Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2020 Jun;27(3):146-154.
doi: 10.1097/MED.0000000000000541.

Functional imaging in ectopic Cushing syndrome

Seda Grigoryan  1 , Anca M Avram  2 , Adina F Turcu  3
Affiliations
Review

Functional imaging in ectopic Cushing syndrome

Seda Grigoryan et al. Curr Opin Endocrinol Diabetes Obes. 2020 Jun.

Abstract

Purpose of review: Ectopic adrenocorticotropic hormone (ACTH)-secreting tumors are commonly small, yet they often lead to fulminant forms of Cushing syndrome. High-resolution functional imaging modalities, such as [Ga]-DOTATATE, have been recently introduced in clinical practice for the identification of neuroendocrine tumors. In this review, we focus on the performance of [Ga]-DOTATATE as a tool for localizing primary and metastatic sources of ectopic Cushing syndrome (ECS).

Recent findings: Prompt surgical removal of ectopic ACTH-secreting tumors is the mainstay of therapy in patients with ECS. Detecting such tumors with conventional cross-sectional imaging is often unsuccessful, owing to their small size. [Ga]-DOTATATE has been approved in 2016 by the Federal Drug Administration for imaging well differentiated neuroendocrine tumors. Data regarding the performance of [Ga]-DOTATATE for detecting ectopic ACTH-secreting tumors remain limited, in part owing to the recent introduction of this imaging modality in clinical practice, and in part because of the low prevalence of ECS. Nevertheless, [Ga]-DOTATATE has been reported to be useful in identifying primary and metastatic ectopic ACTH-secreting lesions that were not apparent on other imaging studies, impacting the clinical care of many patients with ECS.

Summary: [Ga]-DOTATATE-based imaging, which targets the somatostatin receptors abundantly expressed in neuroendocrine tumors, has generally high, although variable resolution in detecting the source(s) of ECS.

PubMed Disclaimer

Conflict of interest statement

Conflicts of interest

The authors have nothing to disclose.

Figures

FIGURE 1.
FIGURE 1.
[68Ga]-DOTATATE PET/CT scan demonstrating physiologic 68Ga-SSA peptide biodistribution: PET axial (A1 and B1) and coronal (C1) images with corresponding CT axial (A2 and B2) and coronal (C2) images and fused hybrid PET/CT axial (A3 and B3) and coronal (C3) images demonstrate diffuse physiologic uptake in the liver and focal intense uptake in the adrenal glands, spleen, and kidneys (images A1, A2, and A3 depict focal [68Ga]-DOTATATE uptake in the right adrenal gland; images B1, B2, and B3 depict focal [68Ga]-DOTATATE uptake in the left adrenal gland).
FIGURE 2.
FIGURE 2.
Proposed algorithm for imaging modality choice for identifying ACTH-secreting tumors. *, Consider MRI for tumors in the head, thyroid, and abdomen outside of gastrointestinal tract. #, Refer to oncology if metastatic/inoperable disease.
See this image and copyright information in PMC

References

    1. Pivonello R, Isidori AM, De Martino MC, et al. Complications of Cushing’s syndrome: state of the art. Lancet Diabetes Endocrinol 2016; 4:611–629. - PubMed
    1. Isidori AM, Sbardella E, Zatelli MC, et al. Conventional and nuclear medicine imaging in ectopic Cushing’s syndrome: a systematic review. J Clin Endocrinol Metab 2015; 100:3231–3244. - PMC - PubMed
    1. Ejaz S, Vassilopoulou-Sellin R, Busaidy NL, et al. Cushing syndrome secondary to ectopic adrenocorticotropic hormone secretion: the University of Texas MD Anderson Cancer Center Experience. Cancer 2011; 117: 4381–4389. - PMC - PubMed
    1. Ilias I, Torpy DJ, Pacak K, et al. Cushing’s syndrome due to ectopic corticotropin secretion: twenty years’ experience at the National Institutes of Health. J Clin Endocrinol Metab 2005; 90:4955–4962. - PubMed
    1. Isidori AM, Kaltsas GA, Pozza C, et al. The ectopic adrenocorticotropin syndrome: clinical features, diagnosis, management, and long-term follow-up. J Clin Endocrinol Metab 2006; 91:371–377. - PubMed

Publication types

MeSH terms