Journal: Current treatment options in oncology
ACTH-secreting neuroendocrine neoplasms (NENs) are rare but often severe tumors with a poor prognosis if not properly managed.
Surgical resection remains the optimal curative treatment.
For unresectable or metastatic cases, controlling hypercortisolism medically—primarily with steroidogenesis inhibitors—is essential.
Bilateral adrenalectomy is reserved for refractory, life-threatening cases.
Peptide receptor radionuclide therapy (PRRT) also shows promise in managing hypercortisolism.
Addressing cortisol-induced complications through preventive and curative measures is critical to reduce morbidity and mortality.
Management is complex and requires an individualized, multidisciplinary approach to optimize outcomes.