ACTH-producing adrenocortical carcinoma: an exceedingly rare diagnosis.

Miguel Saraiva, Raquel da Inez Correia, Sérgio Xavier Azevedo, José Ricardo Brandão, José Carlos Oliveira, Isabel Palma
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Abstract

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.

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促肾上腺皮质激素分泌性肾上腺皮质癌:极为罕见的诊断。
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依赖性库欣综合征。尽管患有肾上腺皮质癌,但我们的患者却出人意料地出现了 ACTH 依赖性库欣综合征:病例报告:一名 26 岁的女性因库欣综合征和腹部肿块前来就诊。影像学检查发现肾上腺肿块与高级别恶性肿瘤一致。实验室检查显示皮质醇分泌过多、雄激素分泌过多和低钾血症,但甲肾上腺素水平正常。出乎意料的是,她的促肾上腺皮质激素水平明显升高。肿瘤样本的病理分析确定为肾上腺皮质癌,ACTH免疫阳性:结论:我们的患者患有异位分泌促肾上腺皮质激素的肾上腺皮质癌。本病例强调,医生在评估罕见的内分泌恶性肿瘤病例时,应具有广阔的视野。
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来源期刊
CiteScore
3.50
自引率
0.00%
发文量
46
审稿时长
11 weeks
期刊介绍: As the official publication of the National Cancer Institute, Cairo University, the Journal of the Egyptian National Cancer Institute (JENCI) is an open access peer-reviewed journal that publishes on the latest innovations in oncology and thereby, providing academics and clinicians a leading research platform. JENCI welcomes submissions pertaining to all fields of basic, applied and clinical cancer research. Main topics of interest include: local and systemic anticancer therapy (with specific interest on applied cancer research from developing countries); experimental oncology; early cancer detection; randomized trials (including negatives ones); and key emerging fields of personalized medicine, such as molecular pathology, bioinformatics, and biotechnologies.
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