What is the place of adjuvant Mitotane therapy in Adrenocortical carcinoma?
H. Elgayar, J.F. Todd, T. Goldstone, W. Dhillo, E. C. Hatfield, N.M. Martin, I. Young, M. Seckl, K. Meeran, T.M-M. Tan, F.F Palazzo
Department of Endocrinology, Charing Cross and Hammersmith Hospitals, Imperial College Healthcare NHS Trust, Du Cane Road, London, W12 0HS
Adrenocortical carcinoma (ACC) is a rare epithelial cancer. It is aggressive; most develop metastatic disease within 1- 3 years of complete surgical resection. Systemic therapies generally do not affect survival, although some patients respond to the adrenolytic drug Mitotane. We present a case of adrenal cancer that was discovered incidentally and later developed early recurrence and metastasis.
A 38yr Caucasian lady presented with intermittent right abdominal pain. An USS of the abdomen organised by the GP revealed a large right retroperitoneal mass. On CT and subsequently MRI of the abdomen a 6.5 x 3cm mass was noted on the right adrenal gland with no evidence of distant metastasis. She also had no symptoms suggestive of adrenal hypersecretion. Endocrinological workup revealed an elevated DHEAS (20 umol/l – NR 0.8-6.9), suppressed ACTH (<5 ng/l), random cortisol of 646 nmol/l, and the 24 hr urine catecholamines and metanephrines were normal.
The patient underwent a right adrenalectomy, with total macroscopic clearance confirmed on CT. Histology showed features consistent with an ACC. Post operatively DHEAS was 0.4umol/l.
Although Mitotane adjuvant therapy was considered, the patient opted to delay the initiation of this medication. Unfortunately, she developed multiple peritoneal and pulmonary metastatic deposits 6 months post surgery. DHEAS was also elevated at 17umol/l with evidence of recurrence of her Cushing’s syndrome.
Mitotane’s potential benefit in postoperative adjuvant therapy following complete tumour resection is still controversial. Although some reports suggest that its use may delay or even prevent tumour recurrence post surgical extirpation, there is no randomized trial that compares this strategy to surgery alone in subjects with similar stages of ACC.