scholarly journals Transsphenoidal surgery for Cushing's disease: a review of success rates, remission predictors, management of failed surgery, and Nelson's Syndrome

2007 ◽  
Vol 23 (3) ◽  
pp. 1-6 ◽  
Author(s):  
Daniel F. Kelly
Keyword(s):  
Cushing’S Disease ◽  
Transsphenoidal Surgery ◽  
Remission Rate ◽  
Cushing's Disease ◽  
Surgical Morbidity ◽  
Nelson’S Syndrome ◽  
Nelson's Syndrome ◽  
Radio Therapy ◽  
Transsphenoidal Adenomectomy

✓ Cushing's disease is a serious endocrinopathy that, if left untreated, is associated with significant morbidity and mortality rates. After diagnostic confirmation of Cushing's disease has been made, transsphenoidal adenomectomy is the treatment of choice. When a transsphenoidal adenomectomy is performed at experienced transsphenoidal surgery centers, long-term remission rates average 80% overall, surgical morbidity is low, and the mortality rate is typically less than 1%. In patients with well-defined noninvasive microadenomas, the long-term remission rate averages 90%. For patients in whom primary surgery fails, treatment options such as bilateral adrenalectomy, stereotactic radiotherapy or radiosurgery, total hypophysectomy, or adrenolytic medical therapy need to be carefully considered, ideally in a multidisciplinary setting. The management of Nelson's Syndrome often requires both transsphenoidal surgery and radio-therapy to gain disease control.

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.

Long Term Results of Transsphenoidal Adenomectomy in Patients with Cushing's Disease

Neurosurgery ◽  
1987 ◽  
Vol 21 (2) ◽  
pp. 218-222 ◽  
Author(s):  
Toshichi Nakane ◽  
Akio Kuwayama ◽  
Masao Watanabe ◽  
Tatsuo Takahashi ◽  
Tetsuo Kato ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Pituitary Adenomas ◽  
Remission Rate ◽  
Hormone Secretion ◽  
Late Recurrence ◽  
Long Term Results ◽  
Cushing's Disease ◽  
Cell Hyperplasia ◽  
Transsphenoidal Adenomectomy

Abstract As part of an ongoing series, 100 patients with Cushing's disease underwent transsphenoidal operations. Pituitary adenomas were confirmed in 93 patients, and initial remission was achieved in 86 (92%) of them. Hypercortisolemia was not corrected in 7 patients, and in 4 this was due to invasive adenomas. These patients were subjected to irradiation, medical treatment, or both after operation. Only 7 of the 100 patients had no pituitary adenoma found at operation, and they obtained no clinical remission even after partial or subtotal hypophysectomy. Follow-up review, with an emphasis on endocrinological studies, was performed on these patients for a mean period of 38 months. Seventy-eight patients were in long term remission after operation and had restoration of noncorticotropic hormone secretion as well as pituitary-adrenal function. Recurrence was noted in 8 patients after 19 to 82 months in remission. In all of these patients, pituitary adenomas were verified by reoperation and no case of corticotrophic cell hyperplasia was noted. We conclude that late recurrence of Cushing's disease may occur after adenoma removal and is due to the regrowth of adenoma cells left behind in the peritumoral tissue at the first operation. In view of the overall remission rate, transsphenoidal adenomectomy is considered a highly effective treatment for Cushing's disease.

Long-term remission and recurrence rates after transsphenoidal surgery for cushing's disease

2014 ◽  
Author(s):  
Paloma Moreno Moreno ◽  
Julia Silva Fernandez ◽  
Maria Rosa Alhambra Exposito ◽  
Maria angeles Galvez Moreno
Keyword(s):  
Cushing’S Disease ◽  
Transsphenoidal Surgery ◽  
Cushing's Disease ◽  
Recurrence Rates

scholarly journals Outcomes of endoscopic transsphenoidal surgery for Cushing's disease

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Zarina Brady ◽  
Aoife Garrahy ◽  
Claire Carthy ◽  
Michael W. O’Reilly ◽  
Christopher J. Thompson ◽  
...  
Keyword(s):  
Diabetes Insipidus ◽  
Cushing’S Disease ◽  
Transsphenoidal Surgery ◽  
Remission Rate ◽  
Cushing's Disease ◽  
Endoscopic Transsphenoidal Surgery ◽  
Endocrine Society ◽  
Csf Leakage ◽  
Remission Rates

Abstract Background Transsphenoidal surgery (TSS) to resect an adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma is the first-line treatment for Cushing’s disease (CD), with increasing usage of endoscopic transsphenoidal (ETSS) technique. The aim of this study was to assess remission rates and postoperative complications following ETSS for CD. Methods A retrospective analysis of a prospective single-surgeon database of consecutive patients with CD who underwent ETSS between January 2012–February 2020. Post-operative remission was defined, according to Endocrine Society Guidelines, as a morning serum cortisol < 138 nmol/L within 7 days of surgery, with improvement in clinical features of hypercortisolism. A strict cut-off of < 50 nmol/L at day 3 post-op was also applied, to allow early identification of remission. Results A single surgeon (MJ) performed 43 ETSS in 39 patients. Pre-operative MRI localised an adenoma in 22 (56%) patients; 18 microadenoma and 4 macroadenoma (2 with cavernous sinus invasion). IPSS was carried out in 33 (85%) patients. The remission rates for initial surgery were 87% using standard criteria, 58% using the strict criteria (day 3 cortisol < 50 nmol/L). Three patients had an early repeat ETSS for persistent disease (day 3 cortisol 306-555 nmol/L). When the outcome of repeat early ETSS was included, the remission rate was 92% (36/39) overall. Remission rate was 94% (33/35) when patients with macroadenomas were excluded. There were no cases of CSF leakage, meningitis, vascular injury or visual deterioration. Transient and permanent diabetes insipidus occurred in 33 and 23% following first ETSS, respectively. There was one case of recurrence of CD during the follow-up period of 24 (4–79) months. Conclusion Endoscopic transsphenoidal surgery produces satisfactory remission rates for the primary treatment of CD, with higher remission rates for microadenomas. A longer follow-up period is required to assess recurrence rates. Patients should be counselled regarding risk of postoperative diabetes insipidus.

scholarly journals Surgical management of pediatric Cushing's disease: an analysis of 15 consecutive cases at a specialized neurosurgical center

2010 ◽  
Vol 54 (1) ◽  
pp. 17-23 ◽  
Author(s):  
Ricardo Santos de Oliveira ◽  
Margaret de Castro ◽  
Sonir Roberto Rauber Antonini ◽  
Carlos Eduardo Martinelli Júnior ◽  
Ayrton Custódio Moreira ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Transsphenoidal Surgery ◽  
Pediatric Patients ◽  
Serum Cortisol ◽  
Plasma Cortisol Level ◽  
Hormone Deficiency ◽  
Cushing's Disease ◽  
Specialized Center ◽  
Biochemical Cure

OBJECTIVE: The aim of this study was to review the results of surgery for pediatric patients with Cushing's disease who were less than 18 years old and underwent transsphenoidal surgery in a specialized center during a 25-year period. SUBJECTS AND METHODS: Retrospective study, in which the medical records, histology and pituitary imaging of 15 consecutive pediatric patients with Cushing's disease (mean age: 13 years) were evaluated by the same team of endocrinologists and a neurosurgeon from 1982 to 2006. Patients were considered cured when there was clinical adrenal insufficiency and serum cortisol levels were below 1. 8 µg/dL or 50 nmol/L after one, two, three, or seven days following surgery; they therefore required cortisone replacement therapy. Follow-up was for a median time of 11.5 years (range: 2 to 25 years). RESULTS: Clinical and biochemical cure was achieved in 9/15 patients (60%) exclusively after transsphenoidal surgery. Hypopituitarism was observed in four patients; growth hormone deficiency, in two; permanent diabetes insipidus, in one case. CONCLUSIONS: Cushing's disease is rare in children and adolescents. Transsphenoidal surgery is an effective and safe treatment in most of these patients. Plasma cortisol level < 1. 8 µg/dL following surgery is the treatment goal and is a good predictive factor for long-term cure of 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.

Long term follow-up of transsphenoidal surgery for the treatment of Cushing's disease in childhood.

1995 ◽  
Vol 80 (8) ◽  
pp. 2475-2479 ◽  
Author(s):  
M C Leinung ◽  
L A Kane ◽  
B W Scheithauer ◽  
P C Carpenter ◽  
E R Laws ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Transsphenoidal Surgery ◽  
Cushing's Disease ◽  
Long Term Follow Up
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