Long-term survival in recurrent adrenocortical cancer

Adrenocortical carcinoma (ACC) comprises approximately 0.02% of all malignant tumours, which are a very small fraction of a group of cancers that affect in 0.7 to 2 in 1 000 000 people per year. Recurrence is very common even after complete resection and prognosis is poor. We report a case of a sporadic form of ACC found in a 41-year-old Asian Bangladeshi man. His tumour was surgically excised completely with negative margins and he did not receive any adjuvant therapy. Four years later, adrenal adenoma was developed at his opposite side which was also excised. Then after a total duration of 7 years, he developed recurrence in both adrenal glands and extensive metastases to bilateral lungs, liver and abdominal wall. As per FIRM-ACT study, we started treatment with etoposide, doxorubicin, cisplatin plus mitotane (EDP-M), and the patient responded dramatically. He became symptom free and achieved radiological partial response just on completion of three cycles. To our knowledge, this is the first case of ACC in Bangladesh published in the literature. Managing rare cancers is always challenging due to the fact that clinicians lack practical experience in it. We believe that patient with a rare cancer with poor prognosis like ACC may also survive long, and extensive metastases can also be controlled.