{"title":"Update in the Management of ACTH-Secreting Gastroenteropancreatic and Thoracic Neuroendocrine Neoplasms.","authors":"Marina Tsoli, Anat Bel-Ange, Karine Atlan, Simona Ben-Haim, Gregory Kaltsas, Simona Grozinsky-Glasberg","doi":"10.1007/s11864-025-01354-0","DOIUrl":null,"url":null,"abstract":"<p><strong>Opinion statement: </strong>The 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.</p>","PeriodicalId":50600,"journal":{"name":"Current Treatment Options in Oncology","volume":" ","pages":""},"PeriodicalIF":4.7000,"publicationDate":"2025-10-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Current Treatment Options in Oncology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1007/s11864-025-01354-0","RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"ONCOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Opinion statement: The 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.
期刊介绍:
This journal aims to review the most important, recently published treatment option advances in the field of oncology. By providing clear, insightful, balanced contributions by international experts, the journal intends to facilitate worldwide approaches to cancer treatment.
We accomplish this aim by appointing international authorities to serve as Section Editors in key subject areas, such as endocrine tumors, lymphomas, neuro-oncology, and cancers of the breast, head and neck, lung, skin, gastrointestinal tract, and genitourinary region. Section Editors, in turn, select topics for which leading experts contribute comprehensive review articles that emphasize new developments and recently published papers of major importance, highlighted by annotated reference lists. We also provide commentaries from well-known oncologists, and an international Editorial Board reviews the annual table of contents, suggests articles of special interest to their country/region, and ensures that topics are current and include emerging research.