Aldosterone-Secreting Adrenal Cortical Carcinoma. A Case Report and Review of the Literature

Abstract

Adrenal cortical carcinomas (ACC) are rare, typically aggressive malignant neoplasms with a reported incidence of 1-2 cases per 1 million population and account for 0.05-0.2 % of all malignancies. The majority of these tumors are functional with approximately 60 % of patients experiencing endocrine symptomatology typically characterized by Cushing's syndrome (40 %) or a mixed hormonal picture of Cushing syndrome seen in association with virilization. Rarely, patients present with a pure hormonal syndrome of feminization or hyperaldosteronism, 6 and 2.5 %, respectively. We report a case of a 76-year-old woman presenting with recently diagnosed hypertension secondary to primary hyperaldosteronism. The patient underwent laparoscopic converted to an open adrenalectomy and a diagnosis of adrenocortical carcinoma (aldosteronoma clinical) was rendered. This case and review of the literature highlight that while rare, aldosterone-secreting adrenal cortical carcinomas may occur. In this case report, we discuss the clinical presentation, pathologic findings, and review the literature for adrenal cortical carcinomas and aldosterone-secreting adrenal cortical carcinomas.

Experience with surgical treatment for primary malignant adrenal tumors

“…Клинические проявления гиперальдостерониз-ма: АГ, низкий уровень калия (характерные для этого слабость, мышечная боль, судороги), гипертермия, похудение. Как правило, это опухоли большого раз-мера, но встречаются и менее 3 см [15,16]. Чрезвычайно редко АКР представлен как опухоль мозгового вещества надпочечника -катехоламин-продуцирующая опухоль.…”

Reversing the established order: Should adrenal venous sampling precede cross‐sectional imaging in the evaluation of primary aldosteronism?

“…One commonly cited argument for performing imaging prior to AVS is the identification of large lesions which may represent adrenocortical carcinomas . However, these lesions are vanishing rare, accounting for only 1–2 cases per million, of which only approximately 2.5% are pure aldosterone‐secreting lesions . By these estimates, the potential of missing a case of adrenocortical carcinoma causing PA is approximately one in 40 million.…”

“…After biochemical diagnosis of PA has been established and confirmed, identification of the underlying cause of the aldosteronism is essential to guide treatment and management. Unilateral secretion of excess aldosterone is typically caused by an aldosterone secreting adenoma or unilateral hyperplasia; in very rare instances, it can represent an adrenocortical carcinoma . These patients are considered potential candidates for surgical resection.…”

Aldosterone to Renin Ratio as Screening Tool in Primary Aldosteronism

“…The two main causes of the disease are unilateral aldosterone producing adenomas and bilateral hyperplasia (sporadic and familial cases). Furthermore, there are less common forms of hyperaldosteronism such as unilateral adrenal hyperplasia, aldosterone-producing adrenocortical carcinomas and ectopic aldosterone-secreting tumors [12][13][14]. In PA, the high aldosterone concentrations are accompanied by inadequately low renin, because renin secretion is suppressed as a consequence of the negative feedback loop.…”