促肾上腺皮质激素（ACTH）产生的肾上腺皮质癌：一种极其罕见的诊断。
ACTH-producing adrenocortical carcinoma: an exceedingly rare diagnosis.
机构信息
Department of Endocrinology, Diabetes and Metabolism, Centro Hospitalar Universitário de Santo António, Largo Professor Abel Salazar, 4099-001, Porto, Portugal.
Department of General Surgery, Centro Hospitalar Universitário de Santo António, Largo Professor Abel Salazar, 4099-001, Porto, Portugal.
出版信息
J Egypt Natl Canc Inst. 2024 Jul 15;36(1):24. doi: 10.1186/s43046-024-00229-z.
BACKGROUND
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.
CASE REPORT
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.
CONCLUSIONS
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.
背景
肾上腺皮质癌是一种非常罕见的内分泌疾病，预后不良，常伴有 ACTH 非依赖性库欣综合征。尽管患有肾上腺皮质癌，但我们的患者出人意料地患有 ACTH 依赖性库欣综合征。
病例报告
一名 26 岁女性因库欣综合征和腹部肿块就诊。影像学研究显示符合高级别恶性肿瘤的肾上腺肿块。实验室检查显示皮质醇增多症、高雄激素血症和低钾血症，而甲氧基肾上腺素水平正常。出乎意料的是，她的 ACTH 水平显著升高。肿瘤样本的病理分析明确诊断为肾上腺皮质癌，免疫组化显示 ACTH 阳性。
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持开放心态。
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