肾上腺皮质癌是一种非常罕见的内分泌疾病，预后不良，并且经常与 ACTH 无关的库欣综合征相关。尽管患有肾上腺皮质癌，我们的患者令人惊讶地患有 ACTH 依赖性库欣综合征。一名 26 岁女性出现库欣综合征和腹部肿块。影像学研究显示肾上腺肿块符合高度恶性肿瘤。实验室检查显示皮质醇增多症、雄激素过多症和低钾血症，但变肾上腺素水平正常。出乎意料的是，她的ACTH水平显着升高。肿瘤样本的病理分析得出了 ACTH 免疫阳性的肾上腺皮质癌的结论。我们的患者患有异位产生 ACTH 的肾上腺皮质癌。该病例强调，医生在评估罕见内分泌恶性肿瘤病例时应采取宽广的胸怀。© 2024。作者。

Adrenocortical carcinoma is a very rare endocrinopathy that has a poor prognosis and is frequently associated with ACTH-independent Cushing's syndrome. Despite having an adrenocortical carcinoma, our patient surprisingly had an ACTH-dependent Cushing's syndrome.A 26-year-old female presented with Cushing's syndrome and an abdominal mass. Imaging studies revealed an adrenal mass consistent with a high-grade malignancy. Laboratory workup showed hypercortisolism, hyperandrogenism, and hypokalemia with normal levels of metanephrines. Unexpectedly, her ACTH levels were remarkably elevated. The pathological analysis of a tumor sample was conclusive for adrenocortical carcinoma with immunopositivity for ACTH.Our patient suffered from an adrenocortical carcinoma that was ectopically producing ACTH. This case emphasizes that physicians should have a broad-minded approach when evaluating cases of rare endocrine malignancies.© 2024. The Author(s).