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Joao Paulo Almeida and Fred Gentili

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Amir R. Dehdashti and Fred Gentili

Object

Transsphenoidal pituitary surgery is the primary therapy for Cushing disease because of its potential to produce lasting remission without the need for long-term drug or hormone replacement therapy. The authors evaluated the current role of pure endoscopic endonasal pituitary surgery in the treatment of Cushing disease.

Methods

Twenty-five patients underwent pure endoscopic surgery for confirmed Cushing disease. Thirteen patients had microadenomas and seven had macroadenomas; magnetic resonance images obtained in five patients were only suspicious or nondiagnostic, and thus they underwent inferior petrosal sinus sampling. Two patients had evidence of cavernous sinus involvement. Final histological results were consistent with adrenocorticotropin hormone (ACTH)–secreting adenoma in 20 patients.

Results

Twenty patients (80%) had clinical remission and laboratory confirmation of hypocortisolemia (serum cortisol < 100 nmol/L requiring substitution therapy), suppression to low-dose dexamethasone, and normal 24-hour urinary free cortisol. The median follow-up period was 17 months (range 3–32 months). There was no recurrence at the time of the last follow-up. Three patients presented with new anterior pituitary deficiency, but no one had permanent diabetes insipidus. In one patient a cerebrospinal fluid leak developed but later resolved following lumbar drainage. Treatment failure was attributable to involvement of the cavernous sinus in two patients, incomplete tumor removal in one, negative exploration in one, and nodular corticotroph hyperplasia of the pituitary gland in one.

Conclusions

Early results indicated that endoscopic endonasal surgery is a safe and effective treatment for ACTH-producing adenomas. The rate of remission in this study is comparable to those in previous series, and the rate of major postoperative complications is extremely low. Further studies with a larger number of patients and longer follow-ups are required to determine whether this more minimally invasive pure endoscopic approach should become the standard of care for the surgical treatment of Cushing disease.

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Giorgio Carrabba, Amir R. Dehdashti and Fred Gentili

Object

Clival lesions pose significant challenges with regard to their surgical management. The expanded endoscopic endonasal (EEE) approach is a promising minimally invasive technique for lesions of the central skull base. The authors' aim in the current paper was to discuss the surgical treatment of clival lesions and to present the technical details, indications, and limitations of the EEE approach. Data from a recent endoscopically treated group will be compared with findings in a previous cohort of patients treated via classic open anterior and lateral approaches.

Methods

Since June 2005, 17 patients with clival lesions underwent surgery via the EEE approach. Suitable candidates were chosen according to lesion characteristics, clinical parameters, and surgical goals. Neurological outcomes, Karnofsky Performance Scale scores, the extent of lesion resection, and complications were evaluated among these patients. Eighteen percent of the patients in the endoscopic group presented with recurrent disease. Another series of 43 patients, who had undergone resection of clival lesions via an anterior (rhinotomy, maxillectomy, microscopic transsphenoidal surgery, or transoral surgery) or lateral (pterional, frontoorbitozygomatic, or combined suprainfratentorial retrosigmoid) approach, was similarly reviewed. Twenty-three of these patients (53%) presented with recurrent disease and thus had undergone prior surgery.

Results

Following the EEE approach, 11 (79%) of 14 patients who had presented with neurological symptoms experienced improvement, and gross-total resection was achieved in 59% of the patients and subtotal removal in 41%. Complications included CSF leakage (24%), tension pneumocephalus (6%), and intracranial hematoma (6%). The patient with the latter complication was the only one who experienced permanent neurological worsening. In the open resection group, neurological worsening occurred in 33% of the patients (14 of 43). Total and grosstotal removals were achieved in 84% of patients and subtotal removal in 14%.

Conclusions

The EEE approach has been shown to be a safe and effective technique for the resection of clival lesions with limited lateral extension. The choice of surgical approach must be tailored according to both patient and tumor characteristics. Although the 2 patient series featured in this paper are not comparable—because of a selection bias—higher rates of neurological morbidity and total and gross-total resections were observed in the open resection group. Given the long survival of some patients, the EEE approach should be favored whenever reasonable.

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Mazda K. Turel, Georgios Tsermoulas, Lior Gonen, George Klironomos, Joao Paulo Almeida, Gelareh Zadeh and Fred Gentili

OBJECTIVE

The treatment of recurrent and residual craniopharyngiomas is challenging. In this study the authors describe their experience with these tumors and make recommendations on their management.

METHODS

The authors performed an observational study of adult patients (≥ 18 years) with recurrent or residual craniopharyngiomas that were managed at their tertiary center. Retrospective data were collected on demographics and clinical, imaging, and treatment characteristics from patients who had a minimum 2-year follow-up. Descriptive statistics were used and the data were analyzed.

RESULTS

There were 42 patients (27 male, 15 female) with a mean age of 46.3 ± 14.3 years. The average tumor size was 3.1 ± 1.1 cm. The average time to first recurrence was 3.6 ± 5.5 years (range 0.2–27 years). One in 5 patients (8/42) with residual/recurrent tumors did not require any active treatment. Of the 34 patients who underwent repeat treatment, 12 (35.3%) had surgery only (transcranial, endoscopic, or both), 9 (26.5%) underwent surgery followed by adjuvant radiation therapy (RT), and 13 (38.2%) received RT alone. Eighty-six percent (18/21) had a gross-total (n = 4) or near-total (n = 14) resection of the recurrent/residual tumors and had good local control at last follow-up. One of 5 patients (7/34) who underwent repeat treatment had further treatment for a second recurrence. The total duration of follow-up was 8.6 ± 7.1 years. The average Karnofsky Performance Scale score at last follow-up was 80 (range 40–90). There was 1 death.

CONCLUSIONS

Based on this experience and in the absence of guidelines, the authors recommend an individualized approach for the treatment of symptomatic or growing tumors. This study has shown that 1 in 5 patients does not require repeat treatment of their recurrent/residual disease and can be managed with a “scan and watch” approach. On the other hand, 1 in 5 patients who had repeat treatment for their recurrence in the form of surgery and/or radiation will require further additional treatment. More studies are needed to best characterize these patients and predict the natural history of this disease and response to treatment.

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Mohammed J. Asha, Hirokazu Takami, Carlos Velasquez, Selfy Oswari, Joao Paulo Almeida, Gelareh Zadeh and Fred Gentili

OBJECTIVE

Transsphenoidal surgery is advocated as the first-line management of growth hormone (GH)–secreting adenomas. Although disease control is defined by strict criteria for biochemical remission, the length of follow-up needed is not well defined in literature. In this report, the authors present their long-term remission rate and identify various predictive factors that might influence the clinical outcome.

METHODS

The authors conducted a single-institute retrospective analysis of all transsphenoidal procedures for GH-secreting adenomas performed from January 2000 to June 2016. The primary outcome was defined as biochemical remission according to the 2010 consensus criteria and measured at the 1-year postoperative mark as well as on the last recorded follow-up appointment.

Secondary variables included recurrence rate, patterns of clinical presentation, and outcome of adjuvant therapy (including repeat surgery). Subgroup analysis was performed for patients who had biochemical or radiological “discordance”—patients who achieved biochemical remission but with incongruent insulin-like growth factor 1 (IGF-1)/GH or residual tumor on MRI. Recurrence-free survival analysis was conducted for patients who achieved remission at 1 year after surgery.

RESULTS

Eighty-one patients (45 female and 36 male) with confirmed acromegaly treated with transsphenoidal surgery were included. In 62 cases the patients were treated with a pure endoscopic approach and in 19 cases an endoscopically assisted microscopic approach was used.

Primary biochemical remission after surgery was achieved in 59 cases (73%) at 1 year after surgery. However, only 41 patients (51%) remained in primary surgical remission (without any adjuvant treatment) at their last follow-up appointment, indicating a recurrence rate of 31% (18 of 59 patients) over the duration of follow-up (mean 100 ± 61 months). Long-term remission rates for pure endoscopic and endoscopically assisted cases were not significantly different (48% vs 52%, p = 0.6). Similarly, no significant difference in long-term remission was detected between primary surgery and repeat surgery (54% vs 33%, p = 0.22).

Long-term remission was significantly influenced by extent of resection, cavernous sinus invasion (radiologically as well as surgically reported), and preoperative and early postoperative GH and IGF-1 levels (within 24–48 hours after surgery) as well as by clinical grade, with lower remission rates in patients with dysmorphic features and/or medical comorbidities (grade 2–3) compared to minimally symptomatic or silent cases (grade 1).

CONCLUSIONS

The long-term surgical remission rate appears to be significantly less than “early” remission rates and is highly dependent on the extent of tumor resection. The authors advocate a long-term follow-up regimen and propose a clinical grading system that may aid in predicting long-term outcome in addition to the previously reported anatomical factors. The role of repeat surgery is highlighted.

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Ependymoma of the pituitary fossa

Case report and review of the literature

Karim Mukhida, Sylvia Asa, Fred Gentili and Patrick Shannon

✓The authors describe a case of pituitary fossa ependymoma and discuss its immunohistochemical and ultrastructural characteristics. A 43-year-old man presented with decreased libido and panhypopituitarism. Magnetic resonance imaging demonstrated a well-demarcated enhancing lesion of the pituitary fossa that was completely resected via a trans-sphenoidal approach. Ependymomas rarely occur in the pituitary fossa, and have been reported in this location only three times in humans and once in a horse. This is the first study in which investigators examined the appearance of a pituitary ependymoma by using electron microscopy. Theories of the origin and treatment of these rare tumors are discussed as they relate to other articles on intracranial ependymomas.

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Lindsay Symon, Alexander D. Wang, Izabel E. Costa e Silva and Fred Gentili

✓ Somatosensory evoked potentials have been recorded during 34 operations for intracranial aneurysm. The central conduction time (CCT), the time between the N14 peak (recorded at C-2) and the N20 peak (recorded at the cortex) in response to median nerve stimulation, has been found to be increased by administration of halothane, by brain retraction, and by temporary vascular occlusion in some instances. Increase of CCT to more than 10 msec, or disappearance of the response was associated in two cases with postoperative neurological deficit, neither permanent. In a further case, prolongation of CCT was used as a guide to the repositioning of an imperfectly placed clip on a middle cerebral artery aneurysm. The value of the technique as a perioperative monitoring system is discussed.

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Warren P. Mason, Fred Gentili, David R. Macdonald, Subramanian Hariharan, Charlene R. Cruz and Lauren E. Abrey

Object. The management of certain meningiomas of the skull base and those involving the dural venous sinuses remains a challenge. In recent reports it has been suggested that hydroxyurea chemotherapy can cause regression of unresectable and recurrent meningiomas. The authors report their experience in using hydroxyurea for the treatment of patients with recurrent or unresectable meningiomas.

Methods. Hydroxyurea was administered at a dosage of approximately 20 mg/kg/day to 11 women and nine men (median age 59 years, range 31–75 years) with recurrent or unresectable intracranial meningiomas (12 basal, two parasagittal, and six multiple). In 16 patients the meningiomas were benign, in three they had atypical features, and in one the meningioma was malignant. All patients had measurable residual disease. Four patients with benign meningiomas had previously received radiotherapy (two were treated with conventional fractionated radiotherapy and two with stereotactic radiosurgery), three with atypical meningiomas received conventional fractionated radiotherapy, and the one with a malignant meningioma received conventional radiotherapy with additional stereotactic radiosurgery. Tumor enlargement was documented in all patients on neuroimages obtained before initiation of hydroxyurea therapy.

All patients were evaluable for response to therapy. In 12 patients with benign meningiomas, the disease had stabilized on neuroimages obtained posttreatment (median duration of treatment 122 weeks, range 8–151 weeks), and two of these showed clinical improvement. One patient with a benign meningioma experienced a minor partial response that was noted after 39 weeks of treatment and was confirmed on neuroimaging and clinical evaluations. In three others with benign meningiomas, progression was confirmed on neuroimages obtained after 41, 55, and 66 weeks, respectively; the 1-year freedom from progression rate was 0.93 (standard error 0.07) in patients with benign meningiomas. In three patients with atypical meningiomas, the tumors had progressed on neuroimages obtained after 12, 19, and 45 weeks, respectively. In the patient with a malignant meningioma, progression was confirmed on neuroimages obtained at 24 weeks. Hydroxyurea has been reasonably well tolerated, although one patient discontinued therapy because of moderate myelosuppression.

Conclusions. Although tumor regression appears uncommon, these results indicate that hydroxyurea may arrest progression of unresectable or recurrent benign meningiomas.

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Mark Bernstein, Robert A. Hegele, Fred Gentili, Michael Brothers, Richard Holgate, William C. Sturtridge and John Deck

✓ The authors report a case of pituitary apoplexy resulting in bilateral internal carotid artery occlusion, with marked depression of consciousness and hemiplegia. After transsphenoidal tumor decompression, restoration of flow in both carotid arteries was documented angiographically and the patient made an excellent clinical recovery. The unique aspect of this case is that the pituitary apoplexy was apparently precipitated by neuroendocrine manipulation, performed as a preoperative test of pituitary function.

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Abdul Rahman Al-Anazi, William Holliday, Bassem Sheikh and Fred Gentili

✓ Low-grade adenocarcinoma arising from the endolymphatic sac is an uncommon tumor that is characterized clinically by hearing loss and radiologically by temporal bone destruction. The authors report a case of low-grade adenocarcinoma of endolymphatic sac origin that mimicked a posterior fossa meningioma in both clinical and neuroimaging examinations. In this case, the most unusual and interesting feature was the lack of auditory symptoms and temporal bone destruction. The lesion occurred in a 21-year-old man who presented with headaches that had been increasing in frequency for 6 months and was associated with blurring of vision, diplopia, occasional nausea and vomiting, and gait disturbance. On examination, this patient exhibited bilateral papilledema, left sixth cranial nerve palsy, and gait ataxia. Neuroimaging studies revealed a large right posterior fossa tumor. At surgery, a hemorrhagic papillary adenocarcinoma of endolymphatic sac origin was found.