American Association of Endocrine SurgeonNelson’s syndrome after bilateral adrenalectomy for Cushing’s disease
Section snippets
Patients and methods
Clinical records of patients subjected to bilateral adrenalectomy for Cushing’s disease at our institution from March 1990 to June 2005 were reviewed for skin hyperpigmentation, ACTH levels, growth of a previous or new pituitary tumor, details of the adrenal surgery, outcome of Cushing symptoms, episodes of adrenal insufficiency and radiotherapy. The latter was considered prophylactic when given prior to or immediately after bilateral adrenalectomy and before development of Nelson’s syndrome,
Results
Our study included 39 patients: 32 females (82%) and 7 males (18%), with a diagnosis of primary or recurrent Cushing’s disease and a minimum follow-up of 12 months. Mean age was 31 years (range, 15 to 58).
The first 11 adrenalectomies were performed using the open posterior approach; the subsequent 28 were laparoscopic (lateral intraperitoneal) with 1 conversion due to bleeding and excessive adipose tissue. Mean operative time was 229 min (range, 180 to 270) for the open procedures and 298 min
Discussion
Transsphenoidal hypophysectomy has proven to be the most cost-effective treatment for patients with Cushing’s disease and is the preferred initial therapy worldwide. Pituitary surgery has an average success rate between 75% and 80%, and a recurrence rate from 7% to 12% in successfully operated patients.6 Although bilateral adrenalectomy is an effective treatment for Cushing’s disease, it is weighed down by significant perioperative mortality and morbidity rates, by the risk of developing
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Cited by (46)
Updates in the outcomes of radiation therapy for Cushing's disease
2021, Best Practice and Research: Clinical Endocrinology and MetabolismNelson's Syndrome: An Update
2020, Endocrinology and Metabolism Clinics of North AmericaCitation Excerpt :Controversy also exists on the role of pituitary radiotherapy in preventing NS development (administered either as therapeutic intervention for the hypercortisolism [prior to BLA] or as prophylactic measure [at the time of BLA]). Although some studies have shown a protective effect,20,22,32 this has not been confirmed in others.23,24 Young age at the time of BLA has been proposed as a risk factor by Kemink and colleagues,27 but subsequent studies did not confirm this,14,22,24 whereas short duration of CD also has been found to be predictive,14 but not in all studies.22,27
Cushing's syndrome – Disease monitoring: Recurrence, surveillance with biomarkers or imaging studies
2020, Best Practice and Research: Clinical Endocrinology and MetabolismCitation Excerpt :Many of the studies in the analysis were, however, limited by a small number of patients, short follow-up time and lack of a clear definition of the syndrome. Twelve studies, including more than 30 patients treated for CD with bilateral adrenalectomy, with provided criteria for Nelson's syndrome, have been published during the last 30 years (Table 2) [5,11,14,22,84–86,88–92]. In these studies, the overall rate of Nelson's syndrome is 32% (201 of 627 patients), varying from 17 to 53%.
Prevention, diagnosis and treatment of Nelson's syndrome
2018, Current Opinion in Endocrine and Metabolic ResearchNelson Syndrome: Update on Therapeutic Approaches
2015, World NeurosurgeryNelson syndrome: Definition and management
2014, Handbook of Clinical NeurologyCitation Excerpt :Such patients also have a higher chance of relapse following TSS (7–12% of patients with Cushing's disease treated with TSS have a relapse within one year following the procedure (Lanzi et al., 1998)) and subsequent need for TBA. Consistent with this hypothesis is the observation that compared with patients with less aggressive forms of pituitary tumor, patients with invasive corticotropinomas at the time of TSS are also at greater risk of subsequently developing Nelson syndrome at an earlier stage following TBA (Nagesser et al., 2000; Gil-Cardenas et al., 2007). The de novo development of a new corticotropinoma that is clonally distinct from the original tumor is also possible in some patients, although there is little evidence to support this alternative theory.