1986 Volume 77 Issue 9 Pages 1519-1523
We reported on a female patient with congenital adrenal hyperplasia, who developed an associated adrenocortical adenoma. She complained of clitoral enlargement at birth, but did not visit us until she was 21 years old. Laboratory data showed high urinary 17-KS which was supressed with 2mg of dexamethazone. RI examination showed the bilateral adrenal hyperplasia. She underwent vaginoplasty and clitorectomy, and followed by the replacement therapy with gluticorticoids. Six years later, a CT scan demonstrated a right adrenal tumor, 2cm in diameter. The right adrenalectomy was performed. The pathology of surgical specimens showed hyperplasia of adrenocortex associated with adrenocortical adenoma (6g). The case reported here suggested that the inadequete management might have a risk for neoplastic change in congenital adrenal hyperplasia.
