肾上腺皮质癌手术和药物治疗进展

Advances in surgical and drug treatment of adrenocortical carcinoma

肾上腺皮质癌(adrenocortical carcinoma,ACC)是一种罕见的高侵袭性内分泌肿瘤。手术是治疗ACC的主要方法,mitotane具有破坏肾上腺皮质细胞活性的作用,是治疗ACC的主要药物,该药血浆药物浓度>14 mg/L可观察到治疗效果。管理mitotane治疗的主要困难与副作用和严重不良反应有关,毒性风险与药物的血浆浓度>20 mg/L呈正相关,这是治疗窗的上限;mitotane可能会导致肾上腺皮质功能不全,糖皮质激素替代治疗必须以比其他原发性肾上腺皮质功能不全患者更高的剂量给药。此外,应考虑预防肝功能不全、呕吐和消化道相关症状,与mitotane相关的其他内分泌系统副作用,特别是甲状腺激素和睾酮代谢也应被重视。免疫检查点抑制剂单药治疗或联合化疗在一些临床试验中取得了较好的结果,但需要更大规模的对照研究来验证。ACC的治疗方案需要内分泌科、泌尿外科、肿瘤内科和放射科等多学科共同决定。

Adrenocortical carcinoma(ACC)is a rare and highly invasive endocrine tumor.Surgical resection is the main treatment for ACC,and mitotane has the effect of destroying the activity of adrenocortical cells,which is the main medicine for ACC treatment.When the plasma concentration of mitotane was more than 14 mg/L,the therapeutic response was observed.The main difficulty in managing the treatment of mitotane is associated with side effects and severe adverse reactions.The risk of toxicity is positively correlated with the plasma concentration of the drug up to 20 mg/L,which is the warning-limit of the treatment window.The treatment of mitotane may lead to adrenocortical dysfunction,so glucocorticoid replacement therapy must be administered at a higher dose than other patients with primary adrenal insufficiency.In addition,the prevention of liver dysfunction,vomiting and gastrointestinal symptoms,and other endocrine side effects related to mitotane,especially the metabolism of thyroid hormone and testosterone,should be considered.Immunocheckpoint inhibitor mono-therapy or combination chemotherapy has achieved good results in some clinical trials recently,but it needs more population controlled studies to verify.The treatment of ACC needs to be decided by endocrinological,urological,oncological and radiological doctors.