Nelson's Syndrome

Kasperlik-Załuska, A; Bonicki, Wiesław; Jeske, W; Janik, Jadwiga; Zgliczyński, Wojciech; Czernicki, Z

doi:10.1055/s-2006-921430

Cited by 27 publications

(44 citation statements)

References 19 publications

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“…Suboptimal or absent steroid replacement therapy post TBA may increase the risk of developing Nelson's syndrome (41,42) or even result in transformation of a pre-existing adenoma into a more aggressive tumour subtype (3,26). However, most studies have not shown this (3,13,32).…”

Section: Insufficient Exogenous Steroid Replacement Therapy Post Tba mentioning

confidence: 92%

“…Pituitary surgery should be the first-line treatment option for Nelson's syndrome, particularly if there is compression of the optic apparatus (25,42,73) with success rates ranging from 10 to 70% (10,25,55,74). Although the usual approach is transsphenoidal, a transcranial procedure may be adopted if there is extrasellar extension (33% of cases (10)).…”

Section: Pituitary Surgerymentioning

confidence: 99%

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Barber

Adams²,

Ansorge³

et al. 2010

European Journal of Endocrinology

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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 (O500 ng/l) in addition to progressive elevations of ACTH (a rise of O30%) 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.

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Section: Insufficient Exogenous Steroid Replacement Therapy Post Tba mentioning

confidence: 92%

Section: Pituitary Surgerymentioning

confidence: 99%

Nelson's syndrome

Barber

Adams²,

Ansorge³

et al. 2010

European Journal of Endocrinology

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“…However, this last issue remains particularly controversial. While most authors indeed report invasive macroadenomas while presenting cases of patients with NS [8][9][10], other authors emphasise that in 20-40% of cases the condition is associated with pituitary microadenomas [7,11,12]. Notably, the efficacy of surgical treatment of small tumours is much higher when compared to the results achieved in cases of invasive macroadenomas [7,8].…”

Section: Prace Oryginalnementioning

confidence: 94%

“…This rare form of CD is caused by ACTH-secreting pituitary adenoma with unpredictable, usually aggressive clinical course that often leads to serious, life-threatening complications [3,7]. Diagnostic criteria for NS were proposed in 1958 and originally included: abnormally high plasma ACTH, enlargement of the sella turcica shown in X-ray of the skull, vision disturbances re- sulting from compression of the optic chiasm by the enlarging pituitary tumour, and hyperpigmentation of the skin and mucous membranes [1,2].…”

Section: Discussionmentioning

confidence: 99%

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Wyniki leczenia neurochirurgicznego gruczolaków kortykotropowych przysadki w zespole Nelsona — pułapki terapeutyczne

Zieliński

Witek

Maksymowicz

2015

Endokrynologia Polska

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Introduction: Nelson's syndrome (NS) is a rare clinical syndrome caused by an enlarging, aggressive corticotroph pituitary adenoma that can occur following bilateral adrenalectomy performed in the treatment of refractory Cushing's disease (CD). Such tumours respond poorly to currently available therapeutic options, which include surgery, radiotherapy, pharmacotherapy, and chemotherapy. They are a challenging problem in neurosurgical practice. The aim of this work was to evaluate the early and long-term results of microsurgery in a single surgeon's series of patients with NS.

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Nelson syndrome and perinatal challenges: A case report and systematic review of the literature

Jones

Reis

Levy

et al. 2023

Intl J Gynecology & Obste

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Nelson syndrome is a rare and potentially life‐threatening complication of treatment with total bilateral adrenalectomy for women with Cushing disease. A successful term pregnancy following fertility treatment in a patient with Nelson syndrome is presented. Our study provides guidance in the prenatal and intrapartum management of this condition. A case report and a systematic review of 14 papers describing 50 pregnancies are presented. An electronic database search included Medline (1946 to September 2022), Embase (1980 to September 2022), Cochrane Library, and UKOSS. A small number of pregnancies in women with Nelson syndrome are reported in literature, but there are no guidelines. Some authors detail the prenatal care provided to their patients. Four studies report prenatal monitoring with visual field checks and two report monitoring with X‐rays. Five studies report the use of parenteral hydrocortisone at the time of delivery. Where described, women delivered appropriately grown newborns at term, with timing and mode of delivery dictated by obstetric indications. Preconception counseling and optimization of maternal health status improve pregnancy outcomes in women with Nelson syndrome. Multidisciplinary review in a combined obstetric‐endocrine prenatal clinic is ideal. Awareness about potential complications during pregnancy and the postnatal period is crucial in providing optimal care to the mother and baby.

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Nelson's Syndrome - 46 Years Later: Clinical Experience with 37 Patients

Abstract: MRI, ophthalmologic examination and plasma ACTH determination were the most valuable investigations for early diagnosis of Nelson's syndrome. Early neurosurgery offered the best outcome in our group of patients.

Cited by 27 publications

References 19 publications

Nelson's syndrome

Nelson's syndrome

Wyniki leczenia neurochirurgicznego gruczolaków kortykotropowych przysadki w zespole Nelsona — pułapki terapeutyczne

Nelson syndrome and perinatal challenges: A case report and systematic review of the literature

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