Nelson syndrome E24.1
Synonym(s)
ACTH-secreting pituitary adenoma; Nelson's syndromes
History This section has been translated automatically.
Nelson, 1958
Definition This section has been translated automatically.
Syndrome characterized by disorders of hypophysial hormone secretion, enlargement of the pituitary gland, intense hyperpigmentary dyschromia of the skin, headache, visual disturbances, menstrual disorders, among others.
Occurrence/Epidemiology This section has been translated automatically.
Very rare. In 40% of patients with bilateral adrenalectomies. Incidence decreasing.
Etiopathogenesis This section has been translated automatically.
Idiopathic. Consequence of therapy of pituitary tumors by bilateral adrenalectomy.
Manifestation This section has been translated automatically.
Long term development (up to 20 years) after bilateral adrenalectomy. Predominant in younger women.
Clinic This section has been translated automatically.
Pronounced ubiquitous melanotic hyperpigmentation of the skin and mucous membranes (including scars, gingiva, nipples). Sclerae are usually not affected. Often there are visual disturbances, weight loss, hypotonus, anorexia, vomiting. Excessive ACTH secretion, increased α-MSH secretion.
Laboratory This section has been translated automatically.
Electrolytes, ACTH, α-MSH, POMC, pituitary hormones, hypothalamus hormones, thyroid hormones.
Diagnosis This section has been translated automatically.
Medical history (almost always Cushing's), blood pressure, laboratory, MRI, CT, thyroid parameters.
Differential diagnosis This section has been translated automatically.
Therapy This section has been translated automatically.
Therapy of the underlying disease (hypophysectomy, radiotherapy of the pituitary gland). If necessary, hormone replacement therapy with glucocorticoids, mineralocorticoids, somatostatin analogues.
Progression/forecast This section has been translated automatically.
Well, with timely treatment of the underlying disease.
Literature This section has been translated automatically.
- Chapuis Y et al (1996) Role and operative risk of bilateral adrenalectomy in hypercortisolism. World J Surg 20: 775-779
- Clayton R et al (1977) Secondary pituitary hyperplasia in Addison's disease. Lancet 2: 954-956
- Imai T et al (1996) Adrenalectomy for treatment of Cushing's syndrome: results in 122 patients and long-term follow-up studies. World J Surg 20: 781-786
- Kelly PA et al (2002) Neurosurgical treatment of Nelson's syndrome. J Clin Endocrinol Metab 87: 5465-5469
- Kobayashi T et al (2002) Gamma knife radiosurgery in the treatment of Cushing disease: long-term results. J Neurosurg 97: (5Suppl): 422-428
- Nelson DH et al (1958) ACTH-producing tumor of the pituitary gland. N Engl J Med 259: 161