Corticotroph Tumor Progression after Adrenalectomy in Cushing’s Disease: A Reappraisal of Nelson’s Syndrome

Assié, Guillaume; Bahurel, Hélène; Coste, Joël; Silvera, S.; Kujas, M.; Dugué, Marie Annick; Karray, Foued; Dousset, B.; Bertherat, Jérôme; Legmann, P.; Bertagna, Xavier

doi:10.1210/jc.2006-1328

Cited by 214 publications

(202 citation statements)

References 41 publications

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“…Furthermore, growth of the pituitary tumor occurred in 67% of the reported cases that were treated with bilateral adrenalectomy as primary treatment. The prevalence of Nelson's syndrome without evidence of distant metastases of the pituitary tumor ranges from approximately 30-50% [58,59] in adrenalectomized patients and thus seems rather high in the reported cases with malignant pituitary CD. Therefore adrenalectomy might trigger transformation of a pituitary tumor in an adenoma-to-carcinoma sequence.…”

Section: Discussionmentioning

confidence: 82%

Malignant pituitary corticotroph adenomas: report of two cases and a comprehensive review of the literature

et al. 2008

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Corticotroph pituitary carcinomas are tumors, defined by the presence of distant metastases that determine their poor prognosis. The diagnosis and therapy of malignant corticotroph adenomas remains a clinical challenge. The molecular mechanisms of malignant transformation of pituitary adenomas are unclear, although they are believed to arise in an adenoma-to-carcinoma sequence. We describe two cases of malignant Cushing's disease with metastases in liver and bone, respectively. The primary pituitary tumors were treated by a combination of radiotherapy and transsphenoidal surgery, but recurred several times in both patients. The time interval between the diagnosis of Cushing's disease and the discovery of metastases was 32 and 17 years, respectively. In the first case the patient died within 6 months after diagnosis of metastasis, whereas the second patient is alive at a followup of 2 years after the discovery of the metastasis. Furthermore, we reviewed all available cases of corticotroph pituitary carcinomas reported in the literature and analyzed their clinical features and therapeutical management. In conclusion, frequent relapses of Cushing's disease, aggressive growth of macroadenoma, Nelson's syndrome after adrenalectomy or persistently high ACTH levels should prompt the clinician to consider the possibility of pituitary corticotroph carcinomas.

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Section: Discussionmentioning

confidence: 82%

Malignant pituitary corticotroph adenomas: report of two cases and a comprehensive review of the literature

et al. 2008

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“…Ó õâîðèõ ³ç õðîí³÷íîþ íàäíèðêîâîþ íåäîñòàò-í³ñòþ, ùî âèíèêëà ÿê ðåçóëüòàò òîòàëüíî¿ àäðå-íàëåêòîì³¿ 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¿ òåðàï³¿ ó 3-4 ðàçè âèùà, í³ae ó çàãàëüí³é ïîïóëÿö³¿.…”

Section: комбінована ксенотрансплантація як метод пролонгації життєздunclassified

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

Ларін¹,

Анастасій²,

Гирявенко³

et al. 2013

CE&ES

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“…More recently, some of these areas of controversy have been addressed by a modern, large and carefully reported series from Cochin Hospital, Paris, France (19). In this single centre series 53 patients treated by bilateral adrenalectomy for Cushing's disease were followed.…”

Section: Predictive Featuresmentioning

confidence: 99%

“…As indicated above measurement of plasma ACTH is essential in monitoring. As indicated above this may be done by measurement of the 0800h plasma ACTH, 20h after the last dose of glucocorticoid and before the morning dose of glucocorticoid, with an increment in the value compared to a previous value of 100 pg/ml being significant (19). A potential drawback of this type of monitoring is that high plasma ACTH levels are found in patients with primary adrenal insufficiency without pituitary pathology.…”

Section: Monitoringmentioning

confidence: 99%

Nelson's Syndrome

Munir

Newell‐Price

2007

Arq Bras Endocrinol Metab

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Nelson's syndrome is a potentially severe complication of bilateral adrenalectomy performed in the treatment of Cushing's disease, and its management remains difficult. Of all of the features of Nelson's syndrome, the one that causes most concern is the development of a locally aggressive pituitary tumour, which, unusually for pituitary disease, may occasionally cause death from the tumour itself. This feature is especially pertinent given the increasing use in Cushing's disease of laparoscopic bilateral adrenal surgery as a highly effective treatment modality to control cortisol-excess. Despite numerous studies and reports, there is no formal consensus of what defines Nelson's syndrome. Thus, some will define Nelson's syndrome according to the classical description with an evolving pituitary mass after bilateral adrenalectomy, whereas others will rely on increasing plasma ACTH levels, even in the absence of a clear pituitary mass lesion on MRI. These factors need to be borne in mind when considering the reports of Nelson's syndrome, as there is great heterogeneity, and it is likely that overall the modern 'Nelson's syndrome' represents a different disease entity from that of the last century. In the present paper, clinical and epidemiological features of Nelson's syndrome, as well as its treatment modalities, are reviewed.

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Corticotroph Tumor Progression after Adrenalectomy in Cushing’s Disease: A Reappraisal of Nelson’s Syndrome

Abstract: After adrenalectomy in Cushing's disease, one should no longer wait for the occurrence of Nelson's syndrome: modern imaging allows early detection and management of corticotroph tumor progression.

Cited by 214 publications

References 41 publications

Malignant pituitary corticotroph adenomas: report of two cases and a comprehensive review of the literature

Malignant pituitary corticotroph adenomas: report of two cases and a comprehensive review of the literature

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

Nelson's Syndrome

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