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Bilateral Simultaneous Cavernous Sinus Sampling Using Corticotropin-Releasing Hormone in the Evaluation of Cushing Disease

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Bilateral Simultaneous Cavernous Sinus Sampling Using Corticotropin-Releasing Hormone in the Evaluation of Cushing Disease Bilateral Simultaneous Cavernous Sinus Sampling Using Corticotropin-Releasing Hormone in the Evaluation of Cushing Disease Patrick J. Oliverio, Lee H. Monsein, Gary S. Wand, and Gerard M. Debrun PURPOSE: To determine whether bilateral, simultaneous, cavernous sinus sampling after corti- cotropin-releasing hormone (CRH) stimulation offers as accurate detection and lateralization of Cushing disease as inferior petrosal sinus sampling does. METHODS: Seventeen consecutive patients with hypercortisolism and with high-dose dexamethasone suppression test results sug- gesting Cushing disease underwent bilateral cavernous sinus sampling with CRH stimulation. The diagnosis of Cushing disease was established in all patients by histologic examination or, if no tumor was found at surgery, by subtotal resection of the gland or radiation therapy resulting in eventual hypocortisolism or normal adrenal function and clinical remission. RESULTS: The sen- sitivity of cavernous sinus sampling with and without CRH in detecting Cushing disease was 94% and 71%, respectively. The abnormal side of the pituitary was correctly identified in all patients who had criteria for lateralization, yielding a positive predictive value of 100%. CONCLUSIONS: This small series suggests that cavernous sinus sampling with CRH is as accurate as inferior petrosal sinus sampling in detecting Cushing disease and perhaps more accurate in lateralizing the abnor- mality within the pituitary gland. Index terms: Cushing disease; Cavernous sinus; Pituitary gland, hyperpituitarism; Interventional neuroradiology, provocative testing AJNR Am J Neuroradiol 17:1669–1674, October 1996 Cushing syndrome is characterized by ele- side the hypothalamus. Depression and alco- vated levels of circulating corticosteroids. In the holism can also cause hypersecretion of CRH majority of cases (68%) it is caused by hyper- (3). secretion of corticotropin by the pituitary gland The high-dose dexamethasone suppression (Cushing disease). In 20% of cases it is caused test is used to distinguish between hypersecre- by hypersecretion of cortisol by adrenal tumors tion of corticotropin by the pituitary and by an that can be simulated by administration of ex- ectopic source. Patients with Cushing disease ogenous steroids. In 12% of cases it is caused by generally have greater than 50% suppression of hypersecretion of corticotropin by tumors out- urinary free cortisol and 17-hydroxysteroids side the pituitary (ectopic corticotropin syn- with high-dose dexamethasone. In contrast, pa- drome) (1, 2). Rarely, it can be caused by hy- tients with ectopic corticotropin syndrome gen- persecretion of corticotropin-releasing hormone erally do not have greater than 50% suppression (CRH) by the hypothalamus or by tumors out- with high-dose dexamethasone; however, there may be considerable overlap in the values ob- tained with pituitary and ectopic sources of cor- Received July 5, 1995; accepted after revision March 5, 1996. ticotropin hypersecretion (4). From the Department of Radiology and Radiological Science (P.J.O., Bilateral, simultaneous, inferior petrosal si- L.H.M., G.M.D.) and the Division of Endocrinology, Department of Medicine nus sampling has been reported to have sensi- (G.S.W.), Johns Hopkins University, Baltimore, Md. Address reprint requests to Lee H. Monsein, MD, MPH, Department of tivities and specificities of 95% and 100%, re- Radiology, Washington Hospital Center, 110 Irving St NW, BA 60, Wash- spectively, without CRH stimulation and 100% ington, DC 20010. and 100%, respectively, after CRH stimulation AJNR 17:1669–1674, Oct 1996 0195-6108/96/1709–1669 in distinguishing between pituitary and ectopic q American Society of Neuroradiology sources of corticotropin hypersecretion (5); 1669 1670 OLIVERIO AJNR: 17, October 1996 Findings in 17 patients with hypercortisolism associated with Cushing disease Met Met Showed Showed Cushing Side of Cure MR Baseline Lateralization Lateralization Side of Surgical Pathologic Long-term Patient Criteria Laterali- after Finding Cushing before CRH after CRH Surgery Finding Finding Result after CRH zation Surgery Criteria Stimulation Stimulation Stimulation 1 L fullness Yes Yes Yes Yes L L Tumor Adenoma No Cure 2 R fullness Yes Yes Yes Yes R R Tumor Adenoma Yes Cure 3 Small No Yes No Yes L L No tumor Inconclusive No Cure gland 4 Normal Yes Yes Yes Yes R R Tumor Adenoma No Cure 5 L fullness Yes Yes No Yes L L Tumor Adenoma No Improved 6 L mass Yes Yes Yes Yes L . Not . Cure feasible 7 R fullness No Yes No Yes R R No tumor Normal No Cure 8 R fullness No No No No . R Tumor Adenoma Yes Cure 9 Normal Yes Yes No Yes L L Tumor Adenoma No Lost to follow up 10 Normal Yes Yes Yes Yes R R No tumor Adenoma Yes Still in radiation therapy 11 Normal Yes Yes Yes Yes R R Tumor Adenoma Yes Cure 12 R fullness Yes Yes Yes Yes R R Tumor Adenoma Yes Cure 13 Normal Yes Yes Yes Yes R R Tumor Adenoma No No cure 14 Normal Yes Yes Yes Yes R R Tumor Adenoma No Still in radiation therapy 15 Normal No Yes Yes Yes L L No tumor Normal Yes Cure 16 Normal No Yes No Yes R R Tumor Adenoma Yes Cure 17 Normal Yes Yes No No . Midline Tumor Normal Yes Cure Note.—CRH indicates corticotropin-releasing hormone. however, inferior petrosal sinus sampling after tition time/echo time/excitations]) images were obtained CRH stimulation is able to determine the abnor- before and after intravenous administration of gado- mal side of the pituitary in only 71% of cases pentetate dimeglumine through the pituitary gland with the (5). use of contiguous 3-mm-thick sections. Axial T2-weighted The purpose of this investigation was to de- (3000/30,100/.5 [rectangular field of view]) images were obtained through the brain. Findings on MR images were termine whether bilateral, simultaneous, cav- classified as normal, as showing an asymmetric fullness, ernous sinus sampling after CRH stimulation or as showing a discrete mass. offers as accurate detection and lateralization of Cushing disease as inferior petrosal sinus sam- pling does. Cavernous Sinus Sampling Procedure Ovine CRH (Ferring Pharmaceuticals; Suffern, NY) was used under the manufacturer’s investigational new drug Materials and Methods exemption after approval was obtained from our institu- Clinical Information tion’s Investigational Review Board. Written consent was obtained for the cavernous sinus sampling and for the use We retrospectively reviewed the medical records of 17 of CRH. consecutive patients who had a clinical diagnosis of hy- After intravenous administration of a sedative and a percortisolism thought to be associated with Cushing dis- local anesthetic, a 7F sheath was placed in both common ease and who underwent cavernous sinus sampling be- femoral veins. Heparin (5000 U) was administered intra- tween 1988 and 1995 (see Table). All patients had a venously, with an additional 1000 U administered every high-dose dexamethasone suppression test before cavern- hour during the procedure. ous sinus sampling. The 458-angled tip of a 7F catheter tapered to a 5F catheter (Berenstein; Bard, USCI Division, Billerica, Mass) was positioned in the jugular bulb. A wire-guided 3F mi- Imaging Studies crocatheter tapered to a 2.7F microcatheter (Tracker Hi- Magnetic resonance (MR) imaging studies (at 1.5 T) Flow; Target Therapeutics, Fremont, Calif) was passed were obtained in each patient before cavernous sinus sam- through the 7F catheter and inferior petrosal sinus into the pling. Sagittal and coronal T1-weighted (500/14/1 [repe- cavernous sinus. The tip was positioned at the junction of AJNR: 17, October 1996 CUSHING DISEASE 1671 the cavernous sinus and the inferior petrosal sinus. Digital years (range, 9 to 60 years; SD, 36). All pa- subtraction angiography of the cavernous sinus was per- tients had high-dose dexamethasone suppres- formed to confirm the catheter’s position. A second micro- sion test results that were suggestive of Cushing catheter was positioned in a similar manner on the oppo- site side. disease (2, 8). Simultaneously, 2-mL samples of venous blood were One patient (case 3) had previously under- obtained from both microcatheters and from a peripheral gone successful transphenoidal surgery for vein before, 5 minutes after, and 10 minutes after the Cushing disease and had been disease free for 5 intravenous injection of ovine CRH (1 mg/kg). Plasma was years. He was referred to our institution because immediately prepared from the blood samples and kept of recurrent disease. frozen at 258C until an assay was performed. Normal One patient (case 1) had had a bilateral adre- saline was infused continuously through the guiding cath- nalectomy as a palliative measure 4 years pre- eter during the procedure. Normal saline was infused con- tinuously through the microcatheter when blood was not viously. She had never had pituitary surgery. being withdrawn. Her referral was prompted by the development of Nelson syndrome. All 17 patients had surgery for Cushing dis- Corticotropin Levels and Ratios ease. Seven of the patients (cases 2, 8, 11, 12, The venous corticotropin levels from both sides of the and 15 through 17), including two who had cavernous sinus and a peripheral vein were used to calcu- normal histopathologic findings (case 15 and late side-to-side intercavernous sinus and bilateral cavern- 17), are now free of disease after surgery alone. ous sinus:peripheral vein ratios. Each of these ratios was One patient (case 10) was initially cured after calculated before, 5 minutes after, and 10 minutes after surgery, but had a recurrence 4 years later and CRH administration. A maximum cavernous sinus:periph- eral vein ratio of at least 3:1 was considered suggestive of was referred to us for radiation therapy. Cushing disease (5). A maximum intercavernous sinus Of the eight patients who were not cured after ratio of at least 1.4:1 was used to indicate lateralization of surgery, seven have had further therapy. Six are corticotropin levels (5, 6).
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