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.

scholarly journals Rosiglitazone for prevention or adjuvant treatment of Nelson’s syndrome after bilateral adrenalectomy

2005 ◽  
Vol 153 (4) ◽  
pp. 503-505 ◽  
Author(s):  
Mikkel Andreassen ◽  
Lars Østergaard Kristensen
Keyword(s):  
Magnetic Resonance Imaging ◽  
Transsphenoidal Surgery ◽  
Bilateral Adrenalectomy ◽  
Resonance Imaging ◽  
Initial Value ◽  
Nelson’S Syndrome ◽  
Nelson's Syndrome ◽  
The Third ◽  
Long Term Studies

Objective: To investigate the effect of Rosiglitazone in three patients treated with bilateral adrenalectomy followed by hyperpigmentation and hypersecretion of ACTH. Patients and methods: One patient had increasing ACTH after previous transsphenoidal surgery for Nelson’s syndrome, and two patients without pituitary adenomas had recurrence of Cushing’s disease after primary and repeated transsphenoidal surgery with need for bilateral adrenalectomy. The patients developed hyperpigmentation and increasing ACTH at nadir 2–4 h after morning hydrocortisone dose. ACTH during Rosiglitazone therapy (4 mg/day for 4 weeks and then 8 mg/day) was measured at regular intervals 24 h after the latest dose of hydrocortisone. Results: In two patients there was a decrease in ACTH by 40% after 5 months. The first of these patients showed an escape with increasing ACTH to the initial value after 11 months. In the third patient no effect was observed. Tumour development or progression on magnetic resonance imaging was not observed. Conclusion: Rosiglitazone might represent an adjuvant therapy in patients with ACTH hypersecretion. Larger long-term studies are needed.

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.

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

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 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.

scholarly journals Aggressive Pituitary Macroadenoma Treated With Capecitabine and Temozolomide Chemotherapy Combination in a Patient With Nelson’s Syndrome: A Case Report

2021 ◽  
Vol 12 ◽  
Author(s):  
Oriol Mirallas ◽  
Francesca Filippi-Arriaga ◽  
Irene Hernandez Hernandez ◽  
Anton Aubanell ◽  
Anas Chaachou ◽  
...  
Keyword(s):  
Therapeutic Option ◽  
Clinical Manifestations ◽  
Pituitary Tumors ◽  
Complete Response ◽  
Severe Side Effect ◽  
Off Label Use ◽  
Nelson’S Syndrome ◽  
Nelson's Syndrome ◽  
Local Brain ◽  
Temozolomide Chemotherapy

Nelson’s syndrome is considered a severe side effect that can occur after a total bilateral adrenalectomy in patients with Cushing’s disease. It usually presents with clinical manifestations of an enlarging pituitary tumor including visual and cranial nerve alterations, and if not treated, can cause death through local brain compression or invasion. The first therapeutic option is surgery but in extreme cases of inaccessible or resistant aggressive pituitary tumors; the off-label use of chemotherapy with capecitabine and temozolomide can be considered. However, the use of this treatment is controversial due to adverse events, lack of complete response, and inability to predict results. We present the case of a 48-year-old man diagnosed with Nelson’s syndrome with prolonged partial response and significant clinical benefit to treatment with capecitabine and temozolomide.

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

Pituitary cushing's syndrome and nelson's syndrome: Diagnostic criteria, surgical therapy, and results

1981 ◽  
Vol 16 (5) ◽  
pp. 316-323 ◽  
Author(s):  
Frank W. Guthrie ◽  
Ivan Ciric ◽  
Steven Hayashida ◽  
William D. Kerr ◽  
E.Dennis Murphy
Keyword(s):  
Diagnostic Criteria ◽  
Surgical Therapy ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Nelson’S Syndrome ◽  
Nelson's Syndrome

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

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