scholarly journals Stereotactic radiosurgery for Cushing disease

2004 ◽  
Vol 16 (4) ◽  
pp. 1-7 ◽  
Author(s):  
Stephen J. Hentschel ◽  
Ian E. McCutcheon

The most common cause of Cushing syndrome is Cushing disease, in which hypercortisolism is produced by a functional adrenocorticotropic hormone–producing adenoma of the anterior pituitary gland. The common therapies available include microsurgical resection, conventional fractionated radiotherapy, and stereotactic radiosurgery (SRS). In this article the authors review the indications, results, and complications associated with SRS in the treatment of Cushing disease. In as many as 90% of patients SRS results in disease remission, which is defined as a normal 24-hour urinary free cortisol level and a normal or subnormal morning serum cortisol level. Although in most patients who are subsequently cured a marked decrease in the serum cortisol level is demonstrated within 3 months after treatment, a biochemical cure may be delayed up to 3 years in some cases. Complications following SRS for pituitary adenomas are uncommon, particularly in patients with microadenomas, which are most commonly seen in Cushing disease. The most common complication is hypopituitarism, which occurs in up to 50% of patients with a mean latency period of 5 years. Radiation-induced optic neuropathy has been reported in less than 2% of cases and induction of a secondary neoplasm in less than 1% of cases. For patients with Cushing disease, the rate of endocrinological cure following SRS appears to be similar to that attained using microsurgical resection. In contrast to surgery, SRS has the benefit of being noninvasive and associated with a very low incidence of diabetes insipidus, although hypopituitarism may be more common with SRS. With continued follow-up patient reviews and additional experience with SRS, it may become possible to make more definitive statements regarding SRS as the initial treatment for patients with Cushing disease.

2020 ◽  
Vol 106 (1) ◽  
pp. e94-e102
Author(s):  
Fang Wang ◽  
Michael P Catalino ◽  
Wenya Linda Bi ◽  
Ian F Dunn ◽  
Timothy R Smith ◽  
...  

Abstract Context Recurrence of Cushing disease (CD) can occur even decades after surgery. Biomarkers to predict recurrence of CD after surgery have been studied but are inconclusive. Objective The aim of our study was to identify specific biomarkers that can predict long-term remission after neurosurgery. Design Identification of specific biomarkers to predict long-term remission of CD was performed by logistic regression analysis followed by Kaplan–Meier survival analysis, using recurrence as the dependent variable. Setting A total of 260 patients with CD identified from our institutional research patient data registry search tool and from patients who presented to our longitudinal multidisciplinary clinic between May 2008 and May 2018 underwent statistical analysis. Interventions Data on clinical features, neuro-imaging study, pathology, biochemistry, and treatments were collected by reviewing digital chart records. Main Outcome Measure Postoperative cortisol as a biomarker to predict long-term remission after surgical treatment for CD. Results By logistic regression analysis, postoperative day 1 (POD1) morning (5-10 am) serum cortisol, female sex, and proliferative index had significant association with CD recurrence (odds ratio [OR] = 1.025, 95% CI: 1.002-1.048, P = .032). In contrast, the postoperative nadir cortisol (OR = 1.081, 95% CI: 0.989-1.181, P = .086), urinary free cortisol (OR = 1.032, 95% CI: 0.994-1.07, P = .098), and late night salivary cortisol (OR = 1.383, 95% CI: 0.841-2.274, P = .201) had no significant correlation with recurrence. A significant association between POD1 morning serum cortisol and long-term CD remission was verified by Kaplan–Meier analysis when using POD1 morning serum cortisol <5 μg/dL as the cut-off. Conclusions The POD1 morning serum cortisol level has a significant association with CD recurrence.


2018 ◽  
Vol 3 (1) ◽  
pp. 1-12 ◽  
Author(s):  
Ty B Carroll ◽  
William J Peppard ◽  
David J Herrmann ◽  
Bradley R Javorsky ◽  
Tracy S Wang ◽  
...  

Abstract Objective Demonstrate the safety and efficacy of a standardized intravenous etomidate infusion protocol in normalizing cortisol levels in patients with severe and life-threatening hypercortisolism. Methods A retrospective case series of seven patients representing nine episodes of severe hypercortisolism at two large academic medical centers was conducted. Patients were included in this series if they received an etomidate infusion for the treatment of severe and life-threatening hypercortisolism. The etomidate infusion was administered via a newly developed protocol designed to safely reduce cortisol levels until more long-term medical or definitive surgical therapy could be instituted. Results Seven patients representing nine episodes received etomidate treatment. In eight of nine episodes of therapy, rapid control of hypercortisolemia was achieved, generally defined as a serum cortisol level of 10 to 20 µg/dL. Patients with a median baseline cortisol of 105 µg/dL (range, 32 to 245 µg/dL) achieved a median nadir serum cortisol of 15.8 µg/dL (range, 6.9 to 27 µg/dL) after a median of 38 hours (range, 26 to 134 hours). Conclusions A standardized continuous intravenous etomidate infusion protocol is a safe and effective means of achieving a serum cortisol level of 10 to 20 µg/dL in patients with severe hypercortisolemia.


2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Farah Laith Al Sabie ◽  
Meenu Jain ◽  
Ammara Aziz

Abstract Introduction: Cushing disease refers to the endogenous overproduction of glucocorticoid due to an ACTH-producing pituitary adenoma. It is important to recognize and treat due to the adverse health outcomes associated with it. We describe an unusual case of Cushing disease which presented very rapidly and progressively with extremely high cortisol levels mimicking those seen in ectopic production of ACTH.Case Presentation: A 43 year old Caucasian man, with no past medical history, presented with hypertensive crisis. He was discharged home with anti-hypertensive medications. Over the next 4 months, he gained 20 pounds, mainly around his abdomen, developed fatigue, and blood pressure continued to be high despite six anti-hypertensive medications, developed diabetes and hypokalemia, requiring 120 meq/day of potassium chloride. On exam, he had plethora, central obesity and wide, purple striae over his abdomen. Work-up for secondary causes of hypertension showed normal renal Doppler US, normal aldosterone and renin activity, normal plasma metanephrines, however, his 24 hour urinary free cortisol was dramatically elevated at 4022ug/day with a urine volume of 4 L, 1 mg dexamethasone suppression test showed unsuppressed serum cortisol of 55ug/dl. Morning ACTH of 125 pg/ml with concurrent serum cortisol level of 53.8 mcg/dl, indicated ACTH-dependent hypercortisolism. Inferior petrosal sinus sampling indicated a pituitary source of ACTH. Sellar MRI initially did not show a pituitary adenoma, however, repeat MRI with a 3-Tesla magnet showed a 4 mm pituitary adenoma. He was treated with ketoconazole and was started on atovaquone for PCP prophylaxis while awaiting trans-sphenoidal resection, which he had a month later. Pathology showed a 4 mm adenoma which stained strongly for ACTH. On postoperative day 1, serum cortisol dropped to 2.1 from 52.3 mcg/dl, and patient was discharged on hydrocortisone replacement. Three weeks later, he had lost 12 pounds, hyperglycemia improved with discontinuation of insulin, hypokalemia resolved and hypertension was well controlled on two anti-hypertensives. Discussion: ACTH-dependent Cushing syndrome is either caused by Cushing disease, or from ectopic ACTH production from a tumor. Cushing disease is characterized by a gradual onset and subtle manifestations of hypercortisolism. Acute, severe presentation favors an ectopic ACTH producing tumor, and is associated with much higher cortisol levels. In our patient, clinical data suggested ectopic ACTH production, yet he was found to have Cushing disease, and was treated successfully with trans-sphenoidal resection of the pituitary adenoma. It is imperative to consider all possibilities, and do the full work up so as not to miss an atypical presentation of Cushing disease, and direct treatment accordingly.


2019 ◽  
Author(s):  
Liana Khatsimova ◽  
Uliana Tsoy ◽  
Natalia Kuritsyna ◽  
Elena Grineva ◽  
Elena Litvinenko ◽  
...  

Neurosurgery ◽  
1979 ◽  
Vol 5 (5) ◽  
pp. 559-565 ◽  
Author(s):  
Paul Steinbok ◽  
Gordon Thompson

Abstract Serial estimations of serum cortisol were performed in 49 patients with craniocerebral trauma. Abnormalities of serum cortisol, including alterations in diurnal rhythm and elevations of serum cortisol level, occurred in 21 patients. The frequency and severity of the abnormalities correlated with the severity of the head injury, and there was a trend suggesting that middle fossa basal skull fractures predisposed to cortisol abnormalities. A further 6 patients were studied to assess the effects of exogenous dexamethasone, and in all patients there was suppression of elevated serum cortisol levels by the dexamethasone. The findings suggest that hypercortisolemia after head injury is related to an alteration rather than an abolition of the normal feedback mechanism.


1970 ◽  
Vol 2 ◽  
pp. 28-33
Author(s):  
SM Selimuzzaman ◽  
Noorzahan Begum ◽  
Nadira Islam ◽  
Shelina Begum

The study was designed to observe the effects of surgical stress on serum level of cortisol in patients undergoing surgical treatment and to find out any differences in hormonal response between elective and emergency surgical procedures. A total number of 60 male subjects aged between 18 and 45 years were included in the study. Of them, 20 were healthy control (Group I), 20 underwent elective surgical treatment (Group II) and emergency surgical interventions were applied in rest 20 subjects (Group III). Study Groups were further divided into subgroups A (preoperative samples were collected 1- hour before operation), B (postoperative samples were collected 1-hour after the end of the operation) and C (postoperative samples were collected 24-hours after operation).Serum cortisol level was estimated by invitro-immunolytic test.Statistical analysis was done by paired, unpaired ‘t' test and regression analysis. The preoperative mean serum cortisol concentration in elective surgical cases was almost similar to that of healthy control. On the contrary, in the emergency surgical cases, a significantly increased mean cortisol level were observed (I vs IIIA and IIA vs IIIA; P < 0.05). The serum cortisol concentrations were increased both in elective and emergency surgical cases after operations but the magnitude of rise was more marked in emergency group of patients (IIB vs IIIB; P < 0.05). Therefore, this study reveals that surgical intervention causes increase in serum cortisol which is more marked in emergency procedure. Key Words: Stress; Cortisol; Surgery DOI:10.3329/jbsp.v2i0.981 J Bangladesh Soc Physiol. 2007 Dec;(2): 28-33.


Author(s):  
Teresa M Canteros ◽  
Valeria De Miguel ◽  
Patricia Fainstein-Day

Summary Severe Cushing syndrome (SCS) is considered an emergency that requires immediate treatment to lower serum cortisol levels. Fluconazole may be considered an alternative treatment in Cushing syndrome when ketoconazole is not tolerated or unavailable. We report a 39-year-old woman with a history of partial pancreaticoduodenectomy due to a periampullary neuroendocrine tumor with locoregional extension. Three years after surgery, she developed liver metastases and was started on 120 mg of lanreotide/month, despite which, liver metastases progressed in the following 6 months. The patient showed extreme fatigue, muscle weakness, delirium, moon face, hirsutism and severe proximal weakness. Laboratory tests showed anemia, hyperglycemia and severe hypokalemia. 24-h urinary free cortisol: 2152 nmol/day (reference range (RR): <276), morning serum cortisol 4883.4 nmol/L (RR: 138–690), ACTH 127.3 pmol/L (RR: 2.2–10). She was diagnosed with ectopic ACTH syndrome (EAS). On admission, she presented with acute upper gastrointestinal tract bleeding and hemodynamic instability. Intravenous fluconazole 400 mg/day was started. After 48 h, her mental state improved and morning cortisol decreased by 25%. The dose was titrated to 600 mg/day which resulted in a 55% decrease in cortisol levels in 1 week, but then had to be decreased to 400 mg/day because transaminase levels increased over 3 times the upper normal level. After 18 days of treatment, hemodynamic stability, lower cortisol levels and better overall clinical status enabled successful bilateral adrenalectomy. This case report shows that intravenous fluconazole effectively decreased cortisol levels in SCS due to EAS. Learning points: Severe Cushing syndrome can be effectively treated with fluconazole to achieve a significant improvement of hypercortisolism prior to bilateral adrenalectomy. Intravenous fluconazole is an alternative treatment when ketoconazole is not tolerated and etomidate is not available. Fluconazole is well tolerated with mild side effects. Hepatotoxicity is usually mild and resolves after drug discontinuation.


2019 ◽  
Vol 12 ◽  
pp. 117955141982583 ◽  
Author(s):  
Cristina Familiar ◽  
Ane Azcutia

Olfactory neuroblastoma (ONB) is an unusual malignant neoplasm originating from the olfactory neuroepithelium. Secretion of adrenocorticotropic hormone (ACTH) from this tumor has been exceptionally reported. We describe a young man with resistant hypertension and a cushingoid phenotype. After hormonal confirmation of an ACTH-dependent Cushing syndrome, non-invasive dynamic tests were carried out to evaluate the cause of the ACTH source. Plasma cortisol decrease after a high-dose dexamethasone suppression test and cortisol increase after a desmopressin (DDAVP) stimulation test suggested a Cushing disease. A magnetic resonance image (MRI) of the brain and an Indium-111 octreotide scan revealed a large mass centered in the sphenoid sinus with lateral and posterior extension. An ACTH secreting ONB was confirmed with a trasnasal biopsy. Patient was offered a combined therapy with surgical resection and radiotherapy but refused surgery. The neoplasm was treated with neoadjuvant cisplatin-based chemotherapy followed by fractionated radiotherapy. Hypercortisolism initially improved with metyrapone but normocortisolism was only achieved after local control of the tumor with radiotherapy. Clinical presentation of ONB is usually related to local symptoms (as nasal obstruction and epistaxis) dependent on its ubication and extension. Cushing syndrome from ACTH production is a rare manifestation of ONB. This case also underlies the difficulties related to the interpretation of dynamic endocrine tests in Cushing syndrome.


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