CASE REPORT
Adrenocortical carcinoma with primary aldosteronism associated with Cushing syndrome during recurrence
H. HISAMATSU, H. SAKAI, J. IRIE*, K. MAEDA and H. KANETAKE Departments of Urology and *Pathology, Nagasaki University School of, Medicine, Nagasaki, Japan
Case report
A 79-year-old woman was referred for evaluation of epi- sodic weakness of all four limbs, associated with marked hypokalaemia and hypertension. CT showed a heteroge- neous mass in the left suprarenal area (Fig. 1a). Endo- crinological studies showed hyperaldosteronism (Table 1) and she underwent transabdominal left adrenalectomy. The hormone studies showed elevated aldosterone and low plasma renin activity. Basal levels of cortisol and adrenocorticotropic hormone (ACTH) were normal, although the urinary excretion of 17-hydroxycorticoster- oid (17-OHCS) was slightly elevated, and the urinary excretion of 17-ketosteroid (17-KS) was normal. The histopathological diagnosis was adrenocortical carci- noma (Fig. 1b). Her BP, serum potassium and plasma aldosterone levels became normal after surgery. Unfortu- nately, evidence of metastatic spread to the lung and liver was detected 3 months after surgery. Repeat endocrino- logical investigations revealed a markedly elevated cortisol level and a suppressed ACTH; aldosterone was at normal levels whilst the plasma renin activity was slightly high. Despite the oral administration of mitotane, her condition deteriorated and she died 1 month later.
| Hormone | Normal range | Before | After | Recur. |
|---|---|---|---|---|
| Aldosterone, pg/L | 360-2400 | 3300 | 860 | 2400 |
| Plasma renin, pg/mL | 9.8-31.3 | <0.1 | 33.1 | |
| Cortisol, µg/mL | 5-15 | 9.2 | 79.4 | |
| ACTH, pg/mL | 8.2-54.8 | 17 | 30.2 | 4.5 |
| Urinary: | ||||
| adrenaline, µg/day | 3-15 | 3.4 | ||
| noradrenaline, ug/day | 26-121 | 67 | ||
| dopamine, ug/day | 190-740 | 519.5 | ||
| 17-0HCS, mg/day | 2.2-7.3 | 9.9 | ||
| 17-KS, mg/day | 2.4-11.0 | 5.9 |
Comment
Adrenocortical carcinoma is a rare tumour and accounts for only 0.05-0.2% of malignant tumours. A hormonally functioning tumour occurs in about half of affected patients. Most patients have clinical manifestations such as Cushing syndrome (50%), virilism (30%) or feminiza- tion (12%). However, primary aldosteronism is rarely seen
R
a
b
in patients with adrenocortical carcinoma [1]. Sasano et al. [2] reported a case of adrenocortical carcinoma with primary aldosteronism expressing all the enzymes required for the biosynthesis of cortisol. Arteaga et al. [3] reported a patient who had high levels of cortisol associ- ated with recurrent disease, and concluded that cancer cells have the potential capacity to indiscriminately pro- duce a wide spectrum of adrenal steroids. The present case reflects the potential ability of adrenocortical carcinoma cells to produce a wide spectrum of adrenal steroids and shows how the hormonal profile of the tumour may alter with time.
References
1 Yoshimoto T, Naruse M, Ito Y et al. Adrenocortical carcinoma manifesting pure primary aldosteronism: a case report and analysis of steroidogenic enzymes. J Endocrinol Invest 2000; 23: 112-7
2 Sasano H, Suzuki T, Nagura H et al. Steroidogenesis in human adrenocortical carcinoma. Biochemical activities, immuno- histochemistry, and in situ hybridization of steroidogenic enzymes and histopathologic study in nine cases. Hum Pathol 1993; 24: 397-404
3 Arteaga E, Biglieri EG, Kater CE et al. Aldosterone-producing adrenocortical carcinoma. Preoperative recognition and course in three cases. Ann Intern Med 1984; 101: 316-21
Authors
H. Hisamatsu, MD, PhD, Assistant Professor.
H. Sakai, MD, PhD, Assistant Professor.
J. Irie, MD, PhD, Assistant Professor.
K. Maeda, MD, PhD, Urologist.
H. Kanetake, MD, PhD, Professor.
Correspondence: H. Hisamatsu, Department of Urology, Nagasaki University School of Medicine, 1-7-1 Sakamoto, Nagasaki 852- 8501, Japan.
e-mail: hisa@net.nagasaki-u.ac.jp