OXFORD
doi: 10.1093/qjmed/hcab276
CLINICAL PICTURE Adrenocortical carcinoma
Case description
A 73-year-old woman with a history of uncontrolled hyperten- sion, diabetes mellitus and dyslipidemia was referred to our hospital with a chief complaint of lower back pain, without any trigger for 1 month. Contrast-enhanced computed tomography (CECT), performed as part of the evaluation of back pain, revealed multiple vertebral fractures as the cause of the back pain (Figure 1a, arrows). Moreover, CECT revealed a heteroge- neous contrast-enhancing right adrenal mass that was 7.5 cm in length, in its largest dimension, suggestive of malignancy (Figure 1a, arrowheads). Laboratory studies were notable for a serum potassium level of 2.7 mmol/l (reference range: 3.6-4.8), and a morning serum cortisol level of 20 µg/dl (reference range: 5.27-22.45) with markedly suppressed plasma adrenocortico- tropic hormone level of <1.5 pg/ml (reference range: 7.2-63.3). The serum cortisol level was not suppressed on performing overnight dexamethasone suppression test (19.9 ug/dl, reference
range: < 1.8 µg/dl). Based on these findings, a clinical diagnosis of Cushing syndrome was made. Right adrenalectomy was per- formed 1 month after referral (Figure 1b), and hydrocortisone re- placement therapy was initiated for adrenal insufficiency immediately after the tumor resection. Histopathological ana- lysis confirmed the presence of high-grade adrenocortical car- cinoma (ACC), and adjuvant mitotane treatment was initiated 1 month after surgery.
Discussion
ACC is a rare and aggressive endocrine malignancy with an inci- dence of ~1 case per million population per year.1 Considering poor prognosis, the initial accurate diagnosis of ACC is critical for the management of the disease.2 Imaging features suggest- ive of ACC include large tumor size and heterogeneous density, reflecting the intratumoral necrosis, hemorrhage and
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calcification,3 and radiological evaluation plays a pivotal role in the differentiation of ACC and benign adrenal tumors. In addition, excess adrenal cortisol is the most common presentation of ACC in up to 40% of cases, indicating that the workup for cortisol ex- cess should be considered in suspected cases of ACC.3,4 In the present case, we concluded that multiple common metabolic complications, manifested with osteoporosis, were interpretable as hypercortisolism caused by ACC. Clinicians should consider the possibility of ACC when encountering a large adrenal mass accompanied by clinical manifestations of hypercortisolism.
Conflict of interest. None declared.
Ethics statement: Written informed consent was obtained from the patient for the publication of this article.
Photographs and text from: M. Hasebe (D; K. Shibue; S. Honjo and; A. Hamasaki ID, Department of Diabetes and Endocrinology, Tazuke Kofukai Medical Research Institute, Kitano Hospital, 2-4-20 Ohgimachi, Kita-ku, Osaka 530-8480, Japan. email: hasebe.masa- shi@gmail.com
References
1. Kebebew E, Reiff E, Duh QY, Clark OH, McMillan A. Extent of disease at presentation and outcome for adrenocortical carcinoma: have we made progress? World J Surg 2006; 30: 872-8.
2. Fassnacht M, Dekkers O, Else T, Baudin E, Berruti A, de Krijger R, et al. European Society of Endocrinology Clinical Practice Guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol 2018; 179:G1-46.
3. Bharwani N, Rockall AG, Sahdev A, Gueorguiev M, Drake W, Grossman AB, et al. Adrenocortical carcinoma: the range of appearances on CT and MRI. AJR Am J Roentgenol 2011; 196: W706-14.
4. Puglisi S, Perotti P, Pia A, Reimondo G, Terzolo M. Adrenocortical carcinoma with hypercortisolism. Endocrinol Metab Clin North Arm 2018; 47:395-407.