Case Rep Endocrinol 2012

Case Rep Endocrinol 2012. outcomes. Surgery can be preceded by adrenolytic agents such as ortho paraprime dichloro diphenyl dichloroethane (Mitotane), ketoconazole or by 7-Chlorokynurenic acid sodium salt aromatase inhibitors, but till now there is not any controlled study to compare the benefit of different drugs. New anti-estrogens can be used too, but their results need to be confirmed in malignant tumors resistant to classical chemotherapy and to conventional radiotherapy. Targeted therapy can be used too, as in other adrenocortical tumors, but the results need to be confirmed. = 33) only two were females. In children there were 10 boys and seven girls. The median age was 42.8 years (19C77) for adults and 5.5 (1.5C14) for children. For Moreno aromatase activity is higher in tissues obtained from FAT than in normal adults adrenal tissues. So, excessive androgens transformation to estrogens leads to an increase in estrogens/androgens ratio responsible for Sox2 gynecomastia and other hypogonadism features and inhibition of the hypothalamic-pituitary-gonadal axis inducing a lack of luteinizing hormone-releasing hormone pulsatility and low luteinizing hormone (LH) and follicle-stimulating hormone (FSH) secretion.[10,11,29,48] Apart from plasma or urinary cortisol, aldosterone may be increased too. Increase in some precursors such as progesterone, 17-hydroxyprogesterone (17-OHP), deoxycorticosterone, D4androstenedione (D4A), dehydroepiandrosterone (DHEA) and DHEA sulfate plead 7-Chlorokynurenic acid sodium salt for malignancy as in other adrenal tumors. Increase in precursors is explained by an acquired deficiency in adrenal enzymes such as 21-hydroxylase, 11-hydroxylase, or 3-beta-hydroxysteroid dehydrogenase.[15] Decrease in testosterone observed in the majority of adult males is probably due to several mechanisms. The first one is the inhibition of FSH and LH secretion and pulsatility due to high concentrations of estrogens at the hypothalamic level.[10,12] The second mechanism can be explained by leydig’s cells inhibition secondary to estrogens high concentrations.[10] The third one is related to an increase in sex hormone binding globulin (SHBG) secondary to estrogens excess too. As SHBG has a great affinity for testosterone, the consequence will be a decrease in free testosterone with hypogonadism exaggeration.[15] High blood pressure is related to an increase in renin precursors synthesis by the liver. As a result, angiotensin I is converted to angiotensin II leading to aldosterone high concentrations.[15] In rare cases, it can be a result of aldosterone high secretion by the tumor itself. Heart troubles, especially cardiac insufficiency with or without ventricular tachycardia are related 7-Chlorokynurenic acid sodium salt to massive estrogens concentration while physiological concentrations are usually cardio-protective.[4] Radiological findings Radiological signs are important to consider although they are not specific of tumors secreting estrogens. As in other adrenal tumors, plain radiographs, excretory urography and nephrotomograms were used in the past to show an abdominal mass compressing or displacing the kidney. Echosonography which is a noninvasive exploration is replacing old explorations as it usually shows the tumor in the supra renal area and demonstrates or not kidney and/or other adjacent organs involvement. Lymph nodes and/or liver metastases can also be shown by echosonography which can also demonstrate vena cava thrombosis. Computed tomography (CT) shows the tumor and provides guidance for malignancy such as: Tumor size 6 cm, inhomogeneous aspect and poor limited margins, spontaneous density 10 Hounsfield units, intense enhancement of the tumor after injection, large areas of necrosis and/or micro-calcifications, and compression of adjacent organs. CT scan can help for fine nodule aspiration too in order to prove the diagnosis as in one of our case, and to confirm estrogen secretion by immunostaining. The positron emission tomography (PET) scan could help too for a precocious diagnosis of the adrenal tumor and its metastases especially.