An Ectopic Cortisol-Producing Adrenocortical Adenoma Masquerading as a Liposarcoma in the Pararenal Space

Article information

Endocrinol Metab. 2018;33(3):423-424

Publication date (electronic) : 2018 August 14

doi : https://doi.org/10.3803/EnM.2018.33.3.423

Sunyoung Kang ¹

, Seung Shin Park ¹

, Jae Hyun Bae ¹

, Kyu Eun Lee ²

, Jung Hee Kim ¹

, Chan Soo Shin ¹

¹Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea.

²Department of General Surgery, Seoul National University College of Medicine, Seoul, Korea.

Corresponding author: Chan Soo Shin. Department of Internal Medicine, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul 03080, Korea. Tel: +82-2-2072-3734, Fax: +82-2-765-3734, csshin@snu.ac.kr

Received 2018 May 31; Accepted 2018 June 18.

Keywords: Adrenocortical adenoma; Adrenal rest tumor; Cushing syndrome; Pararenal space; Liposarcoma

58-year-old man complained of abdominal distension and a moon-shaped face that had lasted for 3 years. The patient had been diagnosed with diabetes mellitus and hypertension 5 years ago. Biochemical tests revealed adrenocorticotropic hormone (ACTH)-independent Cushing syndrome. However, a computed tomography (CT) scan showed bilateral adrenal gland atrophy and a liposarcoma-like mass in the right pararenal space, and the irregularly enhanced mass was deeply surrounded by fat. Needle biopsy indicated an eosinophilic epithelioid cell tumor. The patient underwent surgical removal of the tumor through laparotomy. Microscopic examination favored ectopic adrenocortical neoplasm with a low risk of malignancy (Weiss score 1) and the immunohistochemical stain of the tumor was positive for Melan A and inhibin α, which are positive in over 80% of adrenal cortical tumors [1 2]. The patient's blood pressure and glycemic control improved after surgery. His serum cortisol levels decreased, but the patient has been taking steroids due to postoperative adrenal insufficiency.

Ectopic adrenocortical adenoma is rare, and most of these tumors are located along the pathway of embryonic migration within the urogenital tract [3]. Ectopic ACTH-secreting tumors are not uncommon, but ectopic cortisol-producing tumors, especially in the pararenal sinus, are extremely rare [4]. Our case presented typical features of Cushing syndrome, but CT images suggested the presence of liposarcoma. This finding could lead to misdiagnosis and radical resection of the tumor, including radical nephrectomy [5]. Moreover, if clinicians are not prepared for postoperative adrenal insufficiency, patients can experience adrenal crisis.

In cases of ACTH-independent Cushing syndrome without mass-like lesions on both adrenal glands, clinicians should suspect a cortisol-producing adrenal rest tumor.

Notes

CONFLICTS OF INTEREST: No potential conflict of interest relevant to this article was reported.

References

1. Konstantinov AS, Shelekhova KV. Ectopic adrenal cortical adenoma in the spinal canal: a case report and a review of the literature. Arkh Patol 2016;78:44–48. 27296006.

2. Makino K, Kojima R, Nakamura H, Morioka M, Iyama K, Shigematsu K, et al. Ectopic adrenal cortical adenoma in the spinal region: case report and review of the literature. Brain Tumor Pathol 2010;27:121–125. 21046315.

3. Ayala AR, Basaria S, Udelsman R, Westra WH, Wand GS. Corticotropin-independent Cushing's syndrome caused by an ectopic adrenal adenoma. J Clin Endocrinol Metab 2000;85:2903–2906. 10946901.

4. Tong A, Jia A, Yan S, Zhang Y, Xie Y, Liu G. Ectopic cortisol-producing adrenocortical adenoma in the renal hilum: histopathological features and steroidogenic enzyme profile. Int J Clin Exp Pathol 2014;7:4415–4421. 25120827.

5. Liu Y, Jiang YF, Wang YL, Cao HY, Wang L, Xu HT, et al. Ectopic adrenocortical adenoma in the renal hilum: a case report and literature review. Diagn Pathol 2016;11:40. 27094262.

Article information Continued

Korean Endocrine Society

(open-access, http://creativecommons.org/licenses/by-nc/4.0/) :

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.