Metronomic chemotherapy may be active in heavily pre-treated patients with metastatic adreno-cortical carcinoma.

Objective: the potential benefit of further chemotherapy approaches in patients with adrenocortical carcinoma (ACC) showing progressive disease after 2 chemotherapy lines is actually unknown. This study provide explorative information on the activity of metronomic chemotherapy in heavily pretreated ACC patients. Design and methods: we tested the activity of cytotoxic treatments administered on a metronomic schedule in metastatic ACC patients showing disease progression after treatment with gemcitabine and capecitabine scheme. Results: eight patients out of 28 consecutively enrolled in that trial were treated with several metronomic cytotoxic regimens. Six of them showed disease progression but 2 patients obtained a clear benefit. The first patient was treated with oral etoposide (50 mg daily) as the 6th line therapy and obtained a partial response lasting 24 months, while the second patient obtained a partial response lasting 10 months with metronomic oral cyclophosphamide (50 mg daily) as the 5th chemotherapy line. Both patients had sex hormone secreting tumors and were bearing a rather indolent ACC. Conclusions: the administration of several chemotherapy lines in advanced ACC patients cannot be routinely recommended outside of prospective clinical trials. Few patients with indolent tumors, however, may benefit from this approach. According to our experience, oral cyclophosphamide and oral etoposide may be used in this setting.