The Nelson's syndrome... revisited

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.

Combined xenotransplantation as a method of prolonging the viability and function of the graft in the treatment of various forms of hypocorticism

“…Ó õâîðèõ ³ç õðîí³÷íîþ íàäíèðêîâîþ íåäîñòàò-í³ñòþ, ùî âèíèêëà ÿê ðåçóëüòàò òîòàëüíî¿ àäðå-íàëåêòî쳿 ç ïðèâîäó õâîðîáè ²öåíêà-Êóøèíãà, ìîaeëèâèé ðîçâèòîê ñèíäðîìó Íåëüñîíà, ÿêèé õàðàêòåðèçóºòüñÿ íàÿâí³ñòþ àãðåñèâíî¿ ïóõëè-íè àäåíîã³ïîô³çà -êîðòèêîòðîï³íîìè [52][53][54][55]. Ñë³ä çàçíà÷èòè, ùî ëåòàëüí³ñòü ñåðåä õâîðèõ íà ïîñòàäðåíàëåêòîì³÷íèé ã³ïîêîðòèöèçì ó â³ääà-ëåíèé ïåð³îä íàâ³òü çà óìîâ àäåêâàòíî¿ çàì³ñ-íî¿ òåðàﳿ ó 3-4 ðàçè âèùà, í³ae ó çàãàëüí³é ïîïóëÿö³¿.…”

Nelson's Syndrome

“…This is especially true in recent years since imaging with MRI allows precise documentation of any tumour progression, whereas historically patients presented with masses large enough to be found with CT, simple sella tomography, or with neuro-opthalmic complications (11). Moreover, widespread access to reliable plasma ACTH assays has greatly facilitated monitoring of patients.…”

“…This wide variation in incidence is as likely to be due to differences in definition as much as bias of case mix in different centres. There is also great variation in the latency of onset from adrenalectomy to the diagnosis of Nelson's syndrome, ranging from a few months to 24 years, or more, emphasising the need for careful life-long follow-up (10,11).…”

“…Mass effects include compression of the optic apparatus and visual field defects, headache, external ophthalmoplegias, and hypopituitarism (11). Deep cutaneous pigmentation is frequently present, and is due to ACTH stimulation of the melanocortin 1 receptors on melanocytes in the skin.…”

“…The presence of tumour either at surgery or on imaging has been shown to predict onset, especially if there are aggressive characteristics or macroadenoma (7,12). Young age at the time of adrenalectomy has been suggested to be predictive in some studies (14)(15)(16)(17), but this is not a uniform finding in all (11). The duration of Cushing's disease is predictive in some series (18), but again not in all.…”