Nelson Syndrome is rare today but can still be a challenge to manage. The reason for its rarity is that bilateral adrenalectomies are not performed as often as in the past.
Clinical features:
(1) The patient has a history of an ACTH-secreting pituitary adenoma (corticotrophinoma).
(2) Bilateral adrenalectomy was performed to control release of corticosteroids.
(3) Residual pituitary adenoma is present that produces high serum concentrations of ACTH.
(4) The patient develops a generalized hyperpigmentation involving skin and mucous membranes secondary to the elevated ACTH.
Factors that may identify a patient who will develop Nelson’s syndrome:
(1) residual pituitary tumor seen on imaging studies after surgery
(2) aggressive adenoma
(3) failure to administer adjuvant radiotherapy at the time of initial surgery
(4) rapid rise in serum ACTH concentrations
Therapy may include:
(1) resection of the pituitary adenoma (which may be localized with PET)
(2) radiotherapy of the pituitary adenoma
(3) long term therapy
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