Outcomes after primary treatment for Nelson's syndrome: a study from 13 UK centres

2019 ◽  
Author(s):  
Athanasios Fountas ◽  
Eugenie Lim ◽  
William M Drake ◽  
Andrew Polson ◽  
Mark Gurnell ◽  
...  
Keyword(s):  
Primary Treatment ◽  
Nelson’S Syndrome ◽  
Nelson's Syndrome

scholarly journals Outcomes of Patients with Nelson’s Syndrome after Primary Treatment: A Multicenter Study from 13 UK Pituitary Centers

2019 ◽  
Vol 105 (5) ◽  
pp. 1527-1537 ◽  
Author(s):  
Athanasios Fountas ◽  
Eugenie S Lim ◽  
William M Drake ◽  
Andrew S Powlson ◽  
Mark Gurnell ◽  
...  
Keyword(s):  
Tumor Progression ◽  
Spinal Metastases ◽  
Progression Free Survival ◽  
Primary Treatment ◽  
Tumor Control ◽  
Nelson’S Syndrome ◽  
Nelson's Syndrome ◽  
Primary Management

Abstract Context Long-term outcomes of patients with Nelson’s syndrome (NS) have been poorly explored, especially in the modern era. Objective To elucidate tumor control rates, effectiveness of various treatments, and markers of prognostic relevance in patients with NS. Patients, design, and setting Retrospective cohort study of 68 patients from 13 UK pituitary centers with median imaging follow-up of 13 years (range 1–45) since NS diagnosis. Results Management of Cushing’s disease (CD) prior to NS diagnosis included surgery+adrenalectomy (n = 30; eight patients had 2 and one had 3 pituitary operations), surgery+radiotherapy+adrenalectomy (n = 17; two received >1 courses of irradiation, two had ≥2 pituitary surgeries), radiotherapy+adrenalectomy (n = 2), and adrenalectomy (n = 19). Primary management of NS mainly included surgery, radiotherapy, surgery+radiotherapy, and observation; 10-year tumor progression-free survival was 62% (surgery 80%, radiotherapy 52%, surgery+radiotherapy 81%, observation 51%). Sex, age at CD or NS diagnosis, size of adenoma (micro-/macroadenoma) at CD diagnosis, presence of pituitary tumor on imaging prior adrenalectomy, and mode of NS primary management were not predictors of tumor progression. Mode of management of CD before NS diagnosis was a significant factor predicting progression, with the group treated by surgery+radiotherapy+adrenalectomy for their CD showing the highest risk (hazard ratio 4.6; 95% confidence interval, 1.6–13.5). During follow-up, 3% of patients had malignant transformation with spinal metastases and 4% died of aggressively enlarging tumor. Conclusions At 10 years follow-up, 38% of the patients diagnosed with NS showed progression of their corticotroph tumor. Complexity of treatments for the CD prior to NS diagnosis, possibly reflecting corticotroph adenoma aggressiveness, predicts long-term tumor prognosis.

scholarly journals Nelson's syndrome: a review of the clinical manifestations, pathophysiology, and treatment strategies

2015 ◽  
Vol 38 (2) ◽  
pp. E14 ◽  
Author(s):  
Jimmy Patel ◽  
Jean Anderson Eloy ◽  
James K. Liu
Keyword(s):  
Diagnostic Criteria ◽  
Transsphenoidal Surgery ◽  
Clinical Manifestations ◽  
Treatment Strategies ◽  
Primary Treatment ◽  
Bilateral Adrenalectomy ◽  
Nelson’S Syndrome ◽  
Pharmacological Agents ◽  
Nelson's Syndrome ◽  
Clinical Triad

Nelson's syndrome is a rare clinical manifestation that occurs in 8%–47% of patients as a complication of bilateral adrenalectomy, a procedure that is used to control hypercortisolism in patients with Cushing's disease. First described in 1958 by Dr. Don Nelson, the disease has since become associated with a clinical triad of hyperpigmentation, excessive adrenocorticotropin secretion, and a corticotroph adenoma. Even so, for the past several years the diagnostic criteria and management of Nelson's syndrome have been inadequately studied. The primary treatment for Nelson's syndrome is transsphenoidal surgery. Other stand-alone therapies, which in many cases have been used as adjuvant treatments with surgery, include radiotherapy, radiosurgery, and pharmacotherapy. Prophylactic radiotherapy at the time of bilateral adrenalectomy can prevent Nelson's syndrome (protective effect). The most promising pharmacological agents are temozolomide, octreotide, and pasireotide, but these agents are often administered after transsphenoidal surgery. In murine models, rosiglitazone has shown some efficacy, but these results have not yet been found in human studies. In this article, the authors review the clinical manifestations, pathophysiology, diagnostic criteria, and efficacy of multimodal treatment strategies for Nelson's syndrome.

Gastrin in pituitary tumours

1987 ◽  
Vol 115 (3) ◽  
pp. 419-422 ◽  
Author(s):  
Linda Bardram ◽  
Jörgen Lindholm ◽  
Jens F. Rehfeld
Keyword(s):  
Cushing’S Syndrome ◽  
Pituitary Adenomas ◽  
Cushing's Syndrome ◽  
Pituitary Tumours ◽  
Nelson’S Syndrome ◽  
Nelson's Syndrome

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.

The Sella Turcica in Nelson's Syndrome

Radiology ◽  
1976 ◽  
Vol 118 (2) ◽  
pp. 363-365 ◽  
Author(s):  
Meredith Weinstein ◽  
Blake Tyrrell ◽  
Thomas H. Newton
Keyword(s):  
Sella Turcica ◽  
Nelson’S Syndrome ◽  
Nelson's Syndrome

Synthetic MIF Has No Effect onβ-MSH and ACTH Hypersecietion in Nelson's Syndrome

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

Early diagnosis of Nelson’s syndrome

1996 ◽  
Vol 7 (2) ◽  
pp. 87-90 ◽  
Author(s):  
Anna A. Kasperlik-Zaluska ◽  
Jerzy Walecki ◽  
Woycicell Jeske ◽  
Barbara Migdalska ◽  
Jadwiga Janik ◽  
...  
Keyword(s):  
Early Diagnosis ◽  
Nelson’S Syndrome ◽  
Nelson's Syndrome

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.

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