Skip to main content
Log in

17-hydroxyprogesterone response to ACTH in bilateral and monolateral adrenal incidentalomas

  • Published:
Journal of Endocrinological Investigation Aims and scope Submit manuscript

Abstract

The aim of our study was to assess the frequency of 21-hydroxylase deficiency, a cause of congenital adrenal hyperplasia (CAH), in incidentally discovered asymptomatic adrenal masses (incidentalomas) and to compare the prevalence of this enzymatic disorder in monolateral (M) and bilateral (B) forms. Twenty-seven patients with incidentalomas (12 M and 15 B) and 16 sex and age-matched controls (C) received synthetic adrenocorticotropin (ACTH, 250 μg iv). Plasma 17-OHprogesterone (17-OHP) and Cortisol were collected in basal conditions and after 30, 60, 90 minutes. Basal plasma 17-OHP in C [1.25±0.15 (0.61) ng/ml, mean±SE (SD)] was not significantly different from that in patients with M [0.85±0.13 (0.44) ng/ml] or B [0.94±0.23 (0.90) ng/ml] incidentalomas. After ACTH, 17-OHP levels significantly (p<0.05) increased in C, in M and B incidentalomas. However, the rise in plasma 17-OHP in C both in terms of peak [2.5±0.28 (1.1) ng/ml] and of AUC values [174+16 (64) ng/ml/min] was significantly lower than that observed in M [peak 6.32±1.66 (5.7) ng/ml, p<0.01; AUC 410±111 (385.5) ng/ml/min, p<0.01] and in B [peak 8.84±1.98 (7.65) ng/ml, p<0.001; AUC 613±149 (579.3), ng/ml/min, p<0.001] incidentalomas. Individual data indicated that while 17-OHP response to ACTH in C never reached 5 ng/ml (cut-off for normal response), 16 out of 27 patients with incidentalomas (59.2%) exceeded this value. Moreover, the abnormal response was more frequently observed in B (66.6%) than in M (50%) incidentalomas. Basal and stimulated plasma Cortisol did not differ among the three groups. In conclusion, our data indicate that in adrenal incidentalomas the endocrine pattern of 21-hydroxylase deficiency is very common and that this enzymatic defect is more frequent in bilateral than in monolateral lesions.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Osella G., Terzolo M., Borretta G., Magro G., Ali A., Piovesan A., Paccotti P., Angeli A. Endocrine evaluation in incidentally discovered adrenal masses (incidentalomas). J. Clin. Endocrinol. Metab. 79: 1532, 1994.

    Article  PubMed  CAS  Google Scholar 

  2. Ambrosi B., Peverelli S., Passini E., Re T., Ferrario R., Colombo P., Sartorio A., Faglia G. Abnormalities of endocrine function in patients with clinically “silent” adrenal masses. Eur. J. Endocrinol. 132: 422, 1995.

    Article  PubMed  CAS  Google Scholar 

  3. Reincke M., Nieke J., Krestin P., Saeger W., Allolio B., Winkelmann W. Preclinical Cushing’s Syndrome in adrenal “incidentalomas”: comparison with adrenal Cushing’s Syndrome. J. Clin. Endocrinol. Metab. 75: 826, 1992.

    PubMed  CAS  Google Scholar 

  4. Jockenhovel F., Kuck W., Hauffa B., Reinhardt W., Benker G., Lederbogen S., Ulbricht Th., Reinwein D. Conservative and surgical management of incidentally discoverd adrenal tumors (incidentalomas). J. Endocrinol. Invest. 15: 331,1992.

    Article  PubMed  CAS  Google Scholar 

  5. Kobayashi S., Seki T., Nonomura K., Gotoh T., Togashi M., Koyanagi T. Clinical experience of incidentally discovered adrenal tumor with particular reference to cortical function. J. Urol. 150: 8, 1993.

    PubMed  CAS  Google Scholar 

  6. Turton D.B., O’Brian J.T., Shakir K.M.M. Incidental adrenal nodules: association with exaggerated 17-hydroxyprogesterone response to adrenocorticotropic hormone. J. Endocrinol. Invest. 15: 789, 1992.

    Article  PubMed  CAS  Google Scholar 

  7. Gourmelen M., Pham-Huu-Trung M.T., Bredon M.G., Girard F. 17-hydroxyprogesterone in the cosyntropin test: results in normal and hirsute women and in mild congenital adrenal hyperplasia. Acta Endocrinol. (Copenh.). 90: 481, 1979.

    CAS  Google Scholar 

  8. Chrousos G.P., Loriaux D.L., Mann D., Cutler G.B. Late-onset 21-hydroxylase deficiency is an allelic variant of congenital adrenal hyperplasia characterized by attenuated clinical expression and different HLA haplotype associations. Horm. Res. 16: 193, 1982.

    Article  PubMed  CAS  Google Scholar 

  9. Rosenwaks Z., Lee P.A., Jones G.S., Migeon C.J., Wentz A.C. An attenuated form of congenital virilizing adrenal hyperplasia. J. Clin. Endocrinol. Metab. 49: 335, 1979.

    Article  PubMed  CAS  Google Scholar 

  10. New M.I., Dupont B., Pang S., Pollack M., Levine L.S. An uptake of congenital adrenal hyperplasia. Recent Prog. Horm. Res. 37: 105,1981.

    PubMed  CAS  Google Scholar 

  11. Gutai J.P., Kowarski A.A., Migeon C.J. The detection of the heterozygous carrier for congenital virilizing adrenal hyperplasia. J. Pediatr. 90: 924,1977.

    Article  PubMed  CAS  Google Scholar 

  12. Krensky A.M., Bongiovanni A.M., Marino J., Parks J., Tenore A. Identification of heterozygote carriers of congenital adrenal hyperplasia by radioimmunoassay of serum 17-OH progesterone. J. Pediatr. 90: 930, 1977.

    Article  PubMed  CAS  Google Scholar 

  13. Weil J., Bidlingmaier F., Sippel W.G., Butenandt O., Knorr D. Comparison of the two tests for heterozygocity in congenital adrenal hyperplasia (CAH). Acta Endocrinol. (Copenh.). 91: 109, 1979.

    CAS  Google Scholar 

  14. New M.I., Lorenzen F., Lerner A.J., Kohn B., Oberfield S.E., Pollack M.S., Dupont B., Stoner E., Levy J. D., Pang S., Levine L.S. Genotyping steroid 21-hydroxylase deficiency: hormonal reference data. J. Clin. Endocrinol. Metab. 57: 320, 1983.

    Article  PubMed  CAS  Google Scholar 

  15. Baskin H.J. Screening for late-onset congenital adrenal hyperplasia in hirsutism or amenorrhea. Arch. Intern. Med. 147: 847, 1987.

    Article  PubMed  CAS  Google Scholar 

  16. Fiet J., Gueux B., Gourmelen M., Kuttenn F., Vexiau P., Couillin P., Pham-HuuTrung MI, Villette JM., Raux-Demay MC, Galons H., Julien R. Comparison of basal and adrenocorticotropin-stimulated plasma 21-deoxycortisol and 17-hydroxyprogesterone values as biological markers of late-onset adrenal hyperplasia. J. Clin. Endocrinol. Metab. 66: 659,1988.

    Article  PubMed  CAS  Google Scholar 

  17. Racz K., Pinet F., Marton T., Szende B., Glaz E., Corvol P. Expression of steroidogenic enzyme messenger ribonucleic acids and corticosteroid production in aldosterone-producing and “non-functioning” adrenal adenomas. J. Clin. Endocrinol. Metab. 77: 677,1993.

    PubMed  CAS  Google Scholar 

  18. Symington T. Functional pathology of the human adrenal gland. The Williams and Wilkins Co., Baltimore, 1969, p. 151.

    Google Scholar 

  19. Dobbie J.W. Adrenocortical nodular hyperplasia: the ageing adrenal. J. Pathol. 99: 1, 1969.

    Article  PubMed  CAS  Google Scholar 

  20. Takayama K., Ohashi M., Haji M., Matsumoto T., Mihara Y., Kumazawa J., Kato K-l. Adrenocortical tumor in a patient with untreated congenital adrenocortical hyperplasia owing to 21-hydroxylase deficiency: characterization of steroidogenesis. J. Urol. 140: 803, 1988.

    PubMed  CAS  Google Scholar 

  21. Jaresch S., Kornely E., Kley H-K. Schlaghecke R. Adrenal incidentaloma and patients with homozygous or heterozygous congenital adrenal hyperplasia. J. Clin. Endocrinol. Metab. 74: 685, 1992.

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Giampaolo, B., Brogi, G., Vivaldi, M.S. et al. 17-hydroxyprogesterone response to ACTH in bilateral and monolateral adrenal incidentalomas. J Endocrinol Invest 19, 745–752 (1996). https://doi.org/10.1007/BF03347878

Download citation

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF03347878

Key words

Navigation