Aldosterone-producing adrenocortical carcinoma: an unusual cause of Conn’s syndrome with an ominous clinical course

Abstract

Aldosterone-producing adrenocortical carcinoma (APAC) is a rare cause of hypertension often diagnosed late because of paucity of information. Thus, we delineated its clinical course and survival rates based on two cases referred to us that featured diverging clinical courses, and on a scrutiny of the literature since 1955 when the first case of APAC was identified. Data on demography, imaging results, hormonal assessment, histology, and clinical course were extracted independently by the investigators. We included in our database 58 cases, most presenting with Conn's syndrome. Plasma aldosterone levels were on average increased 14-fold; plasma renin activity was suppressed in 55% of cases. The tumor showed extremely variable size and weight, and no gender or side preference. Metastases were present in 10% of all cases at initial diagnosis and in an additional 48% of cases at follow-up. Median survival was 546 days (95% confidence interval (CI): 240-851); median time to either recurrence or death was 212 days (95% CI: 29-395). No clinical or histological signs predicted survival with Cox regression analysis. We concluded that, although an ominous course with a poor survival rate is common, no sign accurately predicts the course of APAC. Thus, molecular studies to identify diagnostic markers of survival are mandatory.

Aldosterone-Producing Adrenocortical Carcinoma with Co-Secretion of Cortisol and Estradiol: A Case Report

“…Only less than 40% would present as nonfunctioning tumors. 3 Aldosterone-producing adrenocortical carcinoma is even more uncommon presenting as uncontrolled hypertension and severe hypokalemia. The patient initially presented with uncontrolled hypertension and intermittent bilateral leg weakness due to hypokalemia.…”

“…6 Adrenalectomy was also reported to initially cure both hypokalemia and hypertension in most cases of aldosterone-producing adrenocortical carcinomas. 3 Open adrenalectomy is the standard surgical approach to patients with stages I to III adrenocortical carcinomas. Laparoscopic adrenalectomy may be done in patients with small tumors of less than 8 cm without preoperative evidence for invasiveness and suspected to be only potentially malignant.…”

“…2 Aldosterone-producing adrenocortical carcinoma is even more uncommon, comprising only <7% of all functioning adrenocortical carcinomas. 3 A complete hormonal evaluation is needed prior to surgery and is necessary to establish tumor markers for monitoring of tumor recurrence. Management involves complete surgical resection of the tumor followed by chemotherapy depending on the clinical staging.…”

Adrenal Carcinoma - A Case Report

“…High concentration of dehydroepiandrosterone sulfate (DHEA-S) is another clue suggesting adrenocortical carcinoma (ACC), whereas decreased serum DHEA-S concentrations are suggestive of a benign adenoma 7 Aldosteroneproducing adrenocortical carcinomas present with hypertension and pronounced hypokalemia (mean 8 However, severe hypokalemia is more likely to be caused by grossly elevated cortisol secretion, leading to insufficient renal cortisol inactivation by 11â-hydroxysteroid dehydrogenase type 2 with consecutive activation of the mineralocorticoid receptor. In many patients with a seemingly hormonally inactive adrenocortical carcinoma (ACC), high concentrations of steroid precursors like androstenedione or 17á-hydroxyprogesterone can often be demonstrated, thereby establishing the adrenocortical origin of the tumour.…”

Diagnosis dan Tata Laksana Sindrom Conn/Hiperaldosteronisme Primer: Sebuah Studi Kasus

“…Sekitar 1% pasien hiperaldosteronisme primer merupakan kasus karsinoma adrenal yang memproduksi aldosteron. 7 Indikasi untuk dilakukannya tes skrining yaitu pada pasien yang memenuhi satu atau bebrapa kriteria. Kriteria tersebut yaitu: 1) hipertensi stage 2 (sistolik/diastolik >160-179/ 100-109 mmHg) dan hipertensi stage 3 (>180/110 mmHg); 2) hipertensi resisten obat; 3) hipertensi dengan hipokalemia spontan atau yang diinduksi diuretik; 4) hipertensi dengan adrenal insidentaloma; 5) hipertensi dengan riwayat keluarga menderita hipertensi pada onset muda atau penyakit serebrovaskuler di usia muda (<40 tahun); dan 6) pasien dengan riwayat keluarga menderita hiperaldosteronisme primer.…”