Marching beyond the sella: Gerard Guiot and his contributions to neurosurgery.

Gerard Guiot (1912-1998) was one of the most renowned and innovative neurosurgeons of the 20th century. His pivotal and revolutionary role in advancing transsphenoidal surgery has been recorded in many historical vignettes, yet his outstanding contributions to the advancement of neurosurgery outside the confines of the sella have not been described in a detailed fashion. In this article, the authors discuss the life and achievements of Professor Guiot and present a comprehensive description of his contributions to the field of neurosurgery, including cerebrovascular, spine, craniofacial, stereotactic functional, and endoscopic surgery.

Long-term biochemical status and disease-related morbidity in 53 postoperative patients with acromegaly.

UNLABELLED: Assessment of postoperative disease activity of acromegaly is a major challenge. The consensus criteria for cure, which are glucose-suppressed GH less than 1 micro g/liter and normal IGF-I levels, might be discrepant, and their respective relationship to acromegaly-related morbidity is not well known. THE AIMS OF OUR STUDY WERE: firstly, to correlate plasma IGF-I with plasma glucose-suppressed GH concentrations; and secondly, to correlate each of these biochemical parameters with morbidity [impaired glucose tolerance (IGT), diabetes, and hypertension] in postoperative patients with acromegaly. Fifty-three patients with long-term follow-up (mean, 12.7 yr; range, 1-30 yr) after transsphenoidal surgery for acromegaly and 20 healthy subjects matched for age, sex, and body mass index were evaluated for plasma glucose [by 75-g oral glucose tolerance test (OGTT)], GH (by immunoradiometric assay), plasma IGF-I (by immunoradiometric assay), and blood pressure (BP) measurements. Remission was defined by a normal IGF-I. We identified 34 acromegalics in remission and 19 with active disease. There was no statistical difference between all three groups for age, sex, BMI, and for fasting and 2-h post-OGTT plasma glucose. The time elapsed since surgery was similar in both groups of acromegalics. The OGTT-GH nadir was less than 1 micro g/liter in 31 patients in remission (91.2%) and in nine patients with active disease (47.4%). Prevalence of IGT was lower in acromegalics in remission (14.7%) in comparison with patients with active disease (47.4%; P = 0.01). Plasma IGF-I and GH nadir cut-off of 0.25 microg/liter were strong predictors of abnormal glucose tolerance (odds ratio, 13.6; confidence interval, 2.5-73.7; P = 0.003). GH nadir cut-off of 1 microg/liter and basal GH of 2.5 microg/liter failed to predict abnormal glucose tolerance. There was no statistical difference for prevalence of hypertension and systolic BP values, but diastolic BP was significantly lower in patients in remission than in those with active disease (P < 0.05). Our observations indicate that the validity of the GH threshold of 1 microg/liter post OGTT might be inadequate as a criterion of biochemical remission of acromegaly and as a marker of associated comorbidities. However, normalized IGF-I concentrations and a lower GH cut-off value less than 0.25 micro g/liter are strongly associated with a lower prevalence of IGT and lower diastolic BP in long-term postoperative acromegaly.

Granular cell tumor of the sellar and suprasellar region: clinicopathologic study of 11 cases and literature review.

OBJECTIVE: To report a series of cases of surgically resected granular cell tumors in an attempt to better characterize their clinical presentation, imaging features, and treatment outcomes with attention to previously published literature. PATIENTS AND METHODS: This is a retrospective study of 11 patients with granular cell tumors. Information obtained from patients' medical records was supplemented by direct physician and patient telephone contact. RESULTS: The study group consisted of 9 women and 2 men, with a mean +/- SD age of 50 +/- 15 years (range, 26-73 years). Seven patients were symptomatic, 3 of whom presented with visual complaints. Four patients presented primarily with endocrine dysfunction (2) or headaches (2). The duration of symptoms varied from 1 to 12 months (mean +/- SD, 6.0 +/- 4.5 months). Formal visual field testing revealed bitemporal hemianopsia in 6 and a right-sided visual field deficit in 2. The tumor appeared as a well-defined, relatively homogeneously (6) or heterogeneously (1) enhancing suprasellar mass on contrast infusion. Tumor size varied from 1.5 to 6.0 cm (mean +/- SD, 3.1 +/- 1.6 cm). At surgery, the mass was noted to be firm and vascular, and in at least 3 patients these features prevented gross total resection. Only 1 patient who had undergone biopsy of his lesion received adjuvant radiation therapy. Follow-up was obtained in all but 1 patient. Nine of these 10 patients are alive and are either free of disease or have had no disease progression. Follow-up in these 9 patients varied from 1 to 16 years (mean +/- SD, 6.5 +/- 53 years). CONCLUSIONS: A granular cell tumor is generally a surprise finding in that it is seldom considered in the differential diagnosis of contrast-enhancing, demarcated sellar region masses. Granular cell tumors are benign and slow growing with no pronounced tendency for invasion or recurrence. Because of the indolent growth of granular cell tumors, the surgical goal of tumor decompression is a reasonable therapeutic approach.

Pre- and postoperative magnetic resonance imaging appearance of the normal residual pituitary gland following macroadenoma resection: Clinical implications.

BACKGROUND: To assess the relationship between the preoperative magnetic resonance imaging (MRI) appearance of the normal residual pituitary gland (NRPG) and pituitary functional outcome following transsphenoidal resection of pituitary macroadenomas. METHODS: We retrospectively reviewed the medical records of 100 consecutive patients with a pituitary macroadenoma, who underwent transsphenoidal resection. The preoperative configuration of the displaced NRPG was stratified as superior, superolateral or lateral. The extent of postoperative restitution of the NRPG was divided into four groups: Group 1 - normal residual gland or almost normal; Group 2 - more than 50% restitution; Group 3 - less than 50% of the normal residual gland; and Group 4 - barely visible or absent residual gland. The pre- and postoperative NRPG appearance was correlated with pituitary functional status. RESULTS: Preoperatively, the NRPG was identifiable in 79 patients, with extrasellar displacement in 53%. The displacement pattern was superior in 8%, superolateral in 32%, and lateral in 58% of the patients. If the NRPG was displaced laterally, the ipsilateral cavernous sinus was not invaded by the pituitary macroadenoma. Partial or complete pituitary function was lost in 6 / 23 (26.1%) patients with superior or superolateral displacement of the NRPG, compared to only 1 / 36 (2.8%) patients without superior displacement of the NRPG (P = 0.025). Progressive postoperative reconstitution of the NRPG was related to the preservation of the pituitary hormonal axis (Pearson Chi-Square P < 0.001). CONCLUSIONS: Progressive displacement of the NRPG preoperatively, and lack of restitution of the NRPG on postoperative MRI appeared to correlate with the postoperative pituitary functional loss.

Long-term outcome and mortality after transsphenoidal adenomectomy for acromegaly.

OBJECTIVE: Acromegaly has long been associated with increased mortality but few long-term follow-up data are available in patients treated for this disease. We therefore studied a group of 103 patients who underwent transsphenoidal adenomectomy for acromegaly between 1970 and 1999 and were followed for one to 30 years. DESIGN AND PATIENTS: A retrospective chart review was performed on 103 patients living in the province of Quebec, Canada. Mortality data were obtained by hospital charts, contact with the patient's family or death certificates. Stringent biochemical criteria were used to define remission (random GH < 2.5 microg/l, or GH nadir after an oral glucose load is < 1 microg/l and IGF-I within the normal range) and patient survival in the group in remission and the group with persistent disease were compared to survival of the population of Quebec, Canada, using the probabilities of the Poisson distribution. RESULTS: There were four deaths in the perioperative period, one of which was directly related to surgery. Initial remission was obtained in 82% of microadenomas, 60% of macroadenomas and 24% of invasive adenomas. The long-term (> or = 10 years) remission rate for surgery alone was 52%. A second transsphenoidal surgery, radiation therapy and/or octreotide were used in a subset of patients with persistent disease. Long-term remission was obtained in 63% of patients. Five (mean age, 64 years) of the 57 patients in remission died; this rate did not differ significantly from the mortality rate expected in the general population (P = 0.18). Thirteen (mean age, 59.8 years) of the 34 patients with persistent disease died; this rate was significantly higher than that expected in the general population (P = 0.008). CONCLUSIONS: Our observations confirm that uncontrolled acromegaly increases mortality compared to the general population and that mortality rates similar to the general population are restored once remission is induced.