Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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A case of testicular adrenal rest tumor in a male child with congenital adrenal hyperplasia

선천 부신 과다형성 환자에서 발생한 고환 부신 잔류 종양 1례

  • Kim, Joo Hwa (Department of Pediatrics, College of Medicine, Seoul National University) ;
  • Yun, Kyong Ah (Department of Pediatrics, College of Medicine, Seoul National University) ;
  • Shin, Choong Ho (Department of Pediatrics, College of Medicine, Seoul National University) ;
  • Yang, Sei Won (Department of Pediatrics, College of Medicine, Seoul National University)
  • 김주화 (서울대학교 의과대학 소아과학교실) ;
  • 윤경아 (서울대학교 의과대학 소아과학교실) ;
  • 신충호 (서울대학교 의과대학 소아과학교실) ;
  • 양세원 (서울대학교 의과대학 소아과학교실)
  • Received : 2008.04.19
  • Accepted : 2008.08.05
  • Published : 2008.09.15
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Abstract

Testicular adrenal rest tumors are a well-known complication in male patients with congenital adrenal hyperplasia. Corticosteroid suppressive therapy usually results in the regression of these tumors. We describe a patient with 21-hydroxylase deficiency who developed bilateral testicular masses. Despite steroid suppressive therapy, the tumors did not regress and hormonal control was poor. Consequently, bilateral partial orchiectomies were performed.

선천 부신 과다형성 환자에서 고환 부신 잔류 종양은 흔하게 발생한다. 대개 이 종양은 적절한 corticosteroid 억제 치료로 호전될 수 있다. 저자들은 양측성 고환 부신 잔류 종양을 보인 21-hydroxylase 결핍증 환아에게서 corticosteroid를 투여하였으나 반응하지 않아 고환 적출술을 시행한 사례를 경험하였기에 보고하는 바이다.

Keywords

References

  1. Speiser PW, White PC. Congenital adrenal hyperplasia. N Engl J Med 2003;349:776-88 https://doi.org/10.1056/NEJMra021561
  2. Barwick TD, Malhotra A, Webb JA, Savage MO, Reznek RH. Embryology of the adrenal glands and its relevance to diagnostic imaging. Clin Radiol 2005;60:953-9 https://doi.org/10.1016/j.crad.2005.04.006
  3. Stikkelbroeck NM, Otten BJ, Pasic A, Jager GJ, Sweep CG, Noordam K, et al. High prevalence of testicular adrenal rest tumors, impaired spermatogenesis, and Leydig cell failure in adolescent and adult males with congenital adrenal hyperplasia. J Clin Endocrinol Metab 2001;86:5721-8 https://doi.org/10.1210/jc.86.12.5721
  4. Claahsen-van der Grinten HL, Otten BJ, Sweep FC, Span PN, Ross HA, Meuleman EJ, et al. Testicular tumors in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency show functional features of adrenocortical tissue. J Clin Endocrinol Metab 2007;92:3674-80 https://doi.org/10.1210/jc.2007-0337
  5. Ashley RA, McGee SM, Isotaolo PA, Kramer SA, Cheville JC. Clinical and pathological features associated with the testicular tumor of the adrenogenital syndrome. J Urol 2007;177:546-9 https://doi.org/10.1016/j.juro.2006.09.041
  6. Cabrera MS, Vogiatzi MG, New MI. Long term outcome in adult males with classic congenital adrenal hyperplasia. J Clin Endocrinol Metab 2001;86:3070-8 https://doi.org/10.1210/jc.86.7.3070
  7. Claahsen-van der Grinten HL, Sweep FC, Blickman JG, Hermus AR, Otten BJ. Prevalence of testicular adrenal rest tumours in male children with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Eur J Endocrinol 2007;157:339-44 https://doi.org/10.1530/EJE-07-0201
  8. Kim M, Han HS. A case of congenital adrenal hyperplasia misdiagnosed as Leydig cell tumor. J Korean Soc Pediatr Endocrinol 2001;6:182-6
  9. Cho GR, Chueh HW, Kim JP, Jung JA, Yoo JH, Yoon SK, et al. A case of congenital adrenal hyperplasia combined with a testicular adrenal rest tumor and adrenal incidentaloma. J Korean Soc Endocrinol 2007;22:365-70 https://doi.org/10.3803/jkes.2007.22.5.365
  10. Avila NA, Shawker TS, Jones JV, Cutler GB Jr, Merke DP. Testicular adrenal rest tissue in congenital adrenal hyperplasia: serial sonographic and clinical findings. Am J Roentgenol 1999;172:1235-8 https://doi.org/10.2214/ajr.172.5.10227495
  11. Tiryaki T, Aycan Z, Hucumenoglu S, Atayurt H. Testis sparing surgery for steroid unresponsive testicular tumors of the congenital adrenal hyperplasia. Pediatr Surg Int 2005;21:853-5 https://doi.org/10.1007/s00383-005-1547-x
  12. Claahsen-van der Grinten HL, Otten BJ, Takahashi S, Meuleman EJ, Hulsbergen-van de Kaa C, Sweep FC, et al. Testicular adrenal rest tumors in adult males with congenital adrenal hyperplasia: evaluation of pituitary-gonadal function before and after successful testis-sparing surgery in eight patients. J Clin Endocrinol Metab 2007;92:612-5 https://doi.org/10.1210/jc.2006-1311
  13. Mangini J, Olson PR. Pathologic quiz case: a testicular mass in a 19-year-old man. Arch Pathol Lab Med 2000;124:785-6
  14. Merke DP, Bornstein SR, Avila NA, Chrousos GP. NIH conference. Future directions in the study and management of congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Ann Intern Med 2002;136:320-34 https://doi.org/10.7326/0003-4819-136-4-200202190-00012
  15. Claahsen-van der Grinten HL, Otten BJ, Hermus AR, Sweep FC, Hulsbergen-van de Kaa CA. Testicular adrenal rest tumors in patients with congenital adrenal hyperplasia can cause severe testicular damage. Fertil Steril 2008;89:597-601 https://doi.org/10.1016/j.fertnstert.2007.03.051
  16. Valla JS for the Group D' Etude en Urologie Pdiatrique. Testis-sparing surgery for benign testicular tumors in children. J Urol 2001;165:2280-3 https://doi.org/10.1016/S0022-5347(05)66184-8