Kuthiah N, Er C
General Medicine, Alexandra Health System Woodlands Health Campus, Singapore.
Oxf Med Case Reports. 2019 Feb 27;2019(2):omz006. doi: 10.1093/omcr/omz006. eCollection 2019 Feb.
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 . Genotyping of adrenocortical tumours: very frequent deletions of the MEN1 locus in 11q13 and of a 1-centimorgan region in 2p16. 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.
肾上腺皮质癌是一种罕见的内分泌恶性肿瘤,预后较差。肾上腺皮质癌可见于多种家族综合征,如多发性内分泌腺瘤病1型(MEN-1)、李-佛美尼综合征、贝克威思-维德曼综合征和卡尼综合征(凯尔曼,M,罗沙尼,L,特,BT。肾上腺皮质肿瘤的基因分型:11q13上MEN1基因座和2p16上1厘摩区域的频繁缺失。1999年;84:730 - 5)。肾上腺皮质癌的治疗选择有限。我们报告一例50岁女性肾上腺皮质癌病例,该患者随后接受了肾上腺切除术。在发现转移病灶后,她开始接受米托坦作为辅助治疗和化疗。由于该肿瘤罕见,对其治疗方式的了解和经验有限。未来几年可能会有新的治疗选择来改善治疗效果。早期识别肿瘤是提高无进展生存期几率的关键。