Korean J Intern Med 2022;37:1092-1093 https://doi.org/10.3904/kjim.2021.552
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Adrenal cortical carcinoma: a natural progress
Chaiho Jeong1, Ye Seul Yang1, Ok Ran Shin2, Hyun-Shik Son1, and Tae-Seo Sohn1
1Division of Endocrinology and Metabolism, Department of Internal Medicine, 2Department of Hospital Pathology, Uijeongbu St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
A 48-year-old man presented to our en- docrinology department for the evalu- ation of the left adrenal incidentaloma discovered during fatty liver assessment. Abdominal computed tomography re- vealed a 1.8 cm left adrenal incidentalo- ma (Hounsfield units 40). The results of laboratory findings were as follows: se- rum adrenocorticotropic hormone 54.3 pg/mL, cortisol 6.39 µg/dL, renin activity 0.41 ng/ml/hr, aldosterone 12.8 ng/dl,
thyroid-stimulating hormone 4.86 mIU/L, and free thyroxine 1.30 ng/dL. The 24- hour urine collection results were as fol- lows: epinephrine 12.82 µg/day, norepi- nephrine 43.71 µg/day, vanillyl mandelic acid 3.10 mg/day, cortisol 39.3 µg/day. The tumor I-131 metaiodobenzylguani- dine (MIBG) scan showed no abnormal uptake. The patient was diagnosed with nonfunctioning adrenal incidentaloma, and was recommended for regular fol-
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Received : December 29, 2021
Revised : February 9, 2022
Accepted : February 9, 2022
Correspondence to
Tae-Seo Sohn, M.D.
Tel: +82-31-847-8077
Fax: +82-31-820-3652
E-mail: imsts@catholic.ac.kr
https://orcid.org/0000-0002-5135- 3290
Figure 1. Contrast abdominal computed tomography scan shows the increasing course of a left ad- renal mass (adrenal mass; red arrows). (A) Baseline 0 month, size of 1.8 cm in diameter; (B) 15 months, size of 2.7 × 2.3 cm; (C) 39 months, size of 4.7 x 3.3 cm; (D) 74 months, size of 8.0 × 4.3 cm.
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low-up. The adrenal mass continuously increased from 1.8 cm in size at baseline to 2.7 x 2.3 cm at month 15, and finally to 8.0 x 4.3 cm after 6 years of follow-up suggest- ing a high possibility of malignancy (Fig. 1). No hormonal change was observed during follow-up. However, the pa- tient refused surgical management several times for person- al reasons. At last, after 6 years of follow-up, he received the surgery. The pathology revealed adrenal cortical carcinoma (ACC) with 4-5/10 high-power field (HPF) mitotic rate (Fig. 2). After surgery, the patient went through several systemic chemotherapy but expired due to recurrent multiple ACC metastasis.
ACCs are rare tumors with an estimated annual incidence of 0.7 to 2 cases per million population. ACC prognosis is
poor, with an overall survival of 3.21 years from diagnosis. Therefore, evaluation of adrenal incidentaloma is crucial for the early diagnosis of ACC. Even if the initial presentation of adrenal incidentaloma is small without hormonal function, intense regular follow-up is necessary to detect any malig- nancies. Our case highlights the natural growing course of ACC, first diagnosed as a small incidentaloma. The study was approved by Institutional Review Board of Uijeong- bu St. Mary’s Hospital, the Catholic University of Korea (UC21ZISI0128).
Conflict of interest
No potential conflict of interest relevant to this article was reported.