Nelson’s syndrome: physiopathology, management and prognosis

Abstract

A classical description of a postadrenalectomy syndrome in Cushing’s disease, described in 1958 by Don Nelson, included a deep skin hyperpigmentation, presence of a large pituitary tumor, high plasma adrenocorticotropic hormone levels and visual field deficits. The main pathophysiological mechanism of Nelson’s syndrome development is a loss of feedback control of hypercortisolemia on corticotroph as a consequence of the removal of hyperactive adrenal glands. Modern techniques of imaging, computed tomography and MRI, made early detection of pituitary tumor at the stage of microadenoma possible. Determinations of plasma adrenocorticotropic hormone concentration during replacement therapy are the next essential diagnostic element. Absolute temporal scotomas in visual field examination are an early sign of Nelson’s syndrome. Sufficient hydrocortisone therapy is very important to avoid hypocortisolemia, which stimulates corticotroph cells. Methods of treatment include neurosurgery, radiation therapy and pharmacotherapy. Prognosis in Nelson’s syndrome depends on the stage of the disease; it may be poor in invasive pituitary tumors.

Keywords::

• adrenocorticotropic hormone
• bilateral adrenalectomy
• Cushing’s disease
• Nelson’s syndrome
• Nelson’s tumor
• pituitary tumor

Acknowledgements

The author wishes to thank W Bonicki and J Kunicki from the Department of Neurosurgery, Maria Sklodowska-Curie Cancer Center and Institute of Oncology (Warsaw, Poland) for updates on treatment methods of Nelson’s syndrome.

Financial & competing interests disclosure

The author has no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

No writing assistance was utilized in the production of this manuscript.