Neurofibromatosis type 2

OBJECTIVE: The best management for patients with small- to medium-sized vestibular schwannomas (VS) is controversial. METHODS: A prospective cohort study of 82 patients with unilateral, unoperated VS less than 3 cm undergoing surgical resection (n = 36) or radiosurgery (n = 46). Patients undergoing resection were younger (48.2 yr versus 53.9 yr, P = 0.03). The groups were similar with regard to hearing loss, associated symptoms, and tumor size. The mean follow-up period was 42 months (range, 12-62 mo). RESULTS: Normal facial movement and preservation of serviceable hearing was more frequent in the radiosurgical group at 3 months (P < 0.001), 1 year (P < 0.001), and at the last follow-up examination (P < 0.01) compared with the surgical resection group. Patients undergoing surgical resection had a significant decline in the following subscales of the Health Status Questionnaire 3 months after surgery: physical functioning (P = 0.006), role-physical (P < 0.001), energy/fatigue (P = 0.02), and overall physical component (P = 0.004). Patients in the surgical resection group continued to have a significant decline in the physical functioning (P = 0.04) and bodily pain (P = 0.04) subscales at 1 year and in bodily pain (P = 0.02) at the last follow-up examination. The radiosurgical group had no decline on any component of the Health Status Questionnaire after the procedure. The radiosurgical group had lower mean Dizziness Handicap Inventory scores (16.5 versus 8.4, P = 0.02) at the last follow-up examination. There was no difference in tumor control (100 versus 96%, P = 0.50). CONCLUSION: Early outcomes were better for VS patients undergoing stereotactic radiosurgery compared with surgical resection (Level 2 evidence). Unless long-term follow-up evaluation shows frequent tumor progression at currently used radiation doses, radiosurgery should be considered the best management strategy for the majority of VS patients.

Patient outcomes after vestibular schwannoma management: a prospective comparison of microsurgical resection and stereotactic radiosurgery.

BackgroundOver the past 10 years, significant anatomic, technical, and instrumentation advances have facilitated the exposure and resection of intradural lesions via a fully endoscopic expanded end...

Nasoseptal flap reconstruction of high flow intraoperative cerebral spinal fluid leaks during endoscopic skull base surgery.

Objective:The incidence of vestibular schwannomas (VSs) approaches 20 per million/yr. As treatment may depend on tumor growth, there is a demand of a treatment strategy based on hard data on the growth pattern of these tumors. This article reports growth data registered prospectively in 552 patients

/pdf/the-natural-history-of-vestibular-schwannoma-3gcebrkppk.pdf

The natural history of vestibular schwannoma.

Objective To evaluate the oncologic outcomes of patients with sinonasal cancer treated with endoscopic resection. Design Retrospective review. Setting Tertiary care academic cancer center. Patients All patients with biopsy-proved malignant neoplasm of the sinonasal region who were treated with endoscopic resection between 1992 and 2007 were included in the study, and their charts were reviewed for demographics, histopathologic findings, treatment details, and outcome. Main Outcome Measures Oncologic outcomes, including disease recurrence and survival. Results Of a total of 120 patients, 93 (77.5%) underwent an exclusively endoscopic approach (EEA) and 27 (22.5%) underwent a cranioendoscopic approach (CEA) in which the surgical resection involved the addition of a frontal or subfrontal craniotomy to the transnasal endoscopic approach. Of the 120 patients, 41% presented with previously untreated disease, 46% presented with persistent disease that had been partially resected, and 13% presented with recurrent disease after prior treatment. The most common site of tumor origin was the nasal cavity (52%), followed by the ethmoid sinuses (28%). Approximately 10% of the tumors had an intracranial epicenter, most commonly around the olfactory groove. Tumors extended to or invaded the skull base in 20% and 11% of the patients, respectively. An intracranial epicenter (P   .99). The cerebrospinal fluid leak resolved spontaneously in 3 patients, and the fourth patient underwent successful endoscopic repair. With a mean follow-up of 37 months, 18 patients (15%) experienced local recurrence, with a local disease control of 85%. Regional and distant failure occurred as the first sign of disease recurrence in 6% and 5% of patients, respectively. The 5- and 10-year disease-specific survival rates were 87% and 80%, respectively. Disease recurrence and survival did not differ significantly between the EEA group and the CEA group. Conclusions To the best of our knowledge, this is the largest US series to date of patients with malignant tumors of the sinonasal tract treated with endoscopic resection. Our results suggest that, in well-selected patients and with appropriate use of adjuvant therapy, endoscopic resection of sinonasal cancer results in acceptable oncologic outcomes.

https://jamanetwork.com/journals/jamaotolaryngology/articlepdf/410734/ooa90097_1219_1224.pdf

Endoscopic resection of sinonasal cancers with and without craniotomy: oncologic results

BackgroundThe increasing expertise in the field of transnasal endoscopic surgery recently has expanded its indications to include the management of sinonasal malignancies. We report our experience ...

Endoscopic surgery for malignant tumors of the sinonasal tract and adjacent skull base: a 10-year experience.

To compare the results of hearing preservation surgeries using different approaches--the enlarged middle cranial fossa approach and the retrosigmoid approach--and different classification systems, stressing the importance of preserving "normal hearing," we performed a retrospective case review in a tertiary care medical center. The charts of 107 patients with vestibular schwannoma who underwent tumor resection were reviewed. Hearing preservation was reported according to 2 different classification systems: the modified Sanna classification and the classification of the American Academy of Otolaryngology-Head and Neck Surgery. The facial nerve results were graded according to the House-Brackmann scale. The hearing preservation rates differed markedly depending on the classification used. We conclude that hearing preservation in acoustic neuroma is a more difficult proposition than most surgeons appreciate, especially in terms of serviceable hearing.

Hearing preservation surgery in vestibular schwannoma: the hidden truth.

Objective:The origin of vestibular schwannoma has always been a matter of debate. The aim of our study was to identify the nerve origin of this tumour.Study design:Prospective case review. This study was conducted at Gruppo Otologico, a private referral centre for neurotology and skull base surgery.Methods:A total of 200 cases of vestibular schwannoma were included in the study. All the tumours were removed surgically utilising the translabyrinthine approach. The origin of the tumour was sought at the fundus of the internal auditory canal.Results:A total of 200 consecutive cases was included in the study. The origin of the tumour was limited to one nerve at the fundus in 152 cases (76 per cent). Out of these cases, the tumour originated from the inferior vestibular nerve in 139 cases (91.4 per cent), from the superior vestibular nerve in nine cases (6 per cent), from the cochlear nerve in two cases (1.3 per cent) and from the facial nerve in two cases (1.3 per cent).Conclusion:The vast majority of vestibular schwannomas originate from the inferior vestibular nerve; the incidence of involvement of this nerve increases as the tumour size increases. An origin of vestibular schwannoma from the inferior vestibular nerve can be considered as one of the explanatory factors for the poor functional outcome of the extended middle cranial fossa approach, and probably accounts also for the better hearing preservation rate reported in some series for the retrosigmoid approach.

https://www.cambridge.org/core/services/aop-cambridge-core/content/view/S0022215107001028

Nerve origin of vestibular schwannoma: a prospective study

Objective: Jugular paragangliomas with extensive involvement of the internal carotid artery (ICA) represent a true challenge for surgeons, especially in the presence of inadequate collateral circulation through the circle of Willis. The aim of our study is to present a preliminary report of our experience with the surgical removal of three such cases using the stenting of the ICA as the method of choice for protecting and preserving the integrity of the artery.

Methods: This retrospective study was conducted at Gruppo Otologico, a private referral center for neurotology and skull base surgery. The subjects of our study are three cases of jugular paragangliomas with extensive involvement of the ICA and inadequate collateral circulation. These cases are the first three cases operated at our center after stenting of the intratemporal portion of the ICA.

Results: Complete surgical removal of the tumor, including the part involving the ICA, was achieved in all cases. Over a follow-up period of 22 to 30 months, no complications occurred and the patency of the stented arteries was preserved.

Conclusion: Although more follow-up is still needed before establishing the exact long-term outcome of stenting the intratemporal ICA, our preliminary report shows that the stent has facilitated the complete surgical removal of the tumor, preserving the integrity of the ICA.

Surgical removal of jugular paragangliomas after stenting of the intratemporal internal carotid artery: a preliminary report.

Objectives/Hypothesis: The purpose of the report was to present an update on the authors' results for prevention and management of cerebrospinal fluid (CSF) leak after translabyrinthine approach for vestibular schwannoma.



Study Design: Retrospective case review.



Methods: The study was conducted at Gruppo Otologico (Piacenza, Italy), a tertiary referral center for neurotology and skull base surgery. In all, 710 patients underwent translabyrinthine approach for the removal of vestibular schwannoma at that institution between April 1987 and December 2002. The medical records were retrospectively reviewed to identify tumor size, the incidence of postoperative CSF leak, and its treatment.



Results: The overall rate of CSF leak was 1.4%.



Conclusion: The use of proper surgical technique minimizes the risk of CSF leak. Study results show that the continued application of the authors' proposed preventive measures resulted in the maintenance of a low rate of CSF leak. Immediate management of CSF fistulae helps prevent meningitis.

Tarek Khrais

Papers

Endoscopic surgery for malignant tumors of the sinonasal tract and adjacent skull base: a 10-year experience.

Hearing preservation surgery in vestibular schwannoma: the hidden truth.

Nerve origin of vestibular schwannoma: a prospective study

Surgical removal of jugular paragangliomas after stenting of the intratemporal internal carotid artery: a preliminary report.

Cerebrospinal Fluid Leak Prevention After Translabyrinthine Removal of Vestibular Schwannoma