Current diagnosis and treatment of Nelson’s syndrome Review article
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Published:
May 30, 2018
Keywords:
Nelson's syndrome, bilateral adrenalectomy, hyperpigmentation
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Abstract
Nelson’s syndrome is a rare condition of enlarging pituitary mass lesion producing ACTH that can develop after total bilateral adrenalectomy (TBA) in patients with Cushing’s disease. It is characterized by hyperpigmentation of the skin and mucous membranes and elevated plasma levels of ACTH. In this clinical review, the diagnosis and treatment strategies of patients with Nelson’s syndrome including surgery, radiation, and pharmacotherapy are presented
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1.
Ożga K, Zieliński G, Konecki Łukasz, Mazur M, Witek P. Current diagnosis and treatment of Nelson’s syndrome. OncoReview [Internet]. 2018May30 [cited 2024Nov.22];8(2(30):48-3. Available from: https://journalsmededu.pl/index.php/OncoReview/article/view/422
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Section
ENDOCRINOLOGY IN ONCOLOGY
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Copyright: © Medical Education sp. z o.o. This is an Open Access article distributed under the terms of the Attribution-NonCommercial 4.0 International (CC BY-NC 4.0). License (https://creativecommons.org/licenses/by-nc/4.0/), allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.
Address reprint requests to: Medical Education, Marcin Kuźma (marcin.kuzma@mededu.pl)
References
1. Nagesser SK, van Seters AP, Kievit J et al. Long-term results of total adrenalectomy for Cushing’s disease. World J Surg 2000; 24: 108-113.
2. Hopwood NJ, Kenny FM. Incidence of Nelson’s syndrome after adrenalectomy for Cushing’s disease in children: results of a nationwide survey. Am J Dis Child 1977; 131: 1353-1356.
3. De Tommasi C, Vance ML, Okonkwo DO et al. Surgical management of adrenocorticotropic hormone-secreting macroadenomas: outcome and challenges in patients with Cushing’s disease or Nelson’s syndrome. J Neurosurg 2005; 103: 825-830.
4. Nelson DHJW, Meakin JB, Dealy JB Jr et al. ACTH – producing tumor of the pituitary gland. N Engl J Med 1958; 259: 161-164.
5. Kelly PA, Samandouras G, Grossman AB et al. Neurosurgical treatment of Nelson’s syndrome. J Clin Endocrinol Metab 2002; 87: 5465-5469.
6. Rees JR, Zilva JF. Diabetes insipidus complicating total adrenalectomy. J Clin Pathol 1959; 12: 530-534.
7. Baranetsky NGRD, Zipser U, Goebelsmann U. Adrenocorticotropin – dependent virilizing paraovarian tumors in Nelson’s syndrome. J Clin Endocrinol Metab 1979; 49: 381-386.
8. Shekarriz M, Schneider C, Sabanegh E et al. Excessive testosterone production in a patient with Nelson syndrome and bilateral testicular tumors. Urol Int 1996; 56: 200-203.
9. van Aken MO, Pereira AM, van den Berg G et al. Profound amplification of secretoryburst mass and anomalous regularity of ACTH secretory process in patients with Nelson’s syndrome compared with Cushing’s disease. Clin Endocrinol 2004; 60: 765-772.
10. Munir A, Newell-Price J. Nelson’s Syndrome. Arq Bras Endocrinol Metabol 2007; 51: 1392-1396.
11. Banasiak MJ, Malek AR. Nelson syndrome: comprehensive review of pathophysiology diagnosis and management. Neurosurg Focus 2007; 23: E13.
12. Kimura N, Ishikawa T, Sasaki Y et al. Expression of prohormone convertase, PC2, in adrenocorticotropin producing thymic carcinoid with elevated plasma corticotropin-releasing hormone. J Clin Endocrinol Metab 1996; 81: 390-395.
13. Barber TM, Adams E, Ansorge O et al. Nelson’s syndrome. Eur J Endocrinol 2010; 163: 495-507.
14. Tang BN, Levivier M, Heureux M et al. 11C-methionine PET for the diagnosis and management of recurrent pituitary adenomas. Eur J Nucl Med Mol Imaging 2006; 33(2): 169-178.
15. Jeong SY, Lee SW, Lee HJ et al. Incidental pituitary uptake on whole-body 18F-FDG PET/CT: a multicentre study. Eur J Nucl Med Mol Imaging 2010; 37(12): 2334-2343.
16. Patel J, Eloy JA, Liu JK. Nelson’s syndrome: a review of the clinical manifestations, pathophysiology, and treatment strategies. Neurosurg Focus 2015; 38(2): E14.
17. Leber KA, Bergloff J, Pendl G. Dose-response tolerance of the visual pathways and cranial nerves of the cavernous sinus to stereotactic radiosurgery. J Neurosurg 1998; 88: 43-50.
18. Mauermann WJ, Sheehan JP, Chernavvsky DR et al. Gamma Knife surgery for adrenocorticotropic hormone producing pituitary adenomas after bilateral adrenalectomy. J Neurosurg 2007; 106: 988-993.
19. Heaney AP, Fernando M, Yong WH, Melmed S. Functional PPAR-g receptor is a novel therapeutic target for ACTH-secreting pituitary adenomas. Nat Med 2002; 8: 1281-1287.
20. Katznelson L. Sustained improvements in plasma ACTH and clinical status in a patient with Nelson’s syndrome treated with pasireotide LAR, a multireceptor somatostatin analog. J Clin Endocrinol Metab 2013; 98(5): 1803-1807.
21. Kasperlik-Załuska AA, Zgliczyński W, Jeske W et al. ACTH responses to somatostatin, valproic acid and dexamethasone in Nelson’s syndrome. Neuroendocrinol Letters 2005; 26: 709-712.
22. Hofland LJ, van der Hoek J, Feelders R et al. The multi-ligand somatostatin analogue SOM230 inhibits ACTH secretion by cultured human corticotroph adenomas via somatostatin receptor type 5. Eur J Endocrinol 2005; 152: 645-654.
23. Daniel E, Debono M, Caunt S et al. A prospective longitudinal study of Pasireotide in Nelson’s syndrome. Pituitary 2018; 21(3): 247-255.
24. Pivonello R, Faggiano A, Di Salle F et al. Complete remission of Nelson’s syndrome after 1-year treatment with cabergoline. J Endocrinol Invest 1999; 22(11): 860-865.
25. Shraga-Slutzky I, Shimon I, Weinshtein R. Clinical and biochemical stabilization of Nelson’s syndrome with long-term low-dose cabergoline treatment. Pituitary 2006; 9: 151-154.
26. Casulari LA, Naves LA, Mello PA et al. Nelson’s syndrome: complete remission with cabergoline but not with bromocriptine or cyproheptadine treatment. Hormone Res 2004; 62: 300-305.
27. Raverot G, Castinetti F, Jouanneau E et al. Pituitary carcinomas and aggressive pituitary tumours: Merits and pitfalls of temozolomide treatment. Clin Endocrinol 2012; 76(6): 769-775.
28. McCormack AI, Wass JA, Grossman AB. Aggressive pituitary tumours: the role of temozolomide and the assessment of MGMT status. Eur J Clin Investig 2011; 41(10): 1133-1148.
29. Dillard TH, Gultekin SH, Delashaw JB Jr. et al. Temozolomide for corticotroph pituitary adenomas refractory to standard therapy. Pituitary 2011; 14(1): 80-91.
30. Curto L, Torre ML, Ferrau F et al. Temozolomide-induced shrinkage of a pituitary carcinoma causing Cushing’s disease- report of a case and literature review. Sci World J 2010; 10: 2132-2138.
31. Koulouri O, Steuwe A, Gillett D et al. A role for C-methionine PET imaging in ACTH-dependent Cushing’s syndrome. Eur J Endocrinol 2015; 173: M107-M120.
2. Hopwood NJ, Kenny FM. Incidence of Nelson’s syndrome after adrenalectomy for Cushing’s disease in children: results of a nationwide survey. Am J Dis Child 1977; 131: 1353-1356.
3. De Tommasi C, Vance ML, Okonkwo DO et al. Surgical management of adrenocorticotropic hormone-secreting macroadenomas: outcome and challenges in patients with Cushing’s disease or Nelson’s syndrome. J Neurosurg 2005; 103: 825-830.
4. Nelson DHJW, Meakin JB, Dealy JB Jr et al. ACTH – producing tumor of the pituitary gland. N Engl J Med 1958; 259: 161-164.
5. Kelly PA, Samandouras G, Grossman AB et al. Neurosurgical treatment of Nelson’s syndrome. J Clin Endocrinol Metab 2002; 87: 5465-5469.
6. Rees JR, Zilva JF. Diabetes insipidus complicating total adrenalectomy. J Clin Pathol 1959; 12: 530-534.
7. Baranetsky NGRD, Zipser U, Goebelsmann U. Adrenocorticotropin – dependent virilizing paraovarian tumors in Nelson’s syndrome. J Clin Endocrinol Metab 1979; 49: 381-386.
8. Shekarriz M, Schneider C, Sabanegh E et al. Excessive testosterone production in a patient with Nelson syndrome and bilateral testicular tumors. Urol Int 1996; 56: 200-203.
9. van Aken MO, Pereira AM, van den Berg G et al. Profound amplification of secretoryburst mass and anomalous regularity of ACTH secretory process in patients with Nelson’s syndrome compared with Cushing’s disease. Clin Endocrinol 2004; 60: 765-772.
10. Munir A, Newell-Price J. Nelson’s Syndrome. Arq Bras Endocrinol Metabol 2007; 51: 1392-1396.
11. Banasiak MJ, Malek AR. Nelson syndrome: comprehensive review of pathophysiology diagnosis and management. Neurosurg Focus 2007; 23: E13.
12. Kimura N, Ishikawa T, Sasaki Y et al. Expression of prohormone convertase, PC2, in adrenocorticotropin producing thymic carcinoid with elevated plasma corticotropin-releasing hormone. J Clin Endocrinol Metab 1996; 81: 390-395.
13. Barber TM, Adams E, Ansorge O et al. Nelson’s syndrome. Eur J Endocrinol 2010; 163: 495-507.
14. Tang BN, Levivier M, Heureux M et al. 11C-methionine PET for the diagnosis and management of recurrent pituitary adenomas. Eur J Nucl Med Mol Imaging 2006; 33(2): 169-178.
15. Jeong SY, Lee SW, Lee HJ et al. Incidental pituitary uptake on whole-body 18F-FDG PET/CT: a multicentre study. Eur J Nucl Med Mol Imaging 2010; 37(12): 2334-2343.
16. Patel J, Eloy JA, Liu JK. Nelson’s syndrome: a review of the clinical manifestations, pathophysiology, and treatment strategies. Neurosurg Focus 2015; 38(2): E14.
17. Leber KA, Bergloff J, Pendl G. Dose-response tolerance of the visual pathways and cranial nerves of the cavernous sinus to stereotactic radiosurgery. J Neurosurg 1998; 88: 43-50.
18. Mauermann WJ, Sheehan JP, Chernavvsky DR et al. Gamma Knife surgery for adrenocorticotropic hormone producing pituitary adenomas after bilateral adrenalectomy. J Neurosurg 2007; 106: 988-993.
19. Heaney AP, Fernando M, Yong WH, Melmed S. Functional PPAR-g receptor is a novel therapeutic target for ACTH-secreting pituitary adenomas. Nat Med 2002; 8: 1281-1287.
20. Katznelson L. Sustained improvements in plasma ACTH and clinical status in a patient with Nelson’s syndrome treated with pasireotide LAR, a multireceptor somatostatin analog. J Clin Endocrinol Metab 2013; 98(5): 1803-1807.
21. Kasperlik-Załuska AA, Zgliczyński W, Jeske W et al. ACTH responses to somatostatin, valproic acid and dexamethasone in Nelson’s syndrome. Neuroendocrinol Letters 2005; 26: 709-712.
22. Hofland LJ, van der Hoek J, Feelders R et al. The multi-ligand somatostatin analogue SOM230 inhibits ACTH secretion by cultured human corticotroph adenomas via somatostatin receptor type 5. Eur J Endocrinol 2005; 152: 645-654.
23. Daniel E, Debono M, Caunt S et al. A prospective longitudinal study of Pasireotide in Nelson’s syndrome. Pituitary 2018; 21(3): 247-255.
24. Pivonello R, Faggiano A, Di Salle F et al. Complete remission of Nelson’s syndrome after 1-year treatment with cabergoline. J Endocrinol Invest 1999; 22(11): 860-865.
25. Shraga-Slutzky I, Shimon I, Weinshtein R. Clinical and biochemical stabilization of Nelson’s syndrome with long-term low-dose cabergoline treatment. Pituitary 2006; 9: 151-154.
26. Casulari LA, Naves LA, Mello PA et al. Nelson’s syndrome: complete remission with cabergoline but not with bromocriptine or cyproheptadine treatment. Hormone Res 2004; 62: 300-305.
27. Raverot G, Castinetti F, Jouanneau E et al. Pituitary carcinomas and aggressive pituitary tumours: Merits and pitfalls of temozolomide treatment. Clin Endocrinol 2012; 76(6): 769-775.
28. McCormack AI, Wass JA, Grossman AB. Aggressive pituitary tumours: the role of temozolomide and the assessment of MGMT status. Eur J Clin Investig 2011; 41(10): 1133-1148.
29. Dillard TH, Gultekin SH, Delashaw JB Jr. et al. Temozolomide for corticotroph pituitary adenomas refractory to standard therapy. Pituitary 2011; 14(1): 80-91.
30. Curto L, Torre ML, Ferrau F et al. Temozolomide-induced shrinkage of a pituitary carcinoma causing Cushing’s disease- report of a case and literature review. Sci World J 2010; 10: 2132-2138.
31. Koulouri O, Steuwe A, Gillett D et al. A role for C-methionine PET imaging in ACTH-dependent Cushing’s syndrome. Eur J Endocrinol 2015; 173: M107-M120.