선천성 부신피질과형성증 환자에서 동반된 부신피질암
Adrenocortical Carcinoma in a Patient with Congenital Adrenal Hyperplasia
0
조회
0
다운로드
1
인용
0
참고문헌
키워드
초록
선천성 부신피질과형성증 환자에서 부신 피질 종양이 종종 발견되고 있는데 부신피질암은 매우 드물게 보고되고 있다. 생후 2개월에 피부의 과다 색소침착, 탈수와 저나트륨혈증, 고칼륨혈증으로 선천성 부신피질과형성증 진단을 받고 1세까지 스테로이드를 복용하다가 임의로 중단 후, 5세에 성조숙증이 발현하여 다시 스테로이드로 치료받던 환자가 16세경 혈뇨를 주소로 외래 방문하였다. 혈액 검사상 부신피질자극호르몬 33.5 pg/mL, 코티솔 5.2 μg/dL, 17-알파히드록시프로게스테론(17α-hydroxyprogesterone, 17-OHP) 6.3 ng/ mL, dehydroepiandrosterone sulfate (DHEA-S) 31.2 μg/dL 였고 초음파와 복부 전산화단층촬영상 왼쪽 부신에 7.7 × 5.6 × 6.2 cm의 종괴가 발견되어 수술적 제거를 받았고 조직학적으로 부신 피질 암종으로 진단받은 1례를 보고 한다.
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 9α-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 μg/dL, respectively. 17α-hydroxyprogesterone level was 6.3 ng/mL and dehydroepiandrosterone sulfate level was 31.2 μg/dL. Abdominal ultrasonography and computed tomography revealed a solid mass of 7.7 × 5.6 × 6.2 cm in the left adrenal gland. It was totally removed by surgery, and the histopathology was compatible with adrenocortical carcinoma.
참고문헌 (18)
- Miller RW, "Childhood cancer", Cancer, 75(1 Suppl), pp.395~405 (1995)
- Didolkar MS, "Natural history of adrenal cortical carcinoma: a clinicopathologic study of 42 patients", Cancer, 47, pp.2153~2161 (1981)
- Wooten MD, "Adrenal cortical carcinoma. Epidemiology and treatment with mitotane and a review of the literature", Cancer, 72, pp.3145~3155 (1993)
- Latronico AC, "Extensive personal experience: adrenocortical tumors", J Clin Endocrinol Metab, 82, pp.1317~1324 (1997)
- 진명헌, "소아에서 발생한 기능성 부신피질암", Korean Journal of Urology, 43(7), pp.638~640 (2002), 대한비뇨기과학회
- 노승림, "A case of adrenocortical adenoma following long-term treatment in a patient with congenital adrenal hyperplasia", Korean Journal of Pediatrics, 50(4), pp.302~305 (2007), 대한소아과학회
- Fraumeni JF Jr, "Adrenocortical neoplasms with hemihypertrophy, brain tumors, and other disorders", J Pediatr, 70, pp.129~138 (1967)
- Patocs A, "Hormonal evaluation and mutation screening for steroid 21-hydroxylase deficiency in patients with unilateral and bilateral adrenal incidentalomas", Eur J Endocrinol, 147, pp.349~355 (2002)
- Jaresch S, "Adrenal incidentaloma and patients with homozygous or heterozygous congenital adrenal hyperplasia", J Clin Endocrinol Metab, 74, pp.685~689 (1992)
- Nermoen I, "Two adults with adrenal myelolipoma and 21-hydroxylase deficiency", Case Report Med, 10, pp.1155~1158 (2009)
- Merke DP, "Adrenal lymphocytic infiltration and adrenocortical tumors in a patient with 21-hydroxylase deficiency", N Engl J Med, 340, pp.1121~1122 (1999)
- Sandrini R, "Childhood adrenocortical tumors", J Clin Endocrinol Metab, 82, pp.2027~2031 (1997)
- Wajchenberg BL, "Adrenocortical carcinoma: clinical and laboratory observations", Cancer, 88, pp.711~736 (2000)
- Lee PD, "Virilizing adrenocortical tumors in childhood: eight cases and a review of the literature", Pediatrics, 76, pp.437~444 (1985)
- Gröndal S, "Adrenocortical carcinoma. A retrospective study of a rare tumor with a poor prognosis", Eur J Surg Oncol, 16, pp.500~506 (1990)
- Luton JP, "Clinical features of adrenocortical carcinoma, prognostic factors, and the effect of mitotane therapy", N Engl J Med, 322, pp.1195~1201 (1990)
- Mantero F, "Investigation protocol: adrenal enlargement", Clin Endocrinol (Oxf), 50, pp.141~146 (1999)
- Sabbaga CC, "Adrenocortical carcinoma in children: clinical aspects and prognosis", J Pediatr Surg, 28, pp.841~843 (1993)