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Ann Pediatr Endocrinol Metab > Volume 15(3); 2010 > Article
Adrenocortical Carcinoma in a Patient with Congenital Adrenal Hyperplasia.
Min sung Cho, Hye Young Jin, Jin Ho Choi, Dae Yeon Kim, Jung joo Lee, Han Wook Yoo
1Department of Pediatrics, Asan Medical center, University of Ulsan College of Medicine, Seoul, Korea. hwyoo@amc.seoul.kr
2Department of Surgery, Asan Medical center, University of Ulsan College of Medicine, Seoul, Korea.
3Department of Pathology, Asan Medical center, University of Ulsan College of Medicine, Seoul, Korea.
Abstract
Adrenocortical carcinoma is a very rare condition in childhood. There are only a few reports about adrenocortical carcinomas associated with congenital adrenal hyperplasia. A two-month-old male baby presented at the local clinic with skin hyperpigmentation, dehydration, hyponatremia, and hyperkalemia. He was diagnosed with congenital adrenal hyperplasia and had been treated with hydrocortisone and 9alpha-fludrocortisone until 1 year of age. At age 5.8 years, he visited an outpatient clinic because of tall stature and secondary sexual characteristics, and hydrocortisone was administered. At age 16 years he was admitted for treatment of an adrenal tumor, incidentally detected during the evaluation of hematuria. Serum adrenocorticotrophic hormone and cortisol levels were 33.5 pg/mL and 5.2 microg/dL, respectively. 17alpha-hydroxyprogesterone level was 6.3 ng/mL and dehydroepiandrosterone sulfate level was 31.2 microg/dL. Abdominal ultrasonography and computed tomography revealed a solid mass of 7.7 x 5.6 x 6.2 cm in the left adrenal gland. It was totally removed by surgery, and the histopathology was compatible with adrenocortical carcinoma.
Keywords: Adrenocortical carcinoma;Adrenal hyperplasia, congenital


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