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
. 2018 Dec;97(50):e13322.
doi: 10.1097/MD.0000000000013322.

An ectopic adrenocortical adenoma in renal hilum presenting with Cushing's syndrome: A case report and literature review

Difei Lu  1 , Nan Yu, Xiaowei Ma, Junqing Zhang, Xiaohui Guo
Affiliations
Review

An ectopic adrenocortical adenoma in renal hilum presenting with Cushing's syndrome: A case report and literature review

Difei Lu et al. Medicine (Baltimore). 2018 Dec.

Abstract

Rationale: Ectopic adrenal tissue is the adrenal rests along the path from gonads to adrenal glands during embryogenesis. Ectopic adrenocortical adenoma is a rare disease represented with over-production of cortisol by the ectopic adrenocortical tissue.

Patient concerns: An 18-year-old Chinese female patient was presented with weight-gain for 6 months. She had elevated plasma cortisol and a solitary mass was revealed using computed tomography scan in the left renal hilum.

Diagnosis: The tumor was removed and the immunohistochemical profile indicated an ectopic adrenocortical adenoma.

Interventions: After the tumor was removed, the patient was under glucocorticoid replacement therapy in 6-month.

Outcomes: During 6-month of follow-up, the patient showed no signs of tumor recurrence.

Lessons: Ectopic adrenocortical adenoma is difficult to diagnose due to its low incidence, and the ectopic rests in renal hilum could be misdiagnosed as renal cell carcinoma. This case reminds clinicians to be aware of ectopic site in the diagnosis of adrenocorticotropic hormone (ACTH) independent Cushing's syndrome. Immunohistochemical stain may assist in evaluating the origin of the ectopic rests. A certain rate of local recurrence indicated the need of long-term follow-up.

PubMed Disclaimer

Conflict of interest statement

Authors have no conflict of interests.

Figures

Figure 1
Figure 1
A: Abdominal CT of the patient. Enhanced CT showed a well-circumscribed round mass with a maximum diameter of 3.0 cm in the left renal hilum. B: CT scan revealed bilateral adrenal glands were atrophic. 1C: the ectopic adrenal tumor was resected during the operation, and was sized 3.0cm × 2.5cm × 1.5 cm. CT = computed tomography.
Figure 2
Figure 2
The immunohistological stain of the ectopic adrenal tumor. 2A: AE1/AE3 (+). 2B: angiographic positive for CD34. 2C: CgA (−). 2D: CgA-OR (+). 2E and 2F: HE stain. 2G: partially positive for HMB45. 2H: inhibitin (+++). 2L: Melan-A (+). 2J: NSE (−). 2K: Synaptophysin (+). 2L: vimentin (+).
See this image and copyright information in PMC

References

    1. Barwick TD, Malhotra A, Webb JAW, et al. Embryology of the adrenal glands and its relevance to diagnostic imaging. Clin Radiol 2005;60:953–9. - PubMed
    1. Ren PT, Fu H, He XW. Ectopic adrenal cortical adenoma in the gastric wall: case report. World J Gastroenterol 2013;19:778–80. - PMC - PubMed
    1. Makino K, Kojima R, Nakamura H, et al. Ectopic adrenal cortical adenoma in the spinal region: case report and review of the literature. Brain Tumor Pathol 2010;27:121–5. - PubMed
    1. Anderson JR, McLean Ross AH. Ectopic adrenal tissue in adults. Postgrad Med J 1980;56:806–8. - PMC - PubMed
    1. Souverijns G, Peene P, Keuleers H, et al. Ectopic localization of adrenal cortex. Eur Radiol 2000;10:1165–8. - PubMed