New evidences on the regulation of SF-1 expression by POD1/TCF21 in adrenocortical tumor cells

OBJECTIVES: Transcription Factor 21 represses steroidogenic factor 1, a nuclear receptor required for gonadal development, sex determination and the regulation of adrenogonadal steroidogenesis. The aim of this study was to investigate whether silencing or overexpression of the gene Transcription Factor 21 could modulate the gene and protein expression of steroidogenic factor 1 in adrenocortical tumors. METHODS: We analyzed the gene expression of steroidogenic factor 1 using qPCR after silencing endogenous Transcription Factor 21 in pediatric adrenal adenoma-T7 cells through small interfering RNA. In addition, using overexpression of Transcription Factor 21 in human adrenocortical carcinoma cells, we analyzed the protein expression of steroidogenic factor 1 using Western blotting. RESULTS: Transcription Factor 21 knockdown increased the mRNA expression of steroidogenic factor 1 by 5.97-fold in pediatric adrenal adenoma-T7 cells. Additionally, Transcription Factor 21 overexpression inhibited the protein expression of steroidogenic factor 1 by 0.41-fold and 0.64-fold in two different adult adrenocortical carcinoma cell cultures, H295R and T36, respectively. CONCLUSIONS: Transcription Factor 21 is downregulated in adrenocortical carcinoma cells. Taken together, these findings support the hypothesis that Transcription Factor 21 is a regulator of steroidogenic factor 1 and is a tumor suppressor gene in pediatric and adult adrenocortical tumors.

Unusual presentation of the Conn's syndrome: a case report

A 26 -year- old woman presented with rhabdomyolysis secondary to severe hypokalemia. Hypertension and metabolic alkalosis could lead to the suspicion of primary aldosteronism, which was confirmed by a decreased plasma rennin, elevated plasma aldosterone levels and high aldosterone/rennin ratio additionally. Additionally adrenal computed tomography showed an adrenal tumour. Blood pressure and hypokalemia returned to the normal level after adrenaiectomy was performed. This case report highlights the need to be alert to the possibility of primary aldosteronism incidence in a patient presenting with rhabdomyolysis and hypertension caused by severe hypokalemia

Conn's Syndrome Associated with Behcet's Disease

A 39-yr-old woman, who had been treated for Behcet's disease for 4 yr, was admitted for further investigation of recently identified hypokalemia and hypertension. Suppressed plasma renin activity with elevated plasma aldosterone concentration and an anomalous postural decrease in plasma aldosterone were observed. An abdominal CT scan revealed a right adrenal mass. The patient was diagnosed with Conn's syndrome. The association of Conn's syndrome with Behcet's disease was thought to be coincidental. To our knowledge, this is the first case of Conn's syndrome associated with Behcet's disease.

Hiperaldosteronismo primário e tumores adrenais: experiência clínico-cirúrgica em nove pacientes / Cardiac Surgery in Childhood. Impact in Young People Quality of Life

PURPOSE--To report seven patients with diagnosis of primary aldosteronism, five of them due to aldosterone-producing adenoma (APA) and two due to idiopathic hyperaldosteronism (IHA), and two patients with adrenal non-producing tumors in order to discuss our experience on differential diagnosis and treatment of this hypertensive disease. METHODS--Hypokalemia and higher values of urinary potassium in the absence of diuretics were useful to the screening diagnosis of primary aldosteronism, reinforced by suppressed plasma renin activity either at rest and after deambulation and by higher values of plasma aldosterone. Computerized tomography in all patients and selenium-cholesterol scintigraphy were used to make the localization of tumors and differential diagnosis between APA and IHA. RESULTS--The patients with adrenal tumors were submitted to surgical treatment and the two patients with IHA were submitted to spironolacone therapy. After 1 to 5 years of follow-up, we observed cure of hypertension and hypokalemia in three patients after surgery and improvement of blood pressure control and normalization of serum potassium in the six others. CONCLUSION--The diagnosis of primary aldosteronism is important, besides its rarity, because surgical or appropriated clinical treatment provide cure of hypertension or improvement of blood pressure control in most of patients

Partial remission with transarterial embolization in a case of metastatic adrenal cortical carcinoma

A case of metastatic adrenal cortical carcinoma in which partial remission was achieved with transarterial embolization is presented as probably the first reported case in the literature to date. A 29-year-old woman was admitted because of adrenal cortical carcinoma which had not responded to mitotane. A left adrenalectomy with segmentectomy of the involved liver had been done previously. Abdominal computerized tomography demonstrated multiple large metastatic tumors in the liver. Transarterial embolization with Gelfoam and 20 mCi of 131I-labeled lipiodol was performed and resulted in a decrease in tumor size and biochemical parameters. Transarterial embolization can be one of the therapeutic modalities for metastatic adrenal cortical carcinomas.