Synonym(s)
ACTH-secreting pituitary adenoma; Nelson's syndromes
HistoryThis section has been translated automatically.
Nelson, 1958
DefinitionThis 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.
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Occurrence/EpidemiologyThis section has been translated automatically.
Very rare. In 40% of patients with bilateral adrenalectomies. Incidence decreasing.
EtiopathogenesisThis section has been translated automatically.
Idiopathic. Consequence of therapy of pituitary tumors by bilateral adrenalectomy.
ManifestationThis section has been translated automatically.
Long term development (up to 20 years) after bilateral adrenalectomy. Predominant in younger women.
Clinical featuresThis 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.
LaboratoryThis section has been translated automatically.
Electrolytes, ACTH, α-MSH, POMC, pituitary hormones, hypothalamus hormones, thyroid hormones.
DiagnosisThis section has been translated automatically.
Medical history (almost always Cushing's), blood pressure, laboratory, MRI, CT, thyroid parameters.
Differential diagnosisThis section has been translated automatically.
TherapyThis 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/forecastThis section has been translated automatically.
Well, with timely treatment of the underlying disease.
LiteratureThis 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
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Outgoing links (5)
Addison's disease; Dyschromia; Glucocorticosteroids; Hemochromatosis; Neurofibromatosis (overview);Disclaimer
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.
Prof. Dr. med. Peter Altmeyer