Transsphenoidal surgery in Cushing disease: 10 years of experience in 34 consecutive cases

2004 ◽  
Vol 100 (4) ◽  
pp. 634-638 ◽  
Author(s):  
Charlotte Höybye ◽  
Eva GrenbäcK ◽  
Marja Thorén ◽  
Anna-Lena Hulting ◽  
Lars Lundblad ◽  
...  

Object. Cushing disease is a rare disorder. Because of their small size the adrenocorticotropic hormone (ACTH)—producing tumors are often not detectable on neuroimaging studies. To obtain a cure with transsphenoidal surgery (TSS) may therefore be difficult. In this report the authors present 10 years of experience in the treatment of patients with Cushing disease who were followed up with the same protocol and treated by the same surgeon. Methods. Thirty-four patients, 26 of them female and eight of them male (mean age 40 years, range 13–74 years) were studied. All had obvious clinical signs and symptoms of Cushing syndrome. Magnetic resonance (MR) imaging was performed in all patients, and inferior petrosal sinus (IPS) sampling was done in 14. In 12 patients MR imaging indicated a pituitary tumor; 10 were microadenomas and two were macroadenomas. In six patients with no visible tumor, the results of IPS sampling supported the diagnosis. All patients underwent TSS; the mean follow-up duration was 6 ± 0.5 years. Selective adenomectomy was performed in 32 and hemihypophysectomy in the other two patients. A cure was obtained in 31 patients (91%) after one TSS and in two more patients after further TSS; one patient was not cured despite two TSSs and one underwent bilateral adrenalectomy. Disease recurrence was seen in two patients after 3 years, and they were successfully treated with stereotactic gamma knife surgery. Half of the patients had an ACTH deficiency postoperatively, whereas one third had other pituitary hormone insufficiencies. There were no serious complications attributable to the surgical intervention. Conclusions. Transsphenoidal surgery with selective adenomectomy is an effective and safe treatment for Cushing disease. In the patients presented in this study, the surgical outcome seemed to depend on careful preoperative evaluation and the surgeon's experience. For optimal results in this rare disease the authors therefore suggest that the endocrinological, radiological, and surgical procedures be coordinated in a specialized center.

1998 ◽  
Vol 88 (6) ◽  
pp. 949-953 ◽  
Author(s):  
Masanori Kabuto ◽  
Toshihiko Kubota ◽  
Hidenori Kobayashi ◽  
Hiroaki Takeuchi ◽  
Takao Nakagawa ◽  
...  

Object. The authors have used a silicone plate for reconstruction of the sellar floor during rhinoseptoplastic transsphenoidal surgery because it has greater elasticity and is easier to carve than nasal septal cartilage and sphenoid sinus bone. This study was designed to evaluate the usefulness of this technique based on the authors' experience during the past 7.6 years. Methods. A silicone plate was used to reconstruct the sellar floor in 69 consecutive patients with sellar tumors that included 60 pituitary adenomas and nine Rathke's cleft cysts. The patients ranged in age from 16 to 82 years (mean 52 years). The postoperative position of the silicone plate could be clearly identified on sagittal or coronal magnetic resonance (MR) imaging as a very low intensity plate (void signal). No displacement or migration of the implanted silicone plate was observed on follow-up MR imaging in any patient. Infections of the lesion such as a pituitary abscess were not observed clinically or radiologically in any patient. Of the 16 patients with intraoperative cerebrospinal fluid (CSF) leakage, only one patient who had a ghost sella developed postoperative CSF rhinorrhea. In all seven patients who underwent repeated surgery for residual or recurrent tumor, the silicone plate that had been placed at the initial procedure was covered with a relatively thin fibrous capsule and the plate was well preserved. The silicone plate was easily removed at reoperation and was useful for detection of the sellar floor window made previously. Conclusions. These results indicate that a silicone plate can be useful for reconstruction of the sellar floor in rhinoseptoplastic transsphenoidal surgery.


2003 ◽  
Vol 98 (6) ◽  
pp. 1312-1317 ◽  
Author(s):  
Louis J. Kim ◽  
Jeffrey D. Klopfenstein ◽  
Ming Cheng ◽  
Murugasu Nagul ◽  
Stephen Coons ◽  
...  

✓ Despite diagnostic advances, it remains difficult to identify intrasellar and ectopic parasellar adrenocorticotropic hormone (ACTH)—secreting microadenomas. The authors present the case of a 61-year-old woman with Cushing disease in whom a significant central-to-peripheral and lateralized right-sided ACTH gradient was demonstrated on inferior petrosal sinus sampling; no discernible abnormality was seen on magnetic resonance imaging. She underwent transnasal transsphenoidal surgery. No tumor was found on sellar exploration and a total hypophysectomy was performed, yet her hypercortisolemia persisted. The patient died of cardiac events 17 days postsurgery. Autopsy revealed an isolated, right-sided, intracavernous ACTH-secreting adenoma with no intrasellar communication. This case represents the first failed transsphenoidal surgery for Cushing disease in which there is postmortem confirmation of a suspected intracavernous sinus lesion. It supports the hypothesis that Cushing disease associated with nondiagnostic imaging studies, a strong ACTH gradient on venography, and negative findings on sellar exploration may be caused by an ectopic intracavernous ACTH-secreting adenoma. There are no premortem means of confirming the presence of such lesions, but these tumors could underlie similar cases of failed surgery. Radiation therapy targeting the sella turcica and both cavernous sinuses, possibly supplemented with medical treatment, is suggested for similar patients in whom transsphenoidal hypophysectomy has failed. Adrenalectomy may also be appropriate if a rapid reduction in ACTH is necessary.


2005 ◽  
Vol 102 (6) ◽  
pp. 1004-1012 ◽  
Author(s):  
Ronald J. Benveniste ◽  
Wesley A. King ◽  
Jane Walsh ◽  
Jacob S. Lee ◽  
Bradley N. Delman ◽  
...  

Object. In this paper the authors describe the indications for and the results and complications of repeated transsphenoidal surgery (RTSS) to treat recurrent or residual pituitary adenoma. Methods. A retrospective review was conducted of 96 consecutive patients who underwent RTSS to treat recurrent or residual pituitary adenoma. Ninety-six patients underwent RTSS: 42 to treat a recurrent or residual pituitary mass and 54 to treat a recurrent or persistent hormone hypersecretion. There was no case of perioperative death and there was a 1% incidence of major complications. Postoperative endocrinological deficiencies were uncommon unless planned total hypophysectomy was performed to treat Cushing disease. Clinical remission occurred in 93% of patients undergoing RTSS to treat a tumor mass, and 15% of patients initially experienced remission only to face a relapse after a mean of 32 months. Endocrinological remission occurred in 57% of patients undergoing RTSS to treat hormone hypersecretion; most of these patients had Cushing disease. Thirty-five percent of patients with an initial endocrinological remission experienced a relapse of their symptoms after a mean of 31 months (thus, 37% of patients achieved sustained endocrinological remission). We failed to identify factors that accurately predicted initial symptom remission or delayed relapse following RTSS. Ten patients in our series eventually underwent a third transsphenoidal surgery without major complications. Conclusions. Repeated transsphenoidal surgery is a more effective treatment for recurrent or residual mass than it is for hormone hypersecretion and has acceptable rates of morbidity and mortality. If hypophysectomy is not performed, endocrinological deficiencies are unlikely following RTSS.


2001 ◽  
Vol 95 (2) ◽  
pp. 285-291 ◽  
Author(s):  
Hidetoshi Ikeda ◽  
Hidefumi Jokura ◽  
Takashi Yoshimoto

Object. The results of combined transsphenoidal surgery and adjuvant gamma knife surgery (GKS) for growth hormone (GH)—secreting adenoma were investigated using biochemical cure criteria for surgery and biological cure criteria for adjuvant GKS. Methods. Ninety patients (42 male and 48 female patients), ranging from 11 to 75 years of age, underwent transsphenoidal surgery for GH-secreting pituitary adenoma. Preoperative and postoperative GH and insulin-like growth factor-I levels were measured, as was the postoperative GH level after the oral glucose tolerance test. Tumor size, cavernous sinus (CS) invasion, and residual tumor were evaluated using magnetic resonance (MR) imaging. Transsphenoidal microsurgery was performed, followed by adjuvant GKS when there was persistent biochemical evidence of GH hypersecretion with residual tumor detectable in the CS on MR imaging. Patients in whom GKS was contraindicated were treated with conventional radiotherapy or by medical means. Conclusions. The overall surgical cure rate was 57% based on recently accepted biochemical cure criteria. Patients with no CS invasion achieved a 100% cure rate, whereas patients with CS invasion achieved an 82% cure rate (14 of 17 patients) after adjuvant GKS. The combination of transsphenoidal microsurgery and adjuvant GKS is the optimal therapy for patients with GH-secreting adenoma.


2001 ◽  
Vol 95 (1) ◽  
pp. 1-8 ◽  
Author(s):  
Nathan E. Simmons ◽  
Tord D. Alden ◽  
Michael O. Thorner ◽  
Edward R. Laws

Object. Transsphenoidal surgery remains the optimal treatment for Cushing disease, but the definitions of surgical cure and failure remain debatable. In this study the authors evaluated serum cortisol levels in patients before and after they underwent transsphenoidal surgery to elucidate the patterns of cortisol decrease and the optimal time and criteria for determining surgically induced remission. Methods. Twenty-seven patients were evaluated throughout an 8-month period. Serum cortisol levels were obtained before surgery and at 6-hour intervals postoperatively. No exogenous steroid medications were administered until after cortisol sampling was discontinued, following diagnosis of remission. Twenty-one (78%) of 27 cases were labeled initial surgically induced remissions. Twenty-two (81%) of 27 cases were deemed surgically induced remissions at follow-up examination. Following surgery, initial remissions and failures demonstrated divergent patterns of cortisol levels. No patient whose condition was deemed an initial surgically induced remission has experienced definitive relapse of disease since discharge. One patient whose condition was initially deemed a surgical failure, eventually was found to exhibit surgically induced remission without further intervention. Conclusions. Given such findings, exogenous steroid medications do not appear to be required for patients until after the determination of remission. During the 1st postoperative day, there is a time period during which serum cortisol values significantly differ between the categories of surgically induced remissions and surgical failures. Surgically induced remissions were identified when postoperative values of cortisol were lower than preoperative midnight levels and when absolute values of cortisol were less than 10 µg/dl. In a small proportion of patients remission on a delayed basis may also be demonstrated. These data allow for a simple and rapid determination of postoperative remission in patients undergoing transsphenoidal surgery for Cushing disease.


2000 ◽  
Vol 93 (supplement_3) ◽  
pp. 68-73 ◽  
Author(s):  
Pierre-Hugues Roche ◽  
Jean Régis ◽  
Henry Dufour ◽  
Henri-Dominique Fournier ◽  
Christine Delsanti ◽  
...  

Object. The authors sought to assess the functional tolerance and tumor control rate of cavernous sinus meningiomas treated by gamma knife radiosurgery (GKS). Methods. Between July 1992 and October 1998, 92 patients harboring benign cavernous sinus meningiomas underwent GKS. The present study is concerned with the first 80 consecutive patients (63 women and 17 men). Gamma knife radiosurgery was performed as an alternative to surgical removal in 50 cases and as an adjuvant to microsurgery in 30 cases. The mean patient age was 49 years (range 6–71 years). The mean tumor volume was 5.8 cm3 (range 0.9–18.6 cm3). On magnetic resonance (MR) imaging the tumor was confined in 66 cases and extensive in 14 cases. The mean prescription dose was 28 Gy (range 12–50 Gy), delivered with an average of eight isocenters (range two–18). The median peripheral isodose was 50% (range 30–70%). Patients were evaluated at 6 months, and at 1, 2, 3, 5, and 7 years after GKS. The median follow-up period was 30.5 months (range 12–79 months). Tumor stabilization after GKS was noted in 51 patients, tumor shrinkage in 25 patients, and enlargement in four patients requiring surgical removal in two cases. The 5-year actuarial progression-free survival was 92.8%. No new oculomotor deficit was observed. Among the 54 patients with oculomotor nerve deficits, 15 improved, eight recovered, and one worsened. Among the 13 patients with trigeminal neuralgia, one worsened (contemporary of tumor growing), five remained unchanged, four improved, and three recovered. In a patient with a remnant surrounding the optic nerve and preoperative low vision (3/10) the decision was to treat the lesion and deliberately sacrifice the residual visual acuity. Only one transient unexpected optic neuropathy has been observed. One case of delayed intracavernous carotid artery occlusion occurred 3 months after GKS, without permanent deficit. Another patient presented with partial complex seizures 18 months after GKS. All cases of tumor growth and neurological deficits observed after GKS occurred before the use of GammaPlan. Since the initiation of systematic use of stereotactic MR imaging and computer-assisted modern dose planning, no more side effects or cases of tumor growth have occurred. Conclusions. Gamma knife radiosurgery was found to be an effective low morbidity—related tool for the treatment of cavernous sinus meningioma. In a significant number of patients, oculomotor functional restoration was observed. The treatment appears to be an alternative to surgical removal of confined enclosed cavernous sinus meningioma and should be proposed as an adjuvant to surgery in case of extensive meningiomas.


2002 ◽  
Vol 97 ◽  
pp. 600-606 ◽  
Author(s):  
Chihiro Ohye ◽  
Tohru Shibazaki ◽  
Jie Zhang ◽  
Yoshitaka Andou

Object. The treatment of Parkinson disease and other kinds of involuntary movement by gamma knife radiosurgery (GKS) is presented. This is an extension of previous work. The clinical course and thalamic lesions were the main factors examined. Methods. Seventeen new cases were added to the previously reported 36 cases. The course and results for the whole series of 53 patients were examined. Treatment was undertaken using a single 4-mm collimator shot to deliver 130 Gy to the target. The target was determined in the previously treated patients by using classic methods involved in conventional stereotactic thalamotomy with microrecording. More recently, target localization has been performed by relating the target point to the total length of the thalamus. Points may then be defined as percentages of that length measured from the anterior pole. Targets can then be determined in relationship to the appropriate percentage. Thirty-five patients have been followed for more than 2 years and the longest follow up was 8 years. Two kinds of thalamic lesion were seen after GKS. Volumetric analysis on MR imaging revealed that the larger lesion was 400 to 500 mm3 at the beginning and gradually decreased in size. The smaller lesion occupied approximately 200 mm3 and also shrank over several months. Eighty percent of the treated cases showed good results and no significant complications, with the tremor subsiding at 1 year (Type 1). Several cases deviated from this standard course in four different ways (Types 2–5). If tremor persisted, conventional stereotactic thalamotomy with microrecording was performed. During such operations, normal neuronal activity was recorded from the region adjacent to the GKS thalamotomy target. This was the region showing a high signal on MR imaging. The activity patterns included the rhythmical grouped discharge of tremor rhythm. Conclusions. Gamma thalamotomy for functional disorders is still under development, but because the results with careful target planning are satisfactory, there are grounds for increasing optimism.


2005 ◽  
Vol 102 (Special_Supplement) ◽  
pp. 87-97 ◽  
Author(s):  
Wen-Yuh Chung ◽  
Kang-Du Liu ◽  
Cheng-Ying Shiau ◽  
Hsiu-Mei Wu ◽  
Ling-Wei Wang ◽  
...  

Object. The authors conducted a study to determine the optimal radiation dose for vestibular schwannoma (VS) and to examine the histopathology in cases of treatment failure for better understanding of the effects of irradiation. Methods. A retrospective study was performed of 195 patients with VS; there were 113 female and 82 male patients whose mean age was 51 years (range 11–82 years). Seventy-two patients (37%) had undergone partial or total excision of their tumor prior to gamma knife surgery (GKS). The mean tumor volume was 4.1 cm3 (range 0.04–23.1 cm3). Multiisocenter dose planning placed a prescription dose of 11 to 18.2 Gy on the 50 to 94% isodose located at the tumor margin. Clinical and magnetic resonance (MR) imaging follow-up evaluations were performed every 6 months. A loss of central enhancement was demonstrated on MR imaging in 69.5% of the patients. At the latest MR imaging assessment decreased or stable tumor volume was demonstrated in 93.6% of the patients. During a median follow-up period of 31 months resection was avoided in 96.8% of cases. Uncontrolled tumor swelling was noted in five patients at 3.5, 17, 24, 33, and 62 months after GKS, respectively. Twelve of 20 patients retained serviceable hearing. Two patients experienced a temporary facial palsy. Two patients developed a new trigeminal neuralgia. There was no treatment-related death. Histopathological examination of specimens in three cases (one at 62 months after GKS) revealed a long-lasting radiation effect on vessels inside the tumor. Conclusions. Radiosurgery had a long-term radiation effect on VSs for up to 5 years. A margin 12-Gy dose with homogeneous distribution is effective in preventing tumor progression, while posing no serious threat to normal cranial nerve function.


1996 ◽  
Vol 84 (6) ◽  
pp. 962-971 ◽  
Author(s):  
Tohru Mizutani

✓ A long-term follow-up study (minimum duration 2 years) was made of 13 patients with tortuous dilated basilar arteries. Of these, five patients had symptoms related to the presence of such arteries. Symptoms present at a very early stage included vertebrobasilar insufficiency in two patients, brainstem infarction in two patients, and left hemifacial spasm in one patient. Initial magnetic resonance (MR) imaging in serial slices of basilar arteries obtained from the five symptomatic patients showed an intimal flap or a subadventitial hematoma, both of which are characteristic of a dissecting aneurysm. In contrast, the basilar arteries in the eight asymptomatic patients did not show particular findings and they remained clinically and radiologically silent during the follow-up period. All of the lesions in the five symptomatic patients gradually grew to fantastic sizes, with progressive deterioration of the related clinical symptoms. Dilation of the basilar artery was consistent with hemorrhage into the “pseudolumen” within the laminated thrombus, which was confirmed by MR imaging studies. Of the five symptomatic patients studied, two died of fatal subarachnoid hemorrhage (SAH) and two of brainstem compression; the fifth patient remains alive without neurological deficits. In the three patients who underwent autopsy, a definite macroscopic double lumen was observed in both the proximal and distal ends of the aneurysms within the layer of the thickening intima. Microscopically, multiple mural dissections, fragmentation of internal elastic lamina (IEL), and degeneration of media were diffusely observed in the remarkably extended wall of the aneurysms. The substantial mechanism of pathogenesis and enlargement in the symptomatic, highly tortuous dilated artery might initially be macroscopic dissection within a thickening intima and subsequent repetitive hemorrhaging within a laminated thrombus in the pseudolumen combined with microscopic multiple mural dissections on the basis of a weakened IEL. The authors note and caution that symptomatic, tortuous dilated basilar arteries cannot be overlooked because they include a group of malignant arteries that may grow rapidly, resulting in a fatal course.


1999 ◽  
Vol 90 (2) ◽  
pp. 300-305 ◽  
Author(s):  
Leif Østergaard ◽  
Fred H. Hochberg ◽  
James D. Rabinov ◽  
A. Gregory Sorensen ◽  
Michael Lev ◽  
...  

Object. In this study the authors assessed the early changes in brain tumor physiology associated with glucocorticoid administration. Glucocorticoids have a dramatic effect on symptoms in patients with brain tumors over a time scale ranging from minutes to a few hours. Previous studies have indicated that glucocorticoids may act either by decreasing cerebral blood volume (CBV) or blood-tumor barrier (BTB) permeability and thereby the degree of vasogenic edema.Methods. Using magnetic resonance (MR) imaging, the authors examined the acute changes in CBV, cerebral blood flow (CBF), and BTB permeability to gadolinium-diethylenetriamine pentaacetic acid after administration of dexamethasone in six patients with brain tumors. In patients with acute decreases in BTB permeability after dexamethasone administration, changes in the degree of edema were assessed using the apparent diffusion coefficient of water.Conclusions. Dexamethasone was found to cause a dramatic decrease in BTB permeability and regional CBV but no significant changes in CBF or the degree of edema. The authors found that MR imaging provides a powerful tool for investigating the pathophysiological changes associated with the clinical effects of glucocorticoids.


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