Abstract
Adrenocortical carcinoma is a rare endocrine malignancy with poor prognosis. Adrenocortical carcinoma can be seen in familial syndromes such as multiple endocrine neoplasia 1(MEN-1), Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome and Carney complex (Kjellman, M, Roshani, L, The, BT et al. Genotyping of adrenocortical tumours: very frequent deletions of the MEN1 locus in 11q13 and of a 1-centimorgan region in 2p16. J Clin Endocrinol Metab 1999;84:730-5). Treatment options for adrenocortical carcinoma are limited. We report a case of adrenocortical carcinoma in a 50-year-old lady who subsequently underwent adrenalectomy. She was started on mitotane as adjuvant therapy and chemotherapy after the metastatic lesions were found. Due to the rarity of the tumour, the understanding and experience of management modalities is limited. New treatment options may be available in the coming years to improve outcome. Early identification of tumour is key to increase the chances of progression free survival.