Nelson's syndrome

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Phone:617-632-7753

Overview

Nelson's syndrome is the rapid enlargement of a pituitary adenoma that occurs after the removal of both adrenal glands.

Pathophysiology

Removal of both adrenal glands, or bilateral adrenalectomy, is an operation for Cushing's Disease. Removal of both adrenals eliminates production of cortisol, and the lack of cortisol's negative feedback can allow any preexisting pituitary adenoma to grow unchecked. Continued growth can cause mass effects due to physical compression of brain tissue, along with increased production of adrenocorticotrophic hormone (ACTH) and melanocyte stimulating hormone (MSH).

The common signs and symptoms include muscle weakness and skin hyperpigmentation due to excess MSH. Nelson's syndrome is rare because bilateral adrenalectomy is now only used in extreme circumstances.

References

• Nelson D, Meakin J, Thorn G (1960). "ACTH-producing pituitary tumors following adrenalectomy for Cushing's syndrome". Ann Intern Med. 52: 560–9. PMID 14426442.CS1 maint: Multiple names: authors list (link)