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Beidseitige testikuläre Raumforderungen im Rahmen des adrenogenitalen Syndroms

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Zusammenfassung

Testikuläre Raumforderungen in Verbindung mit dem adrenogenitalen Syndrom (AGS) stellen sowohl klinisch als auch pathologisch ein diagnostisches Problem dar. Die wichtigsten Differentialdiagnosen bilden hierbei der Leydig-Zelltumor und der benigne Tumor aufgrund der Ektopie adrenaler Zellen.

Wir berichten von einem 14-jährigen Jungen mit AGS. Im Rahmen der Abklärung seiner Kleinwüchsigkeit und beidseitiger Raumforderungen der Hoden wurde ein 21-Hydroxylasemangel festgestellt. Palpatorisch waren die Hoden unempfindlich, fest und von knotiger Struktur. Die Serumspiegel des adrenokortikotropen Hormons (ACTH), des 17α-Hydroxyprogesterons (17α-OHP), des Testosterons und des Aldosterons waren erhöht. Unter Kortikosteroidtherapie kam es zu einer Normalisierung der Serummarker und zu einer Regression der testikulären Raumforderungen.

Testikuläre Tumoren vergesellschaftet mit einem AGS treten typischerweise beidseitig auf und entwickeln sich durch fehlende oder inadäquate medikamentöse Therapie des AGS.

Abstract

Testicular masses in male individuals with the adrenogenital syndrome (AGS) are a clinical and pathological diagnostic dilemma. The major differential diagnosis of gonadal nodules in this setting includes interstitial Leydig cell tumors and secondary benign tumors possibly of adrenal origin.

We report a case of adrenogenital syndrome occurring in a 14-year-old boy. Examinations to clarify the cause of his dwarfism and bilateral testicular masses revealed 21-hydroxylase deficiency. The testes were not tender and were firm and nodular on palpation. The serum levels of adrenocorticotrophic hormone (ACTH), 17 alpha-hydroxyprogesterone (17-alpha-OHP), testosterone, and aldosterone were found to be elevated. Under corticosteroid therapy the serum marker abnormalities were corrected and there was gradual regression of the tumor lesions in both testes.

Testicular tumors with adrenogenital syndrome are typically bilateral and develop in untreated or inadequately treated males with AGS.

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Literatur

  1. Avila NA, Premkumar A, Merke DP (1999) Testicular adrenal rest tissue in congenital adrenal hyperplasia: comparison of MR imaging and sonographic findings. Am J Roentgenol 172: 1003–1006

    CAS  Google Scholar 

  2. Blumberg-Tick J, Boudou P, Nahoul K, Schaison G (1991) Testicular tumors in congenital adrenal hyperplasia: steroid measurements from adrenal and spermatic veins. J Clin Endocrinol Metab 73: 1129–1133

    Google Scholar 

  3. Cabrera MS, Vogiatzi MG, New MI (2001) Long term outcome in adult males with classic congenital adrenal hyperplasia. J Clin Endocrinol Metab 86: 3070–3078

    Google Scholar 

  4. Clark RV, Albertson BD, Munabi A, Cassorla F, Aguilera G, Warren DW, Loriaux DL (1990) Steroidogenic enzyme activities, morphology, and receptor studies of a testicular adrenal rest in a patient with congenital adrenal hyperplasia. J Clin Endocrinol Metab 70: 1408–1413

    CAS  PubMed  Google Scholar 

  5. Grem JL, Robins HI, Wilson KS, Gilchrist K, Trump DL (1986) Metastatic Leydig cell tumor of the testis. Report of three cases and review of the literature. Cancer 58: 2116–2119

    CAS  PubMed  Google Scholar 

  6. Keely EJ, Matwijiw I, Thliveris JA, Faiman C (1993) Congenital adrenal hyperplasia with testicular tumors, aggression, and gonadal failure. Urology 41: 346–349

    CAS  PubMed  Google Scholar 

  7. Knudsen JL, Savage A, Mobb GE (1991) The testicular 'tumour' of adrenogenital syndrome--a persistent diagnostic pitfall. Histopathology 19: 468–470

    CAS  PubMed  Google Scholar 

  8. Mangini J, Olson PR (2000) Pathologic quiz case: a testicular mass in a 19-year-old man. Arch Pathol Lab Med 124: 785–786

    CAS  PubMed  Google Scholar 

  9. Miller WL, Levine LS (1987) Molecular and clinical advances in congenital adrenal hyperplasia. J Pediatr 111: 1–17

    CAS  PubMed  Google Scholar 

  10. Murphy H, George C, de Kretser D, Judd S (2001) Successful treatment with ICSI of infertility caused by azoospermia associated with adrenal rests in the testes: case report. Hum Reprod 16: 263–267

    Article  CAS  PubMed  Google Scholar 

  11. Oliva E, Young RH (2000) Paratesticular tumor-like lesions. Semin Diagn Pathol 17: 340–358

    CAS  PubMed  Google Scholar 

  12. Prader A (1953) Die Kortisondauerbehandlung des kongenitalen adrenogenitalen Syndroms. Helv Paediat Acta 8: 386–423

    Google Scholar 

  13. Proto G, Di Donna A, Grimaldi F, Mazzolini A, Purinan A, Bertolissi F (2001) Bilateral testicular adrenal rest tissue in congenital adrenal hyperplasia: US and MR features. J Endocrinol Invest 24: 529–531

    Google Scholar 

  14. Rich MA, Keating MA (2000) Leydig cell tumors and tumors associated with congenital adrenal hyperplasia. Urol Clin North Am 27: 519–528

    Google Scholar 

  15. Rich MA, Keating MA, Levin HS, Kay R (1998) Tumors of the adrenogenital syndrome: an aggressive conservative approach. J Urol 160: 1838–1841

    CAS  PubMed  Google Scholar 

  16. Rutgers JL, Young RH, Scully RE (1988) The testicular "tumor" of the adrenogenital syndrome. A report of six cases and review of the literature on testicular masses in patients with adrenocortical disorders. Am J Surg Pathol 12: 503–513

    CAS  PubMed  Google Scholar 

  17. Stikkelbroeck NM, Otten BJ, Pasic A, Jager GJ, Sweep CG, Noordam K, Hermus AR (2001) 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 86: 5721–5728

    Google Scholar 

  18. Ulbright TM, Srigley JR, Hatzianastassiou DK, Young RH (2002) Leydig cell tumors of the testis with unusual features: adipose differentiation, calcification with ossification, and spindle-shaped tumor cells. Am J Surg Pathol 26: 1424–1433

    Article  PubMed  Google Scholar 

  19. Walker BR, Skoog SJ, Winslow BH, Canning DA, Tank ES (1997) Testis sparing surgery for steroid unresponsive testicular tumors of the AGS. J Urol 157: 1460–1463

    CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Klinik für Urologie, Carl-Thiem-Klinikum, Cottbus

    C. Helke, M. May, M. Seehafer & B. Hoschke

  2. Klinik für Kinder- und Jugendmedizin, Carl-Thiem-Klinikum, Cottbus

    S. Stolz & T. Erler

  3. Urologische Klinik, Carl-Thiem-Klinikum, Lehrkrankenhaus der Universitätsklinik Charité zu Berlin, Thiemstraße 111, 03048, Cottbus

    C. Helke

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  1. C. Helke
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  2. M. May
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  4. M. Seehafer
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Correspondence to C. Helke.

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Helke, C., May, M., Stolz, S. et al. Beidseitige testikuläre Raumforderungen im Rahmen des adrenogenitalen Syndroms. Urologe [A] 42, 1607–1610 (2003). https://doi.org/10.1007/s00120-003-0449-6

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  • DOI: https://doi.org/10.1007/s00120-003-0449-6

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