scholarly journals Neurosurgical Treatment of Nelson’s Syndrome

2002 ◽  
Vol 87 (12) ◽  
pp. 5465-5469 ◽  
Author(s):  
P. A. Kelly ◽  
G. Samandouras ◽  
A. B. Grossman ◽  
F. Afshar ◽  
G. M. Besser ◽  
...  
Keyword(s):  
Pituitary Surgery ◽  
Mass Lesion ◽  
Pituitary Hormone ◽  
Plasma Acth ◽  
Pituitary Mass ◽  
Nelson’S Syndrome ◽  
Nelson's Syndrome ◽  
Median Plasma

Abstract Total bilateral adrenalectomy remains the definitive procedure for cure in Cushing’s disease. It is complicated by the development of Nelson’s syndrome, the treatment of which remains troublesome. We report the long-term follow-up, median 17 yr (range, 8–22 yr), of 13 patients (3 males and 10 females) treated with pituitary surgery for Nelson’s syndrome at a median age of 35 yr (range, 21–67 yr). The presence of a pituitary mass lesion necessitated neurosurgery in all. Preoperatively, the median plasma ACTH level was 664 pmol/liter (range, 92–3665 pmol/liter); this fell to 29 pmol/liter (range, <2 to 1124 pmol/liter) postoperatively (P < 0.0005). Cutaneous hyperpigmentation was reduced in all and resolved in 11 patients. The pituitary tumor bulk was clearly reduced in 12 patients. There was no perioperative mortality. No patient developed a visual field defect attributable to surgery. New anterior pituitary hormone deficiency occurred in seven patients, and permanent diabetes insipidus occurred in five patients. At latest follow-up, the median plasma ACTH is 73 pmol/liter (range, <2 to 7759 pmol/liter); six patients have levels of less than 44 pmol/liter and also have a resolution of their pigmentation and no evidence of a recurrence of a pituitary mass lesion. We conclude that pituitary surgery is an efficacious treatment for mass lesions associated with Nelson’s syndrome, has long-term benefit with minimal side effects, and must be considered in the management of this distressing complication.

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

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 Corticotroph tumor progression after bilateral adrenalectomy (Nelson’s syndrome): systematic review and expert consensus recommendations

2021 ◽  
Vol 184 (3) ◽  
pp. P1-P16 ◽  
Author(s):  
Martin Reincke ◽  
Adriana Albani ◽  
Guillaume Assie ◽  
Irina Bancos ◽  
Thierry Brue ◽  
...  
Keyword(s):  
Tumor Progression ◽  
Cumulative Incidence ◽  
Case Series ◽  
Pituitary Surgery ◽  
Bilateral Adrenalectomy ◽  
Nelson’S Syndrome ◽  
Nelson's Syndrome ◽  
Consensus Recommendations ◽  
First Line Therapy

Background Corticotroph tumor progression (CTP) leading to Nelson’s syndrome (NS) is a severe and difficult-to-treat complication subsequent to bilateral adrenalectomy (BADX) for Cushing’s disease. Its characteristics are not well described, and consensus recommendations for diagnosis and treatment are missing. Methods A systematic literature search was performed focusing on clinical studies and case series (≥5 patients). Definition, cumulative incidence, treatment and long-term outcomes of CTP/NS after BADX were analyzed using descriptive statistics. The results were presented and discussed at an interdisciplinary consensus workshop attended by international pituitary experts in Munich on October 28, 2018. Results Data covered definition and cumulative incidence (34 studies, 1275 patients), surgical outcome (12 studies, 187 patients), outcome of radiation therapy (21 studies, 273 patients), and medical therapy (15 studies, 72 patients). Conclusions We endorse the definition of CTP-BADX/NS as radiological progression or new detection of a pituitary tumor on thin-section MRI. We recommend surveillance by MRI after 3 months and every 12 months for the first 3 years after BADX. Subsequently, we suggest clinical evaluation every 12 months and MRI at increasing intervals every 2–4 years (depending on ACTH and clinical parameters). We recommend pituitary surgery as first-line therapy in patients with CTP-BADX/NS. Surgery should be performed before extrasellar expansion of the tumor to obtain complete and long-term remission. Conventional radiotherapy or stereotactic radiosurgery should be utilized as second-line treatment for remnant tumor tissue showing extrasellar extension

scholarly journals Long-term remission of acromegaly after somatostatin analogues withdrawal: a single-centre experience

2021 ◽  
Author(s):  
E. Sala ◽  
G. Carosi ◽  
G. Del Sindaco ◽  
R. Mungari ◽  
A. Cremaschi ◽  
...  
Keyword(s):  
Median Time ◽  
Pituitary Surgery ◽  
Somatostatin Analogues ◽  
Exclusion Criterion ◽  
Single Centre ◽  
Disease Evolution ◽  
Single Centre Experience ◽  
Pituitary Irradiation

Abstract Purpose A long-lasting remission of acromegaly after somatostatin analogues (SAs) withdrawal has been described in some series. Our aim was to update the disease evolution after SAs withdrawal in a cohort of acromegalic patients. Methods We retrospectively evaluated 21 acromegalic patients previously included in a multicentre study (Ronchi et al. 2008), updating data at the last follow-up. We added further 8 patients selected for SAs withdrawal between 2008–2018. Pituitary irradiation represented an exclusion criterion. The withdrawal was suggested after at least 9 months of clinical and hormonal disease control. Clinical and biochemical data prior and after SAs withdrawal were analysed. Results In the whole cohort (29 patients) mean age was 50 ± 14.9 years and 72.4% were females. In 69% pituitary surgery was previously performed. Overall, the median time of treatment before SAs withdrawal was 53 months (IQR = 24–84). At the last follow up in 2019, 23/29 patients (79.3%) had a disease relapse after a median time of 6 months (interquartile range or IQR = 3–12) from the drug suspension, while 6/29 (20.7%) were still on remission after 120 months (IQR = 66–150). IGF-1 levels were significantly lower before withdrawal in patients with persistent remission compared to relapsing ones (IGF-1 SDS: -1.5 ± 0.6 vs -0.11 ± 1, p = 0.01). We did not observe any other difference between patients with and without relapse, including SAs formulation, dosage and treatment duration. Conclusion A successful withdrawal of SAs is possible in a subset of well-controlled acromegalic patients and it challenges the concept that medical therapy is a lifelong requirement.

Long-Term Outcomes of Patients with Acromegaly - A Report from the Swedish Pituitary Register

2022 ◽  
Author(s):  
Steinunn Arnardóttir ◽  
Jacob Järås ◽  
Pia Burman ◽  
Katarina Berinder ◽  
Per Dahlqvist ◽  
...  
Keyword(s):  
High Rate ◽  
Hormone Deficiency ◽  
Visual Field Defects ◽  
Mortality Data ◽  
Pituitary Hormone ◽  
Biochemical Control ◽  
Long Term Outcomes ◽  
Over Time

Objective: To describe treatment and long-term outcomes of patients with acromegaly from all health-care regions in Sweden. Design and Methods: Analysis of prospectively reported data from the Swedish Pituitary Register of 698 patients (51% females) with acromegaly diagnosed from 1991-2011. The latest clinical follow-up date was December, 2012, while mortality data were collected for 28.5 years until June, 2019. Results: The annual incidence was 3.7/million; 71% of patients had a macroadenoma, 18% had visual field defects, and 25% had at least one pituitary hormone deficiency. Eighty-two percent had pituitary surgery, 10% radiotherapy and 39% medical treatment. At the 5- and 10-year follow-ups, IGF-I levels were within the reference range in 69% and 78% of patients, respectively. In linear regression the proportion of patients with biochemical control including adjuvant therapy at 10 year follow-up increased over time with 1.23 % per year. The SMR (95% CI) for all patients was 1.29 (1.11-1.49). For patients with biochemical control at the latest follow-up, SMR was not increased, neither among patients diagnosed 1991-2000, SMR 1.06 (0.85-1.33) or 2001-2011, SMR 0.87 (0.61-1.24). In contrast, non- controlled patients at the latest follow up from both decades had elevated SMR, 1.90 (1.33-2.72) and 1.98 (1.24-3.14), respectively. Conclusions: The proportion of patients with biochemical control increased over time. Patients with biochemically controlled acromegaly have normal life expectancy while non-controlled patients still have increased mortality. The high rate of macroadenomas and unchanged age at diagnosis illustrates the need for improvements in the management of patients with acromegaly.

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 Combined treatment for acromegaly with long-acting somatostatin analogs and pegvisomant: long-term safety for up to 4.5 years (median 2.2 years) of follow-up in 86 patients

2009 ◽  
Vol 160 (4) ◽  
pp. 529-533 ◽  
Author(s):  
SJCMM Neggers ◽  
WW de Herder ◽  
JAMJL Janssen ◽  
RA Feelders ◽  
AJ van der Lely
Keyword(s):  
Combined Therapy ◽  
Combined Treatment ◽  
Somatostatin Analogs ◽  
Pituitary Surgery ◽  
Median Dosage ◽  
Previously Treated ◽  
Long Acting

BackgroundWe previously reported on the efficacy, safety, and quality of life (QoL) of long-acting somatostatin analogs (SSA) and (twice) weekly pegvisomant (PEG-V) in acromegaly and improvement after the addition of PEG-V to long-acting SSA.ObjectiveTo assess the long-term safety in a larger group of acromegalic patients over a larger period of time: 29.2 (1.2–57.4) months (mean (range)).DesignPegvisomant was added to SSA monotherapy in 86 subjects (37 females), to normalize serum IGF1 concentrations (n=63) or to increase the QoL. The median dosage was 60.0 (20–200) mg weekly.ResultsAfter a mean treatment period of 29.2 months, 23 patients showed dose-independent PEG-V related transient liver enzyme elevations (TLEE). TLEE occurred only once during the continuation of combination therapy, but discontinuation and re-challenge induced a second episode of TLEE. Ten of these patients with TLEE also suffered from diabetes mellitus (DM). In our present series, DM had a 2.28 odds ratio (CI 1.16–9.22; p=0.03) higher risk for developing TLEE. During the combined therapy, a clinical significant decrease in tumor size by more than 20% was observed in 14 patients. Two of these patients were previously treated by pituitary surgery, 1 with additional radiotherapy and all other patients received primary medical treatment.ConclusionLong-term combined treatment with SSA and twice weekly PEG-V up to more than 4 years seems to be safe. Patients with both acromegaly and DM have a 2.28 higher risk of developing TLEE. Clinical significant tumor shrinkage was observed in 14 patients during combined treatment.

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.

Clinical and biochemical stabilization of Nelson’s syndrome with long-term low-dose cabergoline treatment

Pituitary ◽  
2006 ◽  
Vol 9 (2) ◽  
pp. 151-154 ◽  
Author(s):  
Ilana Shraga-Slutzky ◽  
Ilan Shimon ◽  
Ruth Weinshtein
Keyword(s):  
Low Dose ◽  
Nelson’S Syndrome ◽  
Nelson's Syndrome
Sign in / Sign up

Export Citation Format

Share Document