Pheochromocytomas

ABSTRACT

Introduction: Pheochromocytomas are rare neuroendocrine tumors, producing catecholamines, which usually affect the adrenal medulla region of the adrenal gland. These tumors may clinically manifest in several ways, presenting themselves in most patients with persistent hypertension or paroxysmal. Ten percent of cases are considered malignant, confirmed by the presence of metastases and approximately 24% of cases are associated with inherited syndromes. Diagnostic confirmation of these syndromes implies preparatory workup, treatment and stringent follow-up, preferably with a multidisciplinary team. Objective: This study is a survey of recent studies to clarify issues related to clinical, diagnosis, genetic and treatment aspects of these patients. Conclusion: It is widely accepted that a significant percentage of patients with sporadic pheochromocytoma may have germline mutations leading to more widespread disease development and/or malignancy, and that surgical treatment in these cases must be complemented by careful clinical surveillance for early diagnosis of recurrences. This study prioritized the importance of conducting a proper pretreatment workup in cases of pheochromocytoma, which provides the additional information required for a rational course of treatment for patients.