Due to both low frequency and the absence of pathognomonic clinical and/or radiological features, formulating a suspicion diagnosis of PT represents a considerable challenge even for experienced professionals. The indication for treatment should be made on an individual basis, but it is inescapable in the presence of a visual field defect. The surgical approach has to be tailored according to the topography of the tumor and preoperative symptoms; the greatest challenges in accomplishing a gross total removal are represented by the degree of adherence and vascularization of the PT.
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
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