Tumor resection for adrenal gonadal syndrome caused by adrenal tumor

Adrenal syndrome, also known as adrenal gland abnormalities, can be caused by congenital adrenal dysfunction or acquired tumors. Sexual signs and external genital abnormalities are prominent clinical manifestations, and some can show atypical Cushing syndrome. As an independent disease, its surgical treatment should be clarified. This disease can occur in the embryonic stage, forming a deformity of the external genitalia, due to the congenital deficiency of various hydroxyl enzymes. These include C21 hydroxylase, C11 hydroxylase, 3-beta hydroxysteroid dehydrogenase, and C17 hydroxylase. According to the lack of special functions of enzymes, the synthesis of cortisol or aldosterone is blocked at different stages, which in turn stimulates excessive secretion of ACTH in the anterior pituitary and promotes hypertrophy of the adrenal cortex. Among the cortisol precursor intermediates that are not accumulated by hydroxylase, androgen is the main cause, resulting in abnormal development of genital malformations and abnormalities in fetuses and infants. This kind of patient accounts for about 90% of the adrenal gland syndrome, especially the female pseudohermaphroditism which is caused by the lack of simple C21 hydroxylase, which accounts for 60%. When adrenal cortical carcinoma or adenoma occurs in a female early childhood, the cortisol precursor secreted by the tumor, which is an excess of androgen, inhibits female hormones and is masculine. When such tumors occur in male infants, they are male precocious or giant penile, and such patients account for only 10% of adrenal syndrome. Pediatric and adult can also occur, but acquired functional cortical tumors only show abnormalities or atypical Cushing syndrome, no external genital malformation. A rare male feminization can also occur in acquired tumors. Of the 52 cases reported, adenomas accounted for 78% and cancers accounted for 22%. There have also been male feminists without adrenal tumors, and the true mechanism of cortical dysfunction remains unclear. Before performing surgery on adrenal syndrome, the tumor must be differentiated from cortical function defects. For the latter, the cortical hyperplasia of congenital adrenal syndrome must be further differentiated from Cushing syndrome. Because the treatment principles of the two are completely different from the surgical methods. Treatment of diseases: adrenal tumor secondary adrenal insufficiency Indication Tumor resection of adrenal gland syndrome caused by adrenal tumors is applicable to: 1. Anyone diagnosed as a tumor, regardless of the pathological nature of the cancer or adenoma, should be performed early adrenal tumor resection. 2. After resection of adrenal tumors, systemic symptoms and signs improve or disappear, but when the clitoris hypertrophy still does not subside, or coexisting labial fusion, a clitorisectomy or labia separation should be performed. Contraindications Male precocious giant penile disease, generally do not need surgery, if the tumor is cured by surgery, the giant penis can gradually disappear. If the cancer cannot be cured, it will not help the treatment of giant penile disease. Preoperative preparation Unilateral tumors, contralateral adrenal glands without atrophy, do not require hormone preparation before surgery. Other preparations are performed according to adrenal surgery. Surgical procedure 1. Adrenalectomy, see "Surgical treatment of functional adrenal cortical tumors and proliferation." 2. Hypertrophic clitoris resection 1 Make a horseshoe-shaped incision in the clitoris, pubic sinus and upper interlabial labyrinth. The ventral side is not cut to preserve the blood circulation and nerves of the clitoris head. 2 After the entire hypertrophic clitoris was peeled off to the pubic symphysis, the two clitoris feet were exposed and separated. 3 The vaginal head is placed in a semi-annular suture with the surrounding flap and the cutting edge of the vestibule.