Skip to main content
Log in

Trans-sphenoidal microsurgical treatment of Nelson's syndrome

  • Trends in pituitary surgery
  • Published:
Neurosurgical Review Aims and scope Submit manuscript

Summary

Clinical features and the operative results in the author's personal series of ten cases with Nelson's syndrome are presented. There were nine females and one male patient with ages ranging from 20 to 44 years (mean 36 y). All patients presented with typical hyperpigmentation of the skin and had abnormally raised plasma ACTH levels. X-ray polytomography detected sellar abnormalities in 80% of the cases. Computerized tomography was positive for pituitary tumour in three patients. Trans-sphenoidal microsurgical removal of Nelson's ACTH adenomas were successful in seven of ten patients. The operative technique of radical-selective resection to achieve endocrinological cure is described. Pertinent literature is reviewed and the incidence of Nelson's syndrome, an issue of controversies regarding the pathogenesis, and the management of Nelson's adenomas are discussed.

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.

Institutional subscriptions

Similar content being viewed by others

References

  1. Aronin, N., D. T. Krieger: Sustained remission of Nelson's syndrome after stopping Cyproheptadine treatment. N. Engl. J. Med. 302 (1980) 453–455

    PubMed  Google Scholar 

  2. Barwich, D., F. Bahner: Pituitary tumors in adrenalectomized patients with Cushing's disease. In: Fahlbusch, R., K. von Werder: Treatment of Pituitary Adenomas (1st European Workshop at Rottach-Egern) pp. 326–330, Georg Thieme, Stuttgart 1978

    Google Scholar 

  3. Burke, C. W., F. H. Doyle, G. F. Joplin, R. N. Arnot, D. P. Maceriean, T. R. Fraser: Cushing's disease. Treatment by pituitary implantation of radioactive gold or yttrium seeds. Q. J. Med. 42 (1973) 693–714

    PubMed  Google Scholar 

  4. Cassar, J., F. H. Doyle, P. D. Lewis, K. Mashiter, S. Van Noorde, G. F. Joplin: Treatment of Nelson's syndrome by pituitary implantation of yttrium-90 or gold-198. Br. Med. J. 2 (1976) 269–272

    PubMed  Google Scholar 

  5. Cassar, J., K. Mashiter, G. F. Joplin: Cyproheptadine in Nelson's syndrome. Lancet (1976) 426

  6. Cohen, K. L., R. H. Noth, T. Pechinski: Incidence of pituitary tumors following adrenalectomy; A longterm follow-up study of patients treated for Cushing's disease. Arch. Intern. Med. 138 (1978) 575–579

    PubMed  Google Scholar 

  7. Conomy, J. P.: Nelson's syndrome: Chickens, eggs and sellar explosions. Arch. Intern. Med. 138 (1978) 691–692

    PubMed  Google Scholar 

  8. Ernest, I., H. Ekman: Adrenalectomy in Cushing's disease, A long term follow-up. Acta Endocrinol. [Suppl.] 160 (1972) 5–41

    Google Scholar 

  9. Espinoza, A., H. Nowakowski, R. Kautzky, D. Lüdecke: ACTH-determinations before and after selective removal of pituitary adenomas in Nelson's syndrome. Acta Endocrinol. [Suppl.] 173 (1973) 34

    Google Scholar 

  10. Espinoza, A., J. Hartmann, D. Schrader, H. Nowakowsky: Plasma-ACTH und Nelson-Tumoren bei bilateral adrenalektomierten Cushing-Patienten. Klin. Wochenschr. 53 (1975) 923–926

    PubMed  Google Scholar 

  11. Forni, C., M. A. Giovanelli: Nelson's syndrome in the paediatric age. Child's Brain 3 (1977) 309–314

    PubMed  Google Scholar 

  12. Garcia, J. H., H. Kalimo, J. R. Givens: Human adenohypophysis in Nelson's syndrome: Ultra structural and clinical study. Arch. Pathol. Lab. Med. 100 (1976) 253–258

    PubMed  Google Scholar 

  13. Glenn, F., H. Mannix: Diagnosis and prognosis of Cushing's syndrome. Surg. Gynecol. Obstet. 126 (1968) 765–776

    Google Scholar 

  14. Hardy, J.: Microsurgery of pituitary disorders. Royal College Lecture, Annals of Royal College of Physicians and Surgeons of Canada, 1980

  15. Hopwood, N., F. Kenny: Increased incidence of postadrenalectomy Nelson's syndrome in pediatric vs. adult Cushing's disease: nationwide study. Pediatr. Res. 9 (1975) 290

    Google Scholar 

  16. Horwith, M., P. E. Stokes: Cushing's syndrome, experience with total adrenalectomy. Adv. Intern. Med. 10 (1960) 259–295

    PubMed  Google Scholar 

  17. Ishibashi, M., T. Yamaji: Considerations on the pathogenesis of Nelson's syndrome. Proceedings of the 55th Annual Metting of the Japan Endocrine Society, Vol 58, P 1155, Kyoto 1982

  18. Jialal, I., N. L. Pillay: Cyproheptadine therapy in Cushing's disease and Nelson's syndrome. S. Afr. Med. J. 24 (1980) 305

    Google Scholar 

  19. Jordan, R. M., D. M., Cook, J. W. Kendall, C. W. Kerber: Nelson's syndrome and spontaneous pituitary tumor infarction. Arch. Intern. Med. 139 (1979) 340–342

    PubMed  Google Scholar 

  20. Kasperlik-Zaluska, A., W. Hartwig, J. Wislawski, G. Guiot: Nelson's syndrome following bilateral total adrenalectomy. Endokrynologia Polska 27 (1976) 401–408

    PubMed  Google Scholar 

  21. Krieger, D. T., M. Luria: Effectiveness of Cyproheptadine in decreasing plasma ACTH concentrations in Nelson's syndrome. J. Clin. Endocrinol. Metab. 43 (1976) 1179–1182

    PubMed  Google Scholar 

  22. Lamberts, S. W., J. C. Birkenhager: Bromocriptine in Nelson's syndrome and Cushing's disease. Lancet ii (1976) 881

    Google Scholar 

  23. Lawrence J. H., M. D. Okerlund, J. A. Linfoot: Heavy-particle treatment of Cushing's disease. N. Engl. J. Med. 285 (1971) 1263–1264

    PubMed  Google Scholar 

  24. Lawrence, J. H., J. A. Linfoot: Treatment of acromegaly, Cushing's disease and Nelson's syndrome. West. J. Med. 133 (1980) 197–202

    PubMed  Google Scholar 

  25. Laws, E. R., R. V. Randall, E. B. Kern, C. F. Abboud: Management of pituitary adenomas and related lesions with emphasis on trans-sphenoidal microsurgery, pp. 376. Appleton-Century-Crofts, New York 1982

    Google Scholar 

  26. Liddle G. W.: The adrenal cortex. In: Williams R. H.: Textbook of Endocrinology, pp. 233–283. W.B. Saunders Company, Philadelphia 1974

    Google Scholar 

  27. Linfoot, J. A., J. H. Lawrence, J. L. Born: The alpha particle or proton beam in radiosurgery of the pituitary gland for Cushing's disease. N. Engl. J. Med. 269 (1963) 597–601

    PubMed  Google Scholar 

  28. Mason, A. S., D. Greenbaum: Cushing's syndrome and skin pigmentation. Br. Med. J. 2 (1962) 445–448

    Google Scholar 

  29. McKenzie, A. D., H. W. McIntosh: Hyperpigmentation and pituitary tumor as sequelae of surgical treatment of Cushing's syndrome. Am. J. Surg. 110 (1965) 135–141

    PubMed  Google Scholar 

  30. Minagi, H., H. L. Steinbach: Roentgen aspects of pituitary tumors manifested after bilateral adrenalectomy for Cushing's syndrome. Radiology 90 (1968) 276–280

    Google Scholar 

  31. Mjølnerød O. K., H. N. Haugen, B. Bretheim: Surgical treatment of Cushing's syndrome in 72 patients. Scand. J. Urol. Nephrol. 8 (1974) 13–18

    PubMed  Google Scholar 

  32. Moore, T.J., R. G. Dluhy, G.H. Williams, J. P. Cain: Nelson's syndrome: Frequency, prognosis, and effect of prior pituitary irradiation. Ann. Intern. Med. 85 (1976) 731–734

    PubMed  Google Scholar 

  33. Müller, O. A., X. Baur, R. Fahlbusch, M. Madler, F. Marguth, C. Uhlig, P. C. Scriba: Diagnosis and treatment of ACTH-producing pituitary tumors. In: Fahlbusch, R., K. von Werder: Treatment of Pituitary Adenomas (1st European Workshop) pp. 343–351, Georg Thieme, Stuttgart 1978

    Google Scholar 

  34. Nabarro, J. D. N.: ACTH secreting pituitary tumours, J. R. Coll. Physicians Lond. 11 (1977) 363–375

    PubMed  Google Scholar 

  35. Nelson, D. H., J. W. Meakin, J. B. Dealy, D. D. Matson, K. Emerson, G. W. Thorn: ACTH-producing tumor of the pituitary gland. N. Engl. J. Med. 259 (1958) 161–164

    PubMed  Google Scholar 

  36. Nelson, D. H., J. W. Meakin, G. W. Thorn: ACTH-producing pituitary tumors following adrenalectomy for Cushing's syndrome. Ann. Intern. Med. 52 (1960) 560–569

    PubMed  Google Scholar 

  37. Nelson, D. H., J. G. Sprunt, R. B. Mims: Plasma ACTH determination in fifty-eight patients before or after adrenalectomy for Cushing's syndrome. J. Clin. Endocrinol. Metabol. 26 (1966) 722–728

    Google Scholar 

  38. Nowakowski, H., H. J. Breustedt, W. Mischke, H. R. Montz: Anterior pituitary function in hypothalamic Cushing's syndrome with and without ACTH-producing adenomas. In: Fahlbusch, R., K. von Werder: Treatment of Pituitary Adenomas (1st European Workshop) pp. 330–332, Georg Thieme, Stuttgart 1978

    Google Scholar 

  39. Orth, D. N., G. W. Liddle: Results of treatment in 108 patients with Cushing's syndrome. N. Engl. J. Med. 285 (1971) 243–247

    PubMed  Google Scholar 

  40. Plotz, C. M., A. I. Knowlton, C. Ragan: The natural history of Cushing's syndrome. Am. J. Med. 13 (1952) 597–614

    PubMed  Google Scholar 

  41. Salassa, R.M., T.P. Kearns, J. W. Kernohan, R. G. Sprague, C. S. MacCarty: Pituitary tumors in patients with Cushing's syndrome. J. Clin. Endocrinol. Metab. 19 (1959) 1523–1539

    PubMed  Google Scholar 

  42. Salassa, R. M., E. R. Laws, P. C. Carpenter, R. C. Northcutt: Transsphenoidal removal of pituitary microadenoma in Cushing's disease. Mayo Clin. Proc. 53 (1978) 24–28

    PubMed  Google Scholar 

  43. Salcman, M., J. H. Garcia, J. D. Abbott, L. G. Martin: Nelson's syndrome: one disease or two? Neurosurgery 5 (1979) 480–484

    PubMed  Google Scholar 

  44. Sasano, N.: Pathology of pituitary adenoma. In: Sano, K., K. Takakura, T. Fukushima: Functioning pituitary adenoma, its pathology and clinic (Proceedings of the First Workshop on Pituitary Tumors), pp. 15–24. Tokyo 1980

  45. Schteingart, D. E.: Cushing's disease: An update. Drug Therapy (Hosp) Feb. (1978) 53–63

  46. Taft, P., F.I.R. Martin, R. Melik: Cushing's syndrome: A review of the response to treatment in 42 patients. Aust. Ann. Med. 19 (1970) 295–303

    PubMed  Google Scholar 

  47. Teramoto, A., T. Fukushima, K. Takakura, K. Sano, R. Y. Osamura: Immunohistochemical classification of pituitary adenomas. In: Brock, M.: Modern Neurosurgery, vol 1, pp. 160–172. Springer, Berlin—Heidelberg—New York 1982

    Google Scholar 

  48. Turkington, R. W.: Secretion of prolactin by patients with pituitary and hypothalamic tumors. J. Clin. Ednocrinol. 34 (1972) 159–164

    Google Scholar 

  49. Tyrrell, J. B., M. Lorenzi, J. E. Gerich. P. H. Forsham: Inhibition by somatostation of ACTH secretion in Nelson's syndrome. J. Clin. Endocrinol. Metab. 40 (1975) 1125–1127

    PubMed  Google Scholar 

  50. Tyrrell, J. B., R. M. Brooks. P. A. Fitzgerald, P. B. Cofoid, P. H. Forsham, C. B. Wilson: Cushing's disease. Selective trans-sphenoidal resection of pituitary microadenomas. N. Engl. J. Med. 298 (1978) 753–758

    PubMed  Google Scholar 

  51. Ullrich, I., G. Lizarralde: Nelson's syndrome: Case report and review of the literature. South Med. J. 70 (1977) 1444–1446

    PubMed  Google Scholar 

  52. Vaisrub, S.: Cushing's syndrome, still a conundrum. JAMA 240 (1978) 865

    PubMed  Google Scholar 

  53. Weinstein, M., B. Tyrrell, T.H. Newton: The sella turcica in Nelson's syndrome. Radiology 118 (1976) 363–365

    PubMed  Google Scholar 

  54. Welbourn, R. B., D. A. D. Montgomery, T.L. Kennedy: Natural history of treated Cushing's syndrome. Br. J. Surg. 58 (1971) 1–16

    PubMed  Google Scholar 

  55. Wild, W., G. L. Nicolis, J. T. Gabrilove: Appearance of Nelson's syndrome despite pituitary irradiation prior to bilateral adrenalectomy for Cushing's syndrome. M. Sinai J. Med. NY 40 (1973) 68–71

    Google Scholar 

  56. Wilson, C. B., L. C. Dempsey: Transsphenoidal microsurgical removal of 250 pituitary adenomas. J. Neurosurg. 48 (1978) 13–22

    PubMed  Google Scholar 

  57. Wilson, C. B.: Pituitary adenomas. In: Wilson, C. B., J. T. Hoff: Current Surgical Management of Neurologic Disease, p. 355. Churchill Livingston, New York 1980

    Google Scholar 

  58. Wilson, C. B., J. B. Tyrrell, P. A. Fitzgerald, L. H. Pitts: Cushing's disease and Nelson's syndrome. Clin. Neurosurg. 27 (1980) 19–30

    PubMed  Google Scholar 

  59. Young, L. M., G. H. Lim, G. B. Forbes, M. F. Brysoe: Postadrenalectomy pituitary adenoma (Nelson's syndrome) in childhood: Clinical and roentgenologic detection. Am. J. Roentgenol. 126 (1976) 550–559

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Fukushima, T. Trans-sphenoidal microsurgical treatment of Nelson's syndrome. Neurosurg. Rev. 8, 185–194 (1985). https://doi.org/10.1007/BF01815443

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF01815443

Keywords

Navigation