The Sella Turcica in Nelson's Syndrome

Radiology ◽  
1976 ◽  
Vol 118 (2) ◽  
pp. 363-365 ◽  
Author(s):  
Meredith Weinstein ◽  
Blake Tyrrell ◽  
Thomas H. Newton
Neurosurgery ◽  
1990 ◽  
Vol 27 (6) ◽  
pp. 961-968 ◽  
Author(s):  
Michael Buchfelder ◽  
Rudolf Fahlbusch ◽  
Paul Thierauf ◽  
Otto A. Muüller

Abstract Nelson's syndrome is generally regarded as an unusual sequela of primary bilateral adrenalectomy when performed for Cushing's disease. It is classically defined by cutaneous hyperpigmentation, considerably elevated adrenocorticotropic hormone (ACTH) levels, and an enlarged sella turcica. In this report, we present three cases initially treated by transsphenoidal sellar exploration for Cushing's disease. In two of these cases, remission of hypercortisolism did not occur after the initial pituitary exploration. A microadenomectomy was performed in one case and, in the other, no microadenoma was found. In both, Nelson's syndrome occurred after adrenalectomy. A second transsphenoidal operation and radiotherapy were required to control tumor growth. In another case, transsphenoidal adenomectomy of an ACTH-secreting tumor initially led to a remission of hypercortisolism for 4 years, but recurrent Cushing's disease necessitated adrenalectomy, and again Nelson's syndrome occurred. The documentation of a pre-existing ACTH-secreting basophilic pituitary microadenoma before adrenalectomy, as seen in two of our cases, has not been previously reported, and these observations of “non-classical” courses have major implications for the pathophysiology of Nelson's syndrome.


1987 ◽  
Vol 115 (3) ◽  
pp. 419-422 ◽  
Author(s):  
Linda Bardram ◽  
Jörgen Lindholm ◽  
Jens F. Rehfeld

Abstract. Twelve of 87 pituitary adenomas from patients with acromegaly, Cushing's syndrome, Nelson's syndrome, hyperprolactinaemia and without symptoms of hormone hypersecretion contained gastrin in concentrations from 0.5 to 166 pmol/g. Only ACTH-producing tumours contained gastrin, which occurred in forms smaller than those present in the normal adenohypophysis. The results indicate that corticotropic tumours may synthesize gastrin in moderate amounts.


1976 ◽  
Vol 42 (6) ◽  
pp. 1145-1148 ◽  
Author(s):  
M. DONNADIEU ◽  
M. F. LAURENT ◽  
J. P. LUTON ◽  
H. BRICAIRE ◽  
F. GIRARD ◽  
...  

1996 ◽  
Vol 7 (2) ◽  
pp. 87-90 ◽  
Author(s):  
Anna A. Kasperlik-Zaluska ◽  
Jerzy Walecki ◽  
Woycicell Jeske ◽  
Barbara Migdalska ◽  
Jadwiga Janik ◽  
...  

2007 ◽  
Vol 51 (8) ◽  
pp. 1392-1396 ◽  
Author(s):  
Alia Munir ◽  
John Newell-Price

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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