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

scholarly journals Rapid response of Nelson’s syndrome to pasireotide in radiotherapy-naive patient

2020 ◽  
Vol 6 (1) ◽  
Author(s):  
Xin He ◽  
Joanna L. Spencer-Segal
Keyword(s):  
Cushing’S Disease ◽  
Pituitary Adenomas ◽  
Bilateral Adrenalectomy ◽  
Cushing's Disease ◽  
Optimal Management ◽  
Nelson’S Syndrome ◽  
Naive Patient ◽  
Nelson's Syndrome ◽  
Skin Hyperpigmentation ◽  
The Right

Abstract Background Nelson’s syndrome is a well-described complication following bilateral adrenalectomy for management of Cushing’s disease. There is no consensus on optimal management of Nelson’s syndrome, characterized by the triad of pituitary corticotroph adenoma growth, elevated serum adrenocorticotropic hormone, and skin hyperpigmentation. Medical therapy with a variety of drug classes have been studied. One potentially promising drug already approved for Cushing’s disease is pasireotide, a somatostatin analog with affinity for multiple somatostatin receptors, including subtype 5, the most highly expressed receptor on corticotroph tumors. Case presentation A 24-year-old female was diagnosed with Cushing’s disease with initial ACTH levels around 700–800 pg/mL. She underwent transsphenoidal surgery without remission, followed by bilateral adrenalectomy. Over the subsequent 3 years, the patient developed skin hyperpigmentation, recurrent elevations of ACTH, and tumor recurrence requiring two additional transsphenoidal surgeries. After her third transsphenoidal resection, ACTH normalized, no residual tumor was seen on radiology, and the patient’s skin hyperpigmentation improved. She then had an uncomplicated full-term pregnancy, during which ACTH levels remained within normal limits. One month after delivery, ACTH levels began rising to a peak at 5,935 pg/mL. Imaging revealed two new bilateral pituitary adenomas, measuring 14 mm on the left, and 7 mm on the right. She was then started on pasireotide. After two months of therapy, ACTH decreased to 609 pg/mL, and repeat pituitary MRI showed interval decrease in size of both pituitary adenomas to 13 mm on the left and 6 mm on the right. Conclusion We report the protracted course of a young female with several recurrences of Nelson’s syndrome following bilateral adrenalectomy and multiple transsphenoidal surgeries, who ultimately responded to pasireotide. Unique features of her case not described previously are the response to pasireotide in a radiotherapy-naive patient, as well as the rapid radiologic response to therapy. Her history illustrates the unresolved challenges of Nelson’s syndrome and the continued need for additional studies to identify optimal management.

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.

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

Observations on the Pathophysiology of Nelson's Syndrome: A Report of Three Cases

Neurosurgery ◽  
1990 ◽  
Vol 27 (6) ◽  
pp. 961-968 ◽  
Author(s):  
Michael Buchfelder ◽  
Rudolf Fahlbusch ◽  
Paul Thierauf ◽  
Otto A. Muüller
Keyword(s):  
Cushing’S Disease ◽  
Sella Turcica ◽  
Cushing's Disease ◽  
Nelson’S Syndrome ◽  
Nelson's Syndrome ◽  
Control Tumor Growth ◽  
Transsphenoidal Adenomectomy ◽  
Control Tumor ◽  
Transsphenoidal Operation ◽  
Acth Secreting

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.

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