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Updated: Jun 21, 2025
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Published on: September 15, 2017
ACTH-producing adrenocortical carcinoma: an exceedingly rare diagnosis
Miguel Saraiva1, Raquel da Inez Correia2, Sérgio Xavier Azevedo3
1Department of Endocrinology, Diabetes and Metabolism, Centro Hospitalar Universitário de Santo António, Largo Professor Abel Salazar, 4099-001, Porto, Portugal. miguelsaraiva.md@gmail.com.
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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