The effect of the long-acting somatostatin analogue SMS 201–995 on ACTH secretion in Nelson's syndrome and Cushing's disease

1989 ◽  
Vol 120 (6) ◽  
pp. 760-766 ◽  
Author(s):  
Steven W.J. Lamberts ◽  
Piet Uitterlinden ◽  
Jan M. G. Klijn
Keyword(s):  
Cushing’S Disease ◽  
Pituitary Tumour ◽  
Cushing's Disease ◽  
Somatostatin Analogue ◽  
Plasma Acth ◽  
Nelson’S Syndrome ◽  
Nelson's Syndrome ◽  
Chronic Therapy ◽  
Long Acting ◽  
Field Defect

Abstract. Chronic therapy of a patient with Nelson's syndrome for 2 years with 300 μg SMS 201–995 per day resulted in a significant decrease in circulating ACTH levels, normalization of the visual field defect and of loss of visual acuity of one eye, and stabilization of tumour growth, without radiological evidence of shrinkage of the pituitary tumour. In two other patients with Nelson's syndrome, SMS 201–995 acutely inhibited circulating ACTH levels. This effect could be shown best if cortisol replacement was temporarily withheld. SMS 201–995 did not affect plasma ACTH and cortisol levels in three patients with untreated Cushing's disease.

scholarly journals Nelson's Syndrome

2007 ◽  
Vol 51 (8) ◽  
pp. 1392-1396 ◽  
Author(s):  
Alia Munir ◽  
John Newell-Price
Keyword(s):  
Cushing’S Disease ◽  
Pituitary Tumour ◽  
Bilateral Adrenalectomy ◽  
Cushing's Disease ◽  
Treatment Modalities ◽  
Pituitary Mass ◽  
Nelson’S Syndrome ◽  
Nelson's Syndrome ◽  
Great Heterogeneity ◽  
The One

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.

scholarly journals Nelson's syndrome

2010 ◽  
Vol 163 (4) ◽  
pp. 495-507 ◽  
Author(s):  
T M Barber ◽  
E Adams ◽  
O Ansorge ◽  
J V Byrne ◽  
N Karavitaki ◽  
...  
Keyword(s):  
Predictive Factors ◽  
Cushing’S Disease ◽  
Pituitary Tumour ◽  
Cushing's Disease ◽  
Current Evidence ◽  
Pituitary Mass ◽  
Nelson’S Syndrome ◽  
Nelson's Syndrome ◽  
Tertiary Centre ◽  
Threatening Condition

Nelson's syndrome is a potentially life-threatening condition that does not infrequently develop following total bilateral adrenalectomy (TBA) for the treatment of Cushing's disease. In this review article, we discuss some controversial aspects of Nelson's syndrome including diagnosis, predictive factors, aetiology, pathology and management based on data from the existing literature and the experience of our own tertiary centre.Definitive diagnostic criteria for Nelson's syndrome are lacking. We argue in favour of a new set of criteria. We propose that Nelson's syndrome should be diagnosed in any patient with prior TBA for the treatment of Cushing's disease and with at least one of the following criteria: i) an expanding pituitary mass lesion compared with pre-TBA images; ii) an elevated 0800 h plasma level of ACTH (>500 ng/l) in addition to progressive elevations of ACTH (a rise of >30%) on at least three consecutive occasions. Regarding predictive factors for the development of Nelson's syndrome post TBA, current evidence favours the presence of residual pituitary tumour on magnetic resonance imaging (MRI) post transsphenoidal surgery (TSS); an aggressive subtype of corticotrophinoma (based on MRI growth rapidity and histology of TSS samples); lack of prophylactic neoadjuvant pituitary radiotherapy at the time of TBA and a rapid rise of ACTH levels in year 1 post TBA. Finally, more studies are needed to assess the efficacy of therapeutic strategies in Nelson's syndrome, including the alkylating agent, temozolomide, which holds promise as a novel and effective therapeutic agent in the treatment of associated aggressive corticotroph tumours. It is timely to review these controversies and to suggest guidelines for future audit.

Vasoactive intestinal polypeptide enhances ACTH levels in some patients with adrenocorticotropin - secreting pituitary adenomas

1987 ◽  
Vol 116 (2) ◽  
pp. 216-220 ◽  
Author(s):  
Bruno Ambrosi ◽  
Domenico Bochicchio ◽  
Alessandro Sartorio ◽  
Francesco Morabito ◽  
Giovanni Faglia
Keyword(s):  
Vasoactive Intestinal Polypeptide ◽  
Cushing’S Disease ◽  
Normal Subjects ◽  
Significant Rise ◽  
Cushing's Disease ◽  
Plasma Acth ◽  
Nelson’S Syndrome ◽  
Nelson's Syndrome ◽  
Cortisol Levels ◽  
Intestinal Polypeptide

Abstract. Vasoactive intestinal polypeptide (VIP) was administered (75 μg iv over 12 min) to 14 patients with Cushing's disease, 1 patient with Nelson's syndrome, and 8 normal subjects. VIP induced a significant rise of plasma ACTH levels in 6 patients with Cushing's disease, from a baseline of 13.2 pmol/l (9.9–18.5 pmol/l) to a peak of 24.5 pmol/l (7.7–18.9 pmol/l), median and range (P < 0.05), and in the patient with Nelson's syndrome, from a baseline of 260.9 to 461.3 pmol/l. A significant elevation of cortisol levels was also observed, from a baseline of 567 nmol/l (185–842 nmol/l) to a peak of 727 nmol/l (364–1029 nmol/l); P < 0.05. No modifications in plasma ACTH and cortisol levels were noticed in the other 8 patients with Cushing's disease, or in the normal subjects. In the responsive patients, the median plasma ACTH level reached after VIP was found to be less than that induced by CRH administration. In 2 of the responsive patients, VIP was injected again after successful microadenomectomy and did not then cause changes in ACTH and cortisol concentration. These data demonstrate that VIP specifically stimulates ACTH release in some patients with corticotropinomas but not in normal subjects; the disappearance of such abnormal ACTH responses after successful adenomectomy suggests the presence of specific VIP receptors only on the adenomatous corticotropes.

Demonstration of pro-opiomelanocortin mRNA in pituitary adenomas and para-adenomatous gland in cushing's disease and Nelson's syndrome

1993 ◽  
Vol 169 (3) ◽  
pp. 335-339 ◽  
Author(s):  
Marita Fehn ◽  
Maura A. Farquharson ◽  
Doris Sautner ◽  
Wolfgang Saeger ◽  
Dieter K. Lüdecke ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Pituitary Adenomas ◽  
Cushing's Disease ◽  
Nelson’S Syndrome ◽  
Nelson's Syndrome

Nelson’s syndrome after bilateral adrenalectomy for Cushing’s disease

Surgery ◽  
2007 ◽  
Vol 141 (2) ◽  
pp. 147-152 ◽  
Author(s):  
Alejandra Gil-Cárdenas ◽  
Miguel F. Herrera ◽  
Araceli Díaz-Polanco ◽  
Juan Manuel Rios ◽  
Juan Pablo Pantoja
Keyword(s):  
Cushing’S Disease ◽  
Bilateral Adrenalectomy ◽  
Cushing's Disease ◽  
Nelson’S Syndrome ◽  
Nelson's Syndrome

MEGAVOLTAGE PITUITARY IRRADIATION IN THE MANAGEMENT OF CUSHING'S DISEASE AND NELSON'S SYNDROME: LONG-TERM FOLLOW-UP

1989 ◽  
Vol 31 (3) ◽  
pp. 309-323 ◽  
Author(s):  
T. A. HOWLETT ◽  
P. N. PLOWMAN ◽  
J. A. H. WASS ◽  
L. H. REES ◽  
A. E. JONES ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Cushing's Disease ◽  
Nelson’S Syndrome ◽  
Nelson's Syndrome ◽  
Long Term Follow Up ◽  
Pituitary Irradiation
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