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Cited by 239Pituitary Gland Disorders and TreatmentsNeuroendocrine Tumor Research AdvancesAdrenal and Paraganglionic Tumors

MANAGEMENT OF ENDOCRINE DISEASE: Cushing’s syndrome due to ectopic ACTH secretion: an expert operational opinion

Young, Jacques, Haissaguerre, Magalie, Viera-Pinto, Oceana, Chabre, Olivier, Baudin, Eric, Tabarin, Antoine·2020·European Journal of Endocrinology
DOI 10.1530/eje-19-0877
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Abstract

Ectopic ACTH syndrome (EAS) is rare but is frequently a severe condition because of the intensity of the hypercortisolism that may be dissociated from the tumoral condition. EAS should often be considered as an endocrine emergency requiring an emergency response both in terms of diagnostic procedures and therapeutic interventions. Patient management is complex and necessitates dual skills, in the diagnosis and treatment of CS and in the specific management of neuroendocrine tumors (NET). Therefore, initial management should be performed ideally by experienced endocrinology teams in collaboration with specialized hormonal laboratory, modern imaging platforms and intensive care units. Diagnostic procedures vary according to the endocrine and tumoral contexts but should be reduced to a minimum in intense hypercortisolism. Preventive and curative treatments of cortisol-induced comorbidities, non-specific management of hypercortisolism and etiological treatments should be considered simultaneously. Therapeutic strategies vary according to (1.) the intensity of hypercortisolism, the general condition of the patient and associated comorbidities and (2.) the tumoral status, ranging from resectable ACTH secreting tumors to non-resectable metastatic endocrine tumors or occult tumors. The ideal treatment is complete excision of the ACTH-secreting tumor that can be performed rapidly or after preoperative preparation using cortisol-lowering drugs. When this is not possible, the therapeutic strategy should be discussed by a multidisciplinary experienced team in a personalized perspective and include variable combinations of pharmacological agents, bilateral adrenalectomy and non-specific tumoral interventions. Here we discuss the diagnosis and therapeutic strategies including the modern, currently available tools and emphasize on the operational effectiveness of care.

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Crossref · 10.1530/eje-19-0877

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190 of 190 references verified0 citation errors · 0 not checkable

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  • Functional imaging using 68Ga-PET/CT or 18FDG PET/CT can detect occult tumors, reinforce tumors seen on CT scan as being neuroendocrine, or contribute to the workup of metastatic tumors.

    “Second-line tests include functional imaging using 68Ga-PET/CT or 18FDG PET/CT scans, which can be used to detect occult tumors, reinforce tumors seen on CT scan as being neuroendocrine, or contribute to the workup of metastatic tumors (9, 81).”

    From Approach to the Patient: Diagnosis of Cushing Syndrome (2022) · cites this paper as [81]

  • Type 2 vasopressin receptors, generally absent in normal pituitary corticotrophs, are expressed in corticotroph adenomas.

    “Desmopressin testing can also be used because type 2 vasopressin receptors, which are generally absent in normal pituitary corticotrophs, have found to be expressed in corticotroph adenomas.”

    From Approach to the Patient: Diagnosis of Cushing Syndrome (2022) · cites this paper as [81]

  • An increase in plasma ACTH and cortisol can be observed after injection of desmopressin in patients with Cushing disease.

    “Therefore, an increase in plasma ACTH and cortisol can be observed after injection of desmopressin in patients with Cushing disease (81).”

    From Approach to the Patient: Diagnosis of Cushing Syndrome (2022) · cites this paper as [81]

  • Dynamic testing with high-dose DST, CRH test, and desmopressin testing can help differentiate between Cushing disease and EAS.

    “Dynamic testing with high-dose DST, CRH test, and desmopressin testing can also be used to help differentiate between Cushing disease and EAS (9, 81).”

    From Approach to the Patient: Diagnosis of Cushing Syndrome (2022) · cites this paper as [81]

  • An absence of both ACTH and cortisol response is expected in patients with EAS, although false-positive results can be seen as EAS tumors may express type 3 vasopressin receptors.

    “On the other hand, an absence of both ACTH and cortisol is expected in patients with EAS, although false-positive results can be seen as EAS tumors may express type 3 vasopressin receptors (9, 74, 81).”

    From Approach to the Patient: Diagnosis of Cushing Syndrome (2022) · cites this paper as [81]

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Authors
Young, J., Haissaguerre, M., Viera-Pinto, O., Chabre, O., Baudin, E., & Tabarin, A.
Affiliations
  • Assistance Publique – Hôpitaux de Paris (FR)
  • Hôpital Cardiologique du Haut-Lévêque (FR)
  • 1Department of Endocrinology, Paris-Sud University and Paris Saclay University, Assistance Publique Hôpitaux de Paris, Bicêtre Hospital, Le Kremlin-Bicêtre, France
  • Université Joseph Fourier (FR)
  • 4Endocrine Oncology and Nuclear Medicine Department, Gustave Roussy Cancer Institute, Paris-Sud University and Paris-Saclay University, Villejuif, France
ORCID
Venue
European Journal of Endocrinology
Publisher
Oxford University Press (OUP)
Published
2020-04
Volume
182
Issue
4
Pages
R29-R58
ISSN
  • 0804-4643 (ISSN-L)
  • 1479-683X
DOI
10.1530/eje-19-0877
OpenAlex
W3003251208
Cited by
232
References
189
Citations by year
2026: 25 · 2025: 45 · 2024: 37 · 2023: 45 · 2022: 43 · 2021: 29 · 2020: 8
FWCI
23.56
Type
journal article
Language
en
Open access
closed
Locations
  • European Journal of Endocrinology (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Pituitary Gland Disorders and Treatments
  • Health Sciences › Medicine › Epidemiology › Neuroendocrine Tumor Research Advances
  • Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
Keywords
ectopic ACTH syndrome, Cushing's syndrome, hypercortisolism, neuroendocrine tumors, ACTH-secreting tumor, bilateral adrenalectomy, tumor resection, multidisciplinary management, endocrine emergencies
MeSH terms
ACTH Syndrome, Ectopic, Cushing Syndrome, Endocrinology, Humans, Treatment Outcome, Neuroendocrine Tumors

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