Sublabial transseptal vs transnasal combined endoscopic microsurgery in patients with Cushing disease and MRI-depicted microadenomas.

OBJECTIVE: To assess whether the type of surgical approach to the sella (sublabial transseptal vs transnasal) affects surgical outcome among patients with Cushing disease. PARTICIPANTS AND METHODS: Both procedures were performed at our institution from January 1, 1995, through January 31, 2003. From a total of 106 patients with Cushing disease who had had surgery, we identified 42 adults undergoing an initial surgery, with microadenoma (<1 cm) determined by magnetic resonance imaging and a minimal follow-up of 3 months. RESULTS: We identified 21 patients (4 male, 17 female) for sublabial transseptal transsphenoidal microsurgery. Mean+/-SD age was 45.0+/-12.9 years (range, 27.0-69.0 years); median duration of symptoms was 2.5 years (range, 1.5-7.5 years). Median follow-up was 1.0 year (range, 0.3-11.0 years). We identified another 21 patients (5 male, 16 female) for endoscopic transsphenoidal microsurgery whose mean+/-SD age was 43.8+/-14.1 years (range, 19.0-70.0 years); median duration of symptoms was 2.4 years (range, 0.2-6.0 years). Median follow-up was 2.5 years (range, 0.3-8.7 years). Complications (cerebrospinal fluid leak and transient diabetes insipidus) and cure (86% initial cure; 76% relapse-included cure) were equivalent between the 2 procedures. However, the endoscopic approach was associated with shorter anesthesia time, less blood loss, and shorter hospital stays. CONCLUSION: The endoscopic transnasal surgical approach led to shorter total anesthesia time, less blood loss, and shorter hospital stay when compared with the sublabial transseptal approach. However, we found no difference between the 2 surgical procedures with respect to cure or complications, suggesting that outcome is more closely related to the experience of the operating team.

Surgery for Cushing's syndrome: an historical review and recent ten-year experience.

BACKGROUND: Cushing's syndrome (CS), due to multiple etiologies, is a disorder associated with the ravages of cortisol excess. The purpose of this review article is to provide a historical synopsis of surgery for CS, review a recent 10-year period of operative management at a tertiary care facility, and to outline a practical approach to diagnosis and management. MATERIALS AND METHODS: From 1996 to 2005, 298 patients underwent 322 operative procedures for CS at Mayo Clinic, Rochester, Minnesota. A retrospective chart review was carried out. Data was gathered regarding demographics, preoperative assessment, procedures performed, and outcomes. Data are presented as counts and percentages. Five-year survival rates were calculated where applicable by the Kaplan-Meier method. Statistical analysis was carried out with SAS, version 9 (SAS Institute, Inc., Cary, NC). RESULTS: Two-hundred thirty-one patients (78%) had ACTH-dependent CS and 67 patients (22%) had ACTH-independent CS. One-hundred ninety-six patients (66%) had pituitary-dependent CS and 35 patients (12%) had ectopic ACTH syndrome. Fifty-four patients (18%) had cortisol-secreting adenomas, 10 patients (3%) had cortisol-producing adrenocortical carcinomas, and 1% had other causes. Cure rates for first time pituitary operations (transsphenoidal, sublabial, and endonasal) were 80% and 55% for reoperations. Most benign adrenal processes could be managed laparoscopically. Five-year survival rates (all causes) were 90%, 51%, and 23% for adrenocortical adenomas, ectopic ACTH syndrome, and adrenocortical carcinomas, respectively. CONCLUSIONS: Surgery for CS is highly successful for pituitary-dependent CS and most ACTH-independent adrenal causes. Bilateral total adrenalectomy can also provide effective palliation from the ravages of hypercortisolism in patients with ectopic ACTH syndrome and for those who have failed transsphenoidal surgery. Unfortunately, to date, adrenocortical carcinomas are rarely cured. Future successes with this disease will likely depend on a better understanding of tumor biology, more effective adjuvant therapies and earlier detection. Clearly, IPSS, advances in cross-sectional imaging, along with developments in transsphenoidal and laparoscopic surgery, have had the greatest impact on today's management of the complex patient with CS.

The clinical conundrum of corticotropin-independent autonomous cortisol secretion in patients with bilateral adrenal masses.

BACKGROUND: Management of patients with bilateral adrenal masses and corticotropin (ACTH)-independent Cushing syndrome (CS) or subclinical CS is problematic. We report our experience with adrenal venous sampling (AVS) in the evaluation of 10 patients with bilateral masses who had ACTH-independent CS or subclinical CS. PATIENTS AND METHODS: Ten patients (9 women, 1 man, mean age 56.4 years) with bilateral adrenal masses and ACTH-independent CS (n=3) or subclinical CS (n=7) underwent AVS. Autonomous cortisol secretion was documented in all cases with suppressed serum ACTH concentrations and lack of cortisol suppression with dexamethasone administration. Adrenal venous sampling was performed on the second day of dexamethasone administration. Cortisol and epinephrine levels were measured from each adrenal vein (AV) and from a peripheral vein (PV). RESULTS: Mean (+/-SD) maximal diameter of the adrenal masses on computed tomography was 3.3+/-1.3 cm (range: 1.2-6.0 cm). Successful catheterization was confirmed with AV:PV epinephrine gradients. A cortisol AV:PV gradient>6.5 was consistent with a cortisol-secreting adenoma in 11 adrenal glands; 5 patients had clinically important bilateral autonomous cortisol hypersecretion, 3 had bilateral cortisol-secreting adenomas, and 2 had ACTH-independent macronodular adrenal hyperplasia. Adrenal venous sampling-guided adrenalectomy was completed in all 10 patients-2 patients had total bilateral adrenalectomy and 2 others had subtotal bilateral adrenalectomy. During a mean follow-up of 36.1 months (range: 0.7-123 months), CS or clinically important cortisol secretory autonomy did not recur. CONCLUSIONS: Adrenal venous sampling contributed to the localization of autonomous hypercortisolism in the setting of ACTH-independent CS or subclinical CS in patients with bilateral adrenal masses.

Dramatic volume reduction of a large GH/TSH secreting pituitary tumor with short term Octreotide therapy.

A case is presented of a huge GH/TSH secreting tumor and marked volumetric reduction in size with only one week of Octreotide therapy. To our knowledge, this is the first reported case of such a dramatic volumetric response to short-term Octreotide therapy.

Rathke cleft cyst: diagnostic and therapeutic considerations.

OBJECTIVE: To highlight diagnostic and therapeutic issues about Rathke cleft cysts for otorhinolaryngologists. STUDY DESIGN: Retrospective. METHODS: We retrospectively reviewed data collected on Rathke cleft cysts between 1978 and 1998: presenting symptoms, visual acuity, surgical treatment, complications, recurrences, and effect on daily activity. RESULTS: Twenty-nine patients were diagnosed with a Rathke cleft cyst (11 male and 18 female patients; mean age, 46 y). The most common presenting symptom was head pain (55%). The majority (59%) of cases demonstrated suprasellar extension on preoperative imaging, with pituitary dysfunction identified in 66%. Recurrence occurred in eight patients (28%). Postoperative visual function improved or remained stable in all patients. Persistent pituitary dysfunction required hormonal supplementation in seven patients (24%). Only one patient with an astrocytoma in addition to a Rathke cleft cyst did not maintain the ability to perform normally on an assessment of activities of daily living, a striking contrast to patients with craniopharyngioma. CONCLUSIONS: Conclusions were as follows: 1) Rathke cleft cysts must be considered as sources of head pain and pituitary dysfunction. 2) Persistent or recurrent cyst formation occurs in approximately one-third of the patients. Recurrence may take many years, and follow-up imaging is recommended for at least a decade. 3) Maintenance of the ability to perform the activities of normal daily living can be expected after surgical management. 4) Most Rathke cleft cysts can be managed through transnasal exposure of the sella. 5) Packing the sella may result in predisposition to recurrent cyst formation.

Results of stereotactic radiosurgery in patients with hormone-producing pituitary adenomas: factors associated with endocrine normalization.

OBJECT: The goal of this study was to determine factors associated with endocrine normalization after radiosurgery is performed in patients with hormone-producing pituitary adenomas. METHODS: Between 1990 and 1999, 43 patients with hormone-producing pituitary adenomas underwent radiosurgery: 26 patients with growth hormone (GH)-producing tumors, nine with adrenocorticotrophic hormone-producing tumors, seven with tumors that produced prolactin (PRL) alone, and one with a tumor that secreted both GH and PRL. The median patient age was 42 years. Thirty-seven patients (86%) had undergone surgery earlier and in 30 (70%) there was tumor extension into the cavernous sinus. The product-limit method was used to calculate endocrine normalization while patients were not receiving any hormone-suppressive medication. The median follow-up period after radiosurgery was 36 months (range 12-108 months). In 20 patients (47%) there was normalization of hormone secretion at a median of 14 months (range 2-44 months) after radiosurgery; no correlation was found between tumor type and cure. Actuarial cure rates were 20, 32, and 61% at 1, 2, and 4 years posttreatment. Multivariate analysis demonstrated that the absence of hormone-suppressive medications at the time of radiosurgery (relative risk 8.9, 95% confidence interval [CI] 1.2-68.7, p = 0.04) and maximum radiation doses greater than 40 Gy (relative risk 3.9, 95% CI 1.3-11.7, p = 0.02) correlated with an endocrine cure. A new anterior pituitary deficiency developed in seven patients (16%), temporal lobe necrosis was identified in two patients, an asymptomatic internal carotid artery stenosis was detected in two patients, and unilateral blindness occurred in one patient. CONCLUSIONS: Radiosurgery provides an endocrine cure for many patients with persistent or recurrent hormone-producing pituitary adenomas. Further study is needed to determine whether pituitary hormone-suppressive medications have a radioprotective effect.