Nelson Syndrome

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John A. H. Wass - One of the best experts on this subject based on the ideXlab platform.

  • Nelson Syndrome: definition and management.
    2014
    Co-Authors: Thomas M. Barber, E Adams, John A. H. Wass
    Abstract:

    Nelson Syndrome is an important complication of treatment with total bilateral adrenalectomy (TBA) for patients with refractory Cushing's disease. Although early cases of Nelson Syndrome often presented with the clinical features of large sellar masses, the modern face of Nelson Syndrome has changed primarily due to earlier detection (with highly resolved magnetic resonance imaging (MRI) and sensitive ACTH assays) and greater awareness of the condition, resulting in reduced morbidity and mortality. Although lack of administration of neoadjuvant pituitary radiotherapy post-TBA surgery may predict future development of Nelson Syndrome, other predictive factors remain controversial. Therefore, Nelson Syndrome should be screened for closely and long-term in all patients with a history of Cushing's disease and TBA. The diagnosis of Nelson Syndrome remains controversial, and the pathogenesis of this condition is incompletely understood. Current hypotheses include the “released negative feedback” mechansism (residual pituitary corticotropinoma cells are “released” from the negative feedback effects of cortisol following TBA), and the “aggressive corticotropinoma” mechanism (Nelson Syndrome is most likely to develop in those patients with refractory treatments – including TBA – for an underlying aggressive corticotropinoma). Effective management of Nelson Syndrome with pituitary surgery and radiotherapy is often a challenge. Other therapies (such as Gamma Knife surgery and temozolomide) play an important role and merit further research into their efficacy and placement in the management pathway of Nelson Syndrome.

Edward G. Vates - One of the best experts on this subject based on the ideXlab platform.

Nalini S Shah - One of the best experts on this subject based on the ideXlab platform.

  • localization of remnant and ectopic adrenal tissues with cosyntropin stimulated 18f fdg pet ct in a patient with Nelson Syndrome with persistent hypercortisolism
    2010
    Co-Authors: Anurag R Lila, Gaurav Malhotra, Vijaya Sarathi, Tushar Bandgar, Padma S Menon, Nalini S Shah
    Abstract:

    A27-yr-oldwomanwithCushing’sdisease (CD)hadpersistent hypercortisolism despite two transsphenoidal operations. She underwent bilateral adrenalectomy (BA) by open surgery, which resulted in temporary remission of the disease. Two years after BA, she had a recurrence of Cushingoid features. Diagnostic evaluation showed hypercortisolism [basal cortisol, 29.1 g/dl (800.25 nmol/liter); 2-mg 48-h dexamethasone suppression test serum cortisol, 17 g/dl (467.5 nmol/liter)] and highly elevated basal ACTH [884 pg/ml (194.04 pmol/liter)] with enlargement of the pituitary tumor (8 8 mm), suggestive of Nelson Syndrome with persistent hypercortisolism. The patient underwent 2-(F)-fluoro-2-deoxy-D-glucose positron emission tomography/computed tomography (FFDG-PET/CT) to look for the source of cortisol production. FDG hypermetabolism was seen at the right [standard uptake value (SUV), 2.2] and left (SUV, 1.6) suprarenal regions (marked with metallic clips during previous surgery) and at a focus near the left renal hilum (SUV, 2.0). SUV at these sites increased to 6.1, 6.8, and 7.2, respectively, after 30 min of sc administration of 250 g of cosyntropin (Figs. 1 and 2), confirming them to be adrenal tissues. The patient declined a repeat adrenalectomy/pituitary adenomectomy and opted for pituitary radiotherapy. The prevalence of Nelson Syndrome varies between 8 and 29%(1).PersistenthypercortisolismafterBA is rare (1 in37) inCDpatients (2). Itmaybeduetoresidualorectopicadrenal tissues. In a previously reported case of CD with recurrence of Cushing’s Syndrome after BA, remnant left adrenal tissue was demonstrated using I-adosterol (6 -iodomethyl-19-norcholesterol) (2). We report a similar case where remnant and ectopic adrenal tissues were identified with ACTH-stimulated F-FDG-PET/CT. This diagnostic modality may be used in clinical practice to localize adrenal tissue.

Thomas M. Barber - One of the best experts on this subject based on the ideXlab platform.

  • Nelson Syndrome: definition and management.
    2014
    Co-Authors: Thomas M. Barber, E Adams, John A. H. Wass
    Abstract:

    Nelson Syndrome is an important complication of treatment with total bilateral adrenalectomy (TBA) for patients with refractory Cushing's disease. Although early cases of Nelson Syndrome often presented with the clinical features of large sellar masses, the modern face of Nelson Syndrome has changed primarily due to earlier detection (with highly resolved magnetic resonance imaging (MRI) and sensitive ACTH assays) and greater awareness of the condition, resulting in reduced morbidity and mortality. Although lack of administration of neoadjuvant pituitary radiotherapy post-TBA surgery may predict future development of Nelson Syndrome, other predictive factors remain controversial. Therefore, Nelson Syndrome should be screened for closely and long-term in all patients with a history of Cushing's disease and TBA. The diagnosis of Nelson Syndrome remains controversial, and the pathogenesis of this condition is incompletely understood. Current hypotheses include the “released negative feedback” mechansism (residual pituitary corticotropinoma cells are “released” from the negative feedback effects of cortisol following TBA), and the “aggressive corticotropinoma” mechanism (Nelson Syndrome is most likely to develop in those patients with refractory treatments – including TBA – for an underlying aggressive corticotropinoma). Effective management of Nelson Syndrome with pituitary surgery and radiotherapy is often a challenge. Other therapies (such as Gamma Knife surgery and temozolomide) play an important role and merit further research into their efficacy and placement in the management pathway of Nelson Syndrome.

Eric B. Hintz - One of the best experts on this subject based on the ideXlab platform.