The adrenocartical carcinoma is a rare tumor with an estimated incidence of 1 case per 1,700,000 population. Despite its rarity, a large number of investigators have studied this neoplasm for the following two reasons. The First is the occasional difficulty of differentiation between careinoma and adenoma at the time of initial surgery, even by histopathologic examination. The other is its unique feature of corticosteroidogenesis. Steroid metabolism of adrenocortieal carcinoma is characterized by its low efficiency of steroid production due to deficiency of steroidogenic enzyme. The deficieney of 11 B-hydroxylase has been indieated in case of adrenoeortical carcinoma by several investigators. In this study, the serum level of cortisol was within normal range, but the serum level of 11-deoxycortisol was 50 times higher than normal. After the removal of tumor, the serum level of ll-deoxycortisol was markedly decm. In conclusion, the results from the this case suggest that measurement of serum 11-deoxy- cortisol may be a useful tool in the diagnosis and follow-up of adrenocortical carcinoma.
Adenoma ; Adrenocortical Carcinoma* ; Cortodoxone ; Diagnosis ; Follow-Up Studies ; Humans ; Hydrocortisone ; Incidence ; Metabolism ; Reference Values ; Research Personnel

Female pseudohermaphroditism due to adrenogenital syndrome is a condition in which individuals with a 46XX karyotype, negative H-Y antigen, normal mullerian duct derivatives, and a lack of development of w lffian duct structures differentiate partially as phenotypic males. They usually manifest masculinization of the external genitalia as a result of excess endogenous androgens. Most female pseudohermaphrodities have one of the types of congenital virilizing adrenal hyperplasia. Adrenogenital syndrome is inborn errors transmitted by autosomal recessive genes and may be due to defects in any of the enzymic steps in the biosynthesis of cortisol. Most affected individuals have a failure of 21-hydroxylation which prevents the conversion of 17 alpha-hydroxyprogesterone to 11-deoxycortisol. Such a defect in 21-hydroxylase leads to excessive production of adrenal androgens causing virilization. The treatment is early endocrinologic support and surgical reconstruction. There are some considerations in surgical repairs including normal sized clitoris with adequate erogenous sensation, sufficiently wide vaginal introitus and normal aesthetic appearance of the external genitalia for her normalized life as a female. We have experienced four cases of female pseudohermaphroditism due to adrenogenital syndrome. In two cases, we performed clitoroplasty with nerve sparing technique, vulvoplasty with mons pubis augmentation, vaginoplasty with posterior perineal flap and urethral reconstruction. In the other cases, we performed clitoroplasty with nerve sparing technique, vulvoplasty and vaginoplasty There was no specific operative complication and the result of the correction was satisfactory.
17-alpha-Hydroxyprogesterone ; 46, XX Disorders of Sex Development* ; Adrenogenital Syndrome* ; Androgens ; Clitoris ; Cortodoxone ; Female* ; Genes, Recessive ; Genitalia ; H-Y Antigen ; Humans ; Hydrocortisone ; Hyperplasia ; Karyotype ; Male ; Sensation ; Steroid 21-Hydroxylase ; Virilism