Solitary pancreatic metastasis of an adrenocortical carcinoma

Adrenocortical carcinoma (ACC) is a rare, highly aggressive malignant tumor. In early stages, curative surgery is an important predictor of survival in ACC patients, while in advanced disease mitotane alone or in combination with cytotoxic drugs is standard of care.

In case of advanced ACC, the disease commonly spreads to the liver, lungs, lymph nodes, and bones. The pancreas has not been yet reported as a single site of metastases.

Here, we present a case of a 45-year old woman, who was diagnosed with ACC in 2006. She had typical signs of Cushing's syndrome. Hormone analysis indicates cortisol excess, elevated dehydroepiandrosterone sulphate (DHEA-S)- and androgen levels. The conducted CT showed a tumor of the left adrenal gland. The patient underwent conventional resection (R0), and the histopathological analysis showed a 5.2 cm adrenal tumor with a Weiss score in the range of benign tumor, but a high Ki-67 expression of 20%. No adjuvant therapy has been performed.

Almost 6 years after resection of the primary tumor a solitary pancreatic metastasis occurred. Hormone analysis indicated over the course of time an increase of androgen and DHEA-S levels. A MRI showed a new hyper-perfused mass in the head of the pancreas and a corresponding high FDG-uptake was observed with PET. The tumor was R0 resected. Histopathological analysis of the resected tissue showed a 1.5 cm sized well differentiated metastasis of the adrenocortical carcinoma with a Ki-67 expression in 10% of the tumor cells. Additionally adjuvant mitotane treatment was initiated. During a follow-up of two years after metastasis surgery, no evidence of tumor recurrence occurred.

Conclusion: Patients with advanced ACC can rarely present also solitary metastasis to the pancreas, even several years after the initial diagnosis. Timely diagnosis of pancreas metastasis and radical surgical resection can achieve a long-time complete resection in selected cases.