF-2a) inhibitors are all viable therapeutic alternatives for cluster 1-related PPGLs [ 2 , 89 , 103 ]. Aside from the fact that only 2% to 4% of metastatic PPGLs possess cluster 2 mutations, cluster 2-related diseases rarely require systemic therapy [ 126 , 127 ].…”

“…The three clusters are aligned in their approach to radionuclide therapy, utilizing 131 I-MIBG radionuclide therapy in cases where a positive MIBG scan has been obtained, and employing PRRT in cases where a positive gallium DOTATAE scan has been obtained [ 2 , 89 , 103 ]. 131 I-MIBG, including the novel high specific activity (HSA) 131 I-MIBG, is the best-studied first-line therapeutic option for patients with PPGLs showing slow to moderate progression [ 2 , 131 – 137 ].…”