Literature DB >> 31149069

BILATERAL ADRENAL AND TESTICULAR MASS IN A PATIENT WITH CONGENITAL ADRENAL HYPERPLASIA.

H Piskinpasa1, S Ciftci Dogansen1, F Kusku Cabuk2, D Guzey3, N A Sahbaz3, Y S Akdeniz1, M Mert1.   

Abstract

INTRODUCTION: Congenital adrenal hyperplasia (CAH) autosomal recessive disorders characterized by impaired adrenal steroid hormone synthesis. The most common form is 21-hydroxylase deficiency (21OHD). Testicular adrenal rest tumors (TARTs) are benign intratesticular masses that occur in male patients with CAH. TARTs are quite common in patients with 21OHD who were diagnosed late. CASE REPORT: A 41-year-old male patient with CAH secondary to 21OHD. The patient was referred to our endocrinology department from the andrology clinic for bilateral adrenal masses. Bilateral orchiectomy had been performed due to bilateral testicular masses and azoospermia two years ago. The pathology was reported as Leydig cell tumor. In hormonal assessment, baseline cortisol levels were low, 17-hydroxyprogesterone levels with baseline and after cosyntropin stimulation test were high. As a result of clinic and laboratory assessment, the patient was diagnosed with simple virilising CAH due to 21OHD and adrenal insufficiency. Then, prednisolone replacement was initiated. Bilateral orchiectomy tissue blocks of the patient were re-assessed and were considered TART. Magnetic resonance imaging revealed bilateral adrenal masses with 88x55 mm on the right and 41x22 mm on the left. Laparoscopic right adrenalectomy was applied and pathology was reported as myelolipoma. Follow-up of the mass on the left adrenal gland is ongoing. The patient is monitored under prednisolone and testosterone replacement therapy. Early diagnosis of CAH is very important because of the complications it causes. It should be considered especially for bilateral testicular and/or adrenal masses. Both fertility and adrenal glands can be protected with an early diagnosis and an early glucocorticoid replacement.

Entities:  

Keywords:  Bilateral adrenal masses; Congenital adrenal hyperplasia; Myelolipoma; Testicular adrenal rest tumors

Year:  2019        PMID: 31149069      PMCID: PMC6535321          DOI: 10.4183/aeb.2019.113

Source DB:  PubMed          Journal:  Acta Endocrinol (Buchar)        ISSN: 1841-0987            Impact factor:   0.877


  23 in total

1.  Adrenal nodules in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency: regression after adequate hormonal control.

Authors:  L R Giacaglia; B B Mendonca; G Madureira; K F Melo; C A Suslik; I J Arnhold; T A Bachega
Journal:  J Pediatr Endocrinol Metab       Date:  2001-04       Impact factor: 1.634

2.  Myelolipoma in the adrenal cortex; myeloadipose structures.

Authors:  A PLAUT
Journal:  Am J Pathol       Date:  1958 May-Jun       Impact factor: 4.307

3.  Clinical and pathological features associated with the testicular tumor of the adrenogenital syndrome.

Authors:  Richard A Ashley; Shawn M McGee; Philip A Isotaolo; Stephen A Kramer; John C Cheville
Journal:  J Urol       Date:  2007-02       Impact factor: 7.450

4.  Successful treatment with ICSI of infertility caused by azoospermia associated with adrenal rests in the testes: case report.

Authors:  H Murphy; C George; D de Kretser ; S Judd
Journal:  Hum Reprod       Date:  2001-02       Impact factor: 6.918

5.  Bilateral adrenal myelolipoma associated with adrenogenital syndrome.

Authors:  Manabu Sakaki; Hirofumi Izaki; Tomoharu Fukumori; Ryuichi Taue; Tomoteru Kishimoto; Hiro-Omi Kanayama
Journal:  Int J Urol       Date:  2006-06       Impact factor: 3.369

6.  High prevalence of testicular adrenal rest tumors, impaired spermatogenesis, and Leydig cell failure in adolescent and adult males with congenital adrenal hyperplasia.

Authors:  N M Stikkelbroeck; B J Otten; A Pasic; G J Jager; C G Sweep; K Noordam; A R Hermus
Journal:  J Clin Endocrinol Metab       Date:  2001-12       Impact factor: 5.958

Review 7.  Congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

Authors:  P C White; P W Speiser
Journal:  Endocr Rev       Date:  2000-06       Impact factor: 19.871

8.  Adrenal tumors associated with inadequately treated congenital adrenal hyperplasia.

Authors:  Jiansong Wang; Mary A Bissada; H Oliver Williamson; Hossam Yakout; Nabil K Bissada
Journal:  Can J Urol       Date:  2002-06       Impact factor: 1.344

9.  Asymptomatic testicular adrenal rest tumours in adolescent and adult males with congenital adrenal hyperplasia: basal and follow-up investigation after 2.6 years.

Authors:  Nike M M L Stikkelbroeck; Ad R M M Hermus; Harold M Suliman; Gerrit J Jager; Barto J Otten
Journal:  J Pediatr Endocrinol Metab       Date:  2004-04       Impact factor: 1.634

10.  Adrenal incidentaloma and patients with homozygous or heterozygous congenital adrenal hyperplasia.

Authors:  S Jaresch; E Kornely; H K Kley; R Schlaghecke
Journal:  J Clin Endocrinol Metab       Date:  1992-03       Impact factor: 5.958

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