The Relative Prevalence of Testicular Adrenal Rest Tumors in Fewer than 20-Years-Old Boys with Congenital Adrenal Hyperplasia

Document Type : Original Article (s)

Authors

1 Assistant Professor, Department of Pediatrics, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran

2 Professor, Department of Pediatrics, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran

3 Student of Medicine, School of Medicine AND Student Research Committee, Isfahan University of Medical Sciences, Isfahan, Iran

Abstract

Background: Testicular adrenal rest tumor (TART) is the presence of unspecified adrenocortical cells in the testis of patients with congenital adrenal hyperplasia (CAH) and is similar to the hyperplastic adrenal glands. These tumors are usually benign; but, due to the tumor site in testicles, can lead to seminiferous tubule obstruction, gonadal dysfunction and infertility. Therefore, early diagnosis and treatment of tumors is important. This study aimed to determine the relative frequency of testicular adrenal rest tumor in children with congenital adrenal hyperplasia.Methods: This descriptive study was performed on 44 boys with congenital adrenal hyperplasia at the age of fewer than 20 years. Height, weight and blood pressure were measured; also, exact examination of testis and scrotum was done to diagnose palpable masses. For all patients, ultrasound of testis was performed by an experienced radiologist. Assessment of patients was based on height, weight and serum level of 17–hydroxy progesterone.Findings: Testicular adrenal rest tumor was reported in 2 patients (4.5%) that in one of them, bilateral tumors were reported in the upper pole of both testes. The other tumor was visible in the mediastinum of left testis. Both patients had types of salt loss, were in the age group of 15-20 years, were under hormonal control and appropriate treatment, and none of them had impaired gonadal function. The average tumor size was 14 mm. Palpable mass was not found in any of the patients. Serum levels of 17-hydroxyprogesterone was less in patients with testicular adrenal rest tumor than the others.Conclusion: The age group of the 2 boys with testicular adrenal rest tumor can show the correlation between duration of disease and the prevalence of the tumor. Examination has not enough accuracy to detect tumor and ultrasound is recommended for all the patients to be performed periodically.

Keywords

References

  1. Walker BR, Skoog SJ, Winslow BH, Canning DA, Tank ES. Testis sparing surgery for steroid unresponsive testicular tumors of the adrenogenital syndrome. J Urol 1997; 157(4): 1460-3.
  2. Robert MK, Richard EB, Hal BJ, Bonita MD. Nelson text book of pediatrics. 18th ed. Philadelphia, PA: Saunders Elsevier; 2007.
  3. Vanzulli A, DelMaschio A, Paesano P, Braggion F, Livieri C, Angeli E, et al. Testicular masses in association with adrenogenital syndrome: US findings. Radiology 1992; 183(2): 425-9.
  4. Tanner JM, Whitehouse RH. Clinical longitudinal standards for height, weight, height velocity, weight velocity, and stages of puberty. Arch Dis Child 1976; 51(3): 170-9.
  5. Mesiano S, Jaffe RB. Developmental and functional biology of the primate fetal adrenal cortex. Endocr Rev 1997; 18(3): 378-403.
  6. Urban MD, Lee PA, Migeon CJ. Adult height and fertility in men with congenital virilizing adrenal hyperplasia. N Engl J Med 1978; 299(25): 1392-6.
  7. Davis JM, Woodroof J, Sadasivan R, Stephens R. Case report: congenital adrenal hyperplasia and malignant Leydig cell tumor. Am J Med Sci 1995; 309(1): 63-5.
  8. Blie L, Sihelnik S, Bloom D, Stutzman R, chiadis J. Ultrasonographic analysis of chronic intratesticular pathology. J Ultrasound Med 1983; 2: 17-23.
  9. Avila NA, Premkumar A, Shawker TH, Jones JV, Laue L, Cutler GB, Jr. Testicular adrenal rest tissue in congenital adrenal hyperplasia: findings at Gray-scale and color Doppler US. Radiology 1996; 198(1): 99-104.
  10. White PC, Speiser PW. Congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Endocr Rev 2000; 21(3): 245-91.
  11. Dahl EV, Bahn RC. Aberrant adrenal contical tissue near the testis in human infants. Am J Pathol 1962; 40: 587-98.
  12. Augarten A, Weissenberg R, Pariente C, Sack J. Reversible male infertility in late onset congenital adrenal hyperplasia. J Endocrinol Invest 1991; 14(3): 237-40.
  13. Shanklin DR, Richardson AP, Rothstein G. Testicular hilar nodules in adrenogenital syndrome. the nature of the nodules. Am J Dis Child 1963; 106: 243-50.
  14. Greulich W, Pyle Sl. Radiographic Atlas of sketetal Development of the Hand and Wrist. California: Stanford university press; 1959.
  15. Swinkels LM, Ross HA, Benraad TJ. A symmetric dialysis method for the determination of free testosterone in human plasma. Clin Chim Acta 1987; 165(2-3): 341-9.
  16. Fleishmann W, Howard JE, Wilkins L. Macrogenitosomia precox associated with hyperplasia of the androgenic tissue of the adrenal and death from corticoadrenal insufficiency. Endocrnology 1940; 26: 385-95.
  17. Swinkels LM, van Hoof HJ, Ross HA, Smals AG, Benraad TJ. Low ratio of androstenedione to testosterone in plasma and saliva of hirsute women. Clin Chem 1992; 38(9): 1819-23.
  18. Avila NA, Shawker TS, Jones JV, Cutler GB Jr, Merke DP. Testicular adrenal rest tissue in congenital adrenal hyperplasia: serial sonographic and clinical findings. AJR Am J Roentgenol 1999; 172(5): 1235-8.
  19. Mugeon CJ, Parker K, Donohue PA. Congenital adrenal hyperplasia. In: Scriver CR, Beaudet AL, Sly ws, Valle D, Childs B, editors. The metabolic and molecular basis of inherited disease. New York, NY: MC Graw Hill; 1995. p. 2929-66.
  20. Stikkelbroeck NM, Suliman HM, Otten BJ, Hermus AR, Blickman JG, Jager GJ. Testicular adrenal rest tumours in postpubertal males with congenital adrenal hyperplasia: sonographic and MR features. Eur Radiol 2003; 13(7): 1597-603.
  21. 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(3): 339-44.
  22. New Ml, Vogiatzi MG, Cabrera M. High frequency of gonadul abnormalities in adultmales with classical congenital adrenal hyperplasia. 308. 1999.
  23. Graham LS. Celiac accessory adrenal glands. Cancer 1953; 6: 149-52.
  24. New MI, Wilson RC. Steroid disorders in children: congenital adrenal hyperplasia and apparent mineralocorticoid excess. Proc Natl Acad Sci U S A 1999; 96(22): 12790-7.
Journal of Isfahan Medical School
Volume 32, Issue 292 - Serial Number 292
May and June 2014
Pages 1019-1027

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History

  • Receive Date: 24 April 2012
  • Revise Date: 01 November 2024
  • Accept Date: 06 April 2022

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