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The Nelson's syndrome... revisited

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Abstract

Adrenalectomy is a radical therapeutic approach to control hypercortisolism in some patients with Cushing's disease. However it may be complicated by the Nelson's syndrome, defined by the association of a pituitary macroadenoma and high ACTH secretion after adrenalectomy. This definition has not changed since the end of the fifties. Today the Nelson's syndrome must be revisited with new to criteria using more sensitive diagnostic tools, especially the pituitary magnetic resonance imaging.

In this paper we will review the pathophysiological aspects of corticotroph tumor growth, with reference to the impact of adrenalectomy. The main epidemiological data on the Nelson's syndrome will be presented.

More importantly, we will propose a new pathophysiological and practical approach to this question which attempts to evaluate the Corticotroph Tumor Progression after adrenalectomy, rather than to diagnose the Nelson's syndrome. We will discuss the consequences for the management of Cushing's disease patients after adrenalectomy, and will also draw some perspectives.

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Authors and Affiliations

  1. Université René Descartes Endocrinology, Cochin Hospital, Paris 5, France

    Guillaume Assié, Jérôme Bertherat & Xavier Bertagna

  2. Radiology Departments, Cochin Hospital, Paris 5, France

    Hélène Bahurel & Paul Legmann

  3. Pathology Department, La Pitié Hospital, Paris, France

    Michèle Kujas

  4. Endocrinology Department, Pavillon Cornil, Hôpital Cochin, 27, rue du Fg St Jacques, 75014, Paris, France

    Xavier Bertagna

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  1. Guillaume Assié
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  2. Hélène Bahurel
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  4. Michèle Kujas
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  6. Xavier Bertagna
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Correspondence to Xavier Bertagna.

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Assié, G., Bahurel, H., Bertherat, J. et al. The Nelson's syndrome... revisited. Pituitary 7, 209–215 (2004). https://doi.org/10.1007/s11102-005-1403-y

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