Neurosurg Focus
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Review Case Reports
Ectopic ACTH-secreting pituitary adenoma of the sphenoid sinus: case report of endoscopic endonasal resection and systematic review of the literature.
Ectopic pituitary adenomas are exceedingly rare entities that are often misdiagnosed. The resulting delay in diagnosis may be particularly concerning in the case of Cushing syndrome caused by an ectopic adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma. Although the total resection of ectopic adenomas results in rapid and durable remission, persistent Cushing syndrome is often associated with permanently damaging invasive procedures and significantly higher risk of mortality. ⋯ Any patient presenting with signs and symptoms of Cushing syndrome without any obvious pituitary adenoma or other sources of hypercortisolemia should be thoroughly screened for an ectopic adenoma. However, as with the case presented here, the coincident existence of a sellar mass should not preclude the possibility of an ectopic source. There should be a high degree of clinical suspicion for any mass in the general area surrounding the sella when evaluating Cushing syndrome.
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Nelson's syndrome is a rare clinical manifestation that occurs in 8%-47% of patients as a complication of bilateral adrenalectomy, a procedure that is used to control hypercortisolism in patients with Cushing's disease. First described in 1958 by Dr. Don Nelson, the disease has since become associated with a clinical triad of hyperpigmentation, excessive adrenocorticotropin secretion, and a corticotroph adenoma. ⋯ The most promising pharmacological agents are temozolomide, octreotide, and pasireotide, but these agents are often administered after transsphenoidal surgery. In murine models, rosiglitazone has shown some efficacy, but these results have not yet been found in human studies. In this article, the authors review the clinical manifestations, pathophysiology, diagnostic criteria, and efficacy of multimodal treatment strategies for Nelson's syndrome.
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OBJECT Cushing's disease (CD) is a potentially lethal neuroendocrinopathy that often requires specialized multidisciplinary treatment to achieve optimized outcomes. The authors analyzed data pertaining to patient, hospital, and admission characteristics as they relate to outcomes following transsphenoidal surgery (TSS) in more than 5500 patients treated for CD. METHODS The Nationwide Inpatient Sample (NIS) database was used to identify all patients admitted with CD between 2002 and 2010. ⋯ Patients with emergency admissions had a risk of higher hospital charges (RR 3.06, 95% CI 1.26-7.46; p = 0.01) and nonendocrine complications (RR 3.18, 95% CI 1.22-8.28; p = 0.02). CONCLUSIONS This review of NIS data in more than 5500 patients treated surgically for CD pointed to major outcome disparities predicted primarily by payer status, admission type, and hospital region. Identification and targeting of such barriers to quality health care in patients with CD may help optimize patient outcomes on a national level and present an opportunity to improve access of high-risk patient subgroups to specialty centers of excellence.
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OBJECT Adrenocorticotropic hormone (ACTH) adenomas have been recognized as a more aggressive and invasive subtype of pituitary adenomas. An additional and clinically relevant peculiarity of these tumors is their ability to modify their clinical expression from a silent form to Cushing disease or vice versa. The aim of this study was to review a series of patients with pituitary adenomas and analyze the clinical implications of the transformation of clinical expression in 5 cases that showed this phenomenon. ⋯ Furthermore, this bizarre and unpredictable postoperative tumor behavior can lead to misinterpretation of clinical and endocrinological outcomes. Even if these cases are very rare, they are not anecdotal in large series. Thus, ACTH adenomas require careful biohumoral and neuroradiological follow-up to detect possible transformations.
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OBJECT The purpose of this study was to describe complications associated with the endonasal, transsphenoidal approach for the treatment of adrenocorticotropic hormone (ACTH)-positive staining tumors (Cushing's disease [CD] and silent corticotroph adenomas [SCAs]) performed by 1 surgeon at a high-volume academic medical center. METHODS Medical records from Brigham and Women's Hospital were retrospectively reviewed. Selected for study were 82 patients with CD who during April 2008-April 2014 had consecutively undergone transsphenoidal resection or who had subsequent pathological confirmation of ACTH-positive tumor staining. ⋯ CONCLUSIONS Transsphenoidal surgery is the treatment of choice for patients with CD and other ACTH-positive staining tumors. Recent advances in endoscopic technology and increasing surgeon comfort with this technology are making transsphenoidal procedures safer, faster, and more effective. Serious complications are uncommon and can be managed successfully.