gms | German Medical Science

Background: Adrenocortical carcinoma (ACC) is a rare and highly malignant tumour. More than 50% of adult patients with ACC present with endocrine dysfunctions (so-called functional ACC). Prognosis of recurrent or metastatic ACC is poor. Unfortunately, only little information is available concerning appropriate management of therapy and follow-up in these cases.

Case report: We report a case of metastatic ACC in a 55-year-old woman. First, an adrenalectomy was performed upon reveal of an adrenocortical tumour on the left side in computed tomography causing an adrenal Cushing’s disease. Histologic examination revealed only an atypical adrenal adenoma at this time. Two years later, hypercortisolism occurred again causing recurrence of her Cushing’s disease. Diagnostic imaging showed unclear hepatic lesions and a right hemihepatectomy was performed due to suspected metastasized ACC. Histopathologic examination confirmed metastases of ACC, subsequently the earlier diagnosis was changed. Postoperatively, an additional application of o, p'-DDD (Metatone®) was begun but not tolerated from the patient. A year later, a solitary osseous metastasis occurred at the left side of the sacrum. Curettage and stabilization followed by localized radiotherapy with 37.5 Gy led to a remission again.

Conclusion: This case report emphasises the need for careful clinical and radiographic follow-up. A repeated surgical approach with consolidating radiotherapy in selected cases should be adopted whenever possible providing long-term survival in metastatic disease. Further investigations are needed to evaluate the best multimodal management in recurrent or metastatic ACC.