Nelson’s syndrome post-bilateral adrenalectomy

Abstract

Rapid Nelson's syndrome development after bilateral adrenalectomy is a rare event. A 34-year-old man was referred in 2012 with suspected Cushing's syndrome (CS). Hormonal investigations showed hypercortisolism [serum cortisol 49.2 lg/dl (normal range, NR: 5-25); 24 h urinary-free cortisol, UFC 1903 lg/24 h (NR: <140); serum cortisol after 1 mg dexametasone 23:00 h, 42.1 lg/dl (N < 1.8)] with increased plasma ACTH concentration (120 pg/ml; NR: 5-46)]. Magnetic resonance imaging (MRI) of the pituitary gland was normal (Figure 1A). Inferior petrosal sinus sampling (IPSS) with plasma ACTH quantification before and after 100 lg of corticotropin-releasing hormone (CRH) showed no gradient of central/peripheral plasma ACTH both baseline (IPS:peripheral ratio < 2) and post-CRH (IPS:peripheral ratio <3) suggesting ectopic ACTH secretion (EAS). Localizing studies by chest radiography, thoracoabdominal CT scan, abdominal MRI, octreoscan, bronchoscopy, echo-endoscopy, colonoscopy and 18

Hyperpigmentation and ACTH – an overview of literature

“…nelson's syndrome represents another particular situation where aCtH increase is part of the pathogenic loop and its values can get extremely high (22,23,24). this happens after bilateral adrenalectomy for Cushing's disease and it represents a rather atypical option nowadays due to others several medical and surgical therapeutical resources which are preferred (22,25,26). the complication is rare; it involves high aCtH with known complications including the skin,and corticotropinoma enlargement (26,27).…”

“…this happens after bilateral adrenalectomy for Cushing's disease and it represents a rather atypical option nowadays due to others several medical and surgical therapeutical resources which are preferred (22,25,26). the complication is rare; it involves high aCtH with known complications including the skin,and corticotropinoma enlargement (26,27). Prior radiotherapy might be protec-tive, yet the general data are inconclusive (26,27).…”

“…the complication is rare; it involves high aCtH with known complications including the skin,and corticotropinoma enlargement (26,27). Prior radiotherapy might be protec-tive, yet the general data are inconclusive (26,27). there is not general guideline as approach but radiotherapy, neurosurgery of the pituitary gland an and potential use of somatostatin analogues and/or dopamine agonists are the therapy lines (26,27).…”

“…Prior radiotherapy might be protec-tive, yet the general data are inconclusive (26,27). there is not general guideline as approach but radiotherapy, neurosurgery of the pituitary gland an and potential use of somatostatin analogues and/or dopamine agonists are the therapy lines (26,27). a study published in 2019 showed that 38% of the subjects with nelson's syndrome displayed some level of progression during a follow-up of 10 years (28).…”