ReviewCurrent treatment options in oncology2025
Update in the Management of ACTH-Secreting Gastroenteropancreatic and Thoracic Neuroendocrine Neoplasms.
Review in Current treatment options in oncology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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6 authors.
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Abstract
opinion statementThe adrenocorticotropic hormone (ACTH)-secreting neuroendocrine neoplasms (NENs) represent a rare but frequently severe disease that is associated with poor prognosis, if not adequately managed. Treatment strategies vary according to the characteristics of the tumor and the severity of hypercortisolism. The optimal treatment is surgical resection of the NEN with a curative intent. In unresectable or metastatic tumors, medical control of hypercortisolism with concomitant treatment of NENs based on current guidelines is suggested. Steroidogenesis inhibitors are the principal choice to control hypercortisolism while bilateral adrenalectomy should be considered in cases of failure to control severe life-threatening hypercortisolism. Additionally, amongst the various anti-tumor systemic treatment options, peptide receptor radionuclide therapy (PRRT) seems to also be an effective option for the control of hypercortisolism. Preventive and curative treatment of cortisol-induced complications and comorbidities is also important to reduce morbidity and mortality. Overall, the management of ACTH secreting NENs may be very complex and requires an individualized approach in a multidisciplinary context to achieve the best and timely outcome for the patient.
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