Bilateral pheochromocytoma: case series and review of treatment strategies based on genetic mutations

Based on our experience and a review of the literature, adrenal-sparing surgery allows preservation of adrenal function and can eliminate the need for postoperative steroid replacement in many patients with bilateral PCC. Although the risk of recurrence in the residual adrenal gland may be higher than that after total adrenalectomy, recurrence at other sites and overall mortality appear comparable between the two surgical approaches. Therefore, adrenal-sparing surgery represents a valuable surgical option in carefully selected patients. Furthermore, recent studies have identified pathogenic mutations in several genes associated with bilateral PCC, with incidence and prognosis varying according to genetic background. Gene mutations are more likely in younger patients, those with tumors ≥5 cm, paragangliomas (PGLs), norepinephrine-secreting tumors lacking epinephrine production, and individuals with known hereditary syndromes, who also exhibit a higher risk of recurrence. Accordingly, current guidelines recommend lifelong surveillance and individualized management based on genetic and clinical factors.