Pheochromocytoma and Paraganglioma: From Treatment to Follow-up

Pheochromocytomas (PCC) and paragangliomas (PGL) are rare neuroendocrine tumors arising from chromaffin cells in the adrenal medulla, sympathetic or parasympathetic ganglia. Currently, the only curative treatment option of pheochromocytomas/paraganglioma (PPGL) is surgical resection. Surgery aims to eliminate both risks of hypersecretion and tumor growth. The consequences of hypersecretion should be carefully controlled with medical therapy before and during the surgery. Postoperative major complications are hypotension and rebound hypoglycemia, and patients should be followed closely for 24-48 hours. The choice of surgical approach is determined based on multiple factors, including germline genetic test results, the size of the tumor, body mass index, surgeon’s experience, and the likelihood of malignancy. Primary tumor resection does not completely eliminate the risk of tumor persistence and recurrence. Therefore, all patients with PPGL who are surgically treated should be followed for at least 10 years for recurrent disease and new tumor formation. Although surgical resection is the only curative treatment for PPGLs, surgical treatment is palliative except for resectable locoregional metastases in metastatic disease or for isolated distant metastases. The purpose of palliative treatment is to reduce hormone secretion and prevent metastasis-related complications in a critical anatomical location Combined and alfa;- and beta- adrenergic blockade is usually applied in patients with PPGL preoperatively. Some patients may present with pheochromocytoma multisystem crisis, which is a life-threatening condition that can involve cardiovascular, pulmonary, neurological, gastrointestinal, renal, hepatic and metabolic systems. Pheochromocytoma crisis may be spontaneous or may present with the tumor manipulation, trauma, corticosteroids, beta-blockers, anesthetic drugs, and the stimulation of non-adrenal surgical stress. These patients should be considered as medical emergencies rather than surgical emergencies. In this review, it was aimed to evaluate the pre-, per and post-operative management, curative and palliative surgical management, and postoperative outcomes and follow-up of the patients with PPGLs.