Cortisol as a possible marker of metastatic adrenocortical carcinoma: a case report with 3-year follow-up.

BACKGROUND

Adrenocortical carcinoma (ACC) is a rare tumour, sometimes causing glucocorticoid hypersecretion. Treatment guidelines have not been established, but are currently under investigation.

CASE REPORT

A 55-year-old Caucasian woman presented with adrenal Cushing's disease. Histological examination after a left adrenalectomy revealed a benign tumour. Postoperatively, elevated serum cortisol levels normalized. Hypercortisolism occurred again two years later. Diagnostic work-up revealed hepatic metastatic lesions of an ACC which were treated by right hemihepatectomy. Initial histological diagnosis was revised according to the increased proliferative changes. Postoperatively, cortisol declined to normal levels. Treatment with mitotane (o, p'-DDD) as a cytostatic agent was not tolerated. One year later, the patient was diagnosed with a solitary osseous metastasis at the left side of the sacrum because of back pain. Following curettage and stabilization, radiotherapy of this region with 37.5 Gy was performed, improving slightly elevated cortisol levels and neurological symptoms.

CONCLUSION

Careful clinical and radiographic follow-up of patients with ACC are important. In this case of oligometastasizing ACC, serum cortisol values correlated with the clinical course. An aggressive multimodal treatment, including repeated surgical approach with consolidating radiotherapy in cases of incomplete resection, might be indicated to provide symptom control and possible long-term survival in oligometastatic disease of ACC.