Recognizing schwannomatosis and distinguishing it from neurofibromatosis type 1 or 2

BACKGROUND DATA: Schwannomatosis has become a newly recognized classification of neurofibromatosis. Although the genetic loci are on chromosome 22, it lacks the classic bilateral vestibular schwannomas as seen in NF-2. We present the surgical treatment of 4 patients with schwannomatosis, including a brother and sister. METHOD: Case 1 presented with multiple progressively enlarging peripheral nerve sheath tumors. Case 4 presented with a trigeminal schwannoma and a vagal nerve schwannoma. Three of 4 patients had spinal intradural, extramedullary nerve sheath tumors. Surgery in all was multistaged and consisted of spinal laminectomies, site-specific explorations, and microsurgical tumor dissection and resection, with intraoperative neurophysiologic monitoring (including somatosensory-evoked and motor-evoked potentials, upper extremity electromyography and intraoperative nerve action potential monitoring, as appropriate). RESULTS: Intraoperatively the schwannomas had cystic and solid features and in all surgical cases the tumors arose from discrete fascicles of sensory nerve roots or sensory peripheral nerve branches. None of the patients experienced neurologic worsening as a result of their resections. Pathologic analysis of specimens from all cases demonstrated schwannoma. CONCLUSIONS: Not all patients with multiple schwannomas of cranial nerve, spinal nerve root, or peripheral nerve origin have NF-1 or NF-2. In schwannomatosis, these lesions are present in the absence of cutaneous stigmata, neurofibromas, vestibular schwannomas, or parenchymal brain tumors. Schwannomas in schwannomatosis can be large, cystic, and multiple. However, the predominant nerve involvement seems to be sensory and discrete fascicular in origin, facilitating microsurgical resection with minimal deficit.     

雪旺细胞瘤病的诊断与治疗

目的 雪旺(施万氏)细胞瘤病是新近被认识的一种少见的周围神经系统肿瘤,与Ⅱ型神经纤维瘤病的临床特点不同.本文旨在介绍其诊治原则.方法 2002至2007年间,共治疗6例周围神经雪旺细胞瘤病患者.女5例,男1例;年龄22～54岁,平均36.5岁.累及神经:桡神经、隐神经1例,腓肠神经、腓总神经1例,双侧腓总神经1例,胫神经1例,正中神经和指总神经2例.所有患者均采用手术治疗.术后经病理证实有两个或多个雪旺细胞瘤.回顾性调查这6例患者的临床表现、影像学资料、组织学特点和治疗结果.结果 雪旺细胞瘤病患者常见症状为多发局部包块,疼痛,触之加重,并放射至神经分布区.均无牛奶咖啡斑.MR检查可见肿物呈圆形或椭圆形,边界清晰,两端可见条索状神经影像.T1加权像低或中度信号;T2加权像高信号,密度不均.术中所见与单发雪旺细胞瘤相似.病理检查肿物主要由Antoni A区和Antoni B区组成.2例经颅脑MR检查排除前庭神经肿瘤.术后平均随访23个月.4例症状消失,功能满意;1例手掌处肿物复发.结论 雪旺细胞瘤病好发年龄30～60岁,女性多见.特点是多发雪旺细胞瘤,无前庭神经雪旺细胞瘤的临床证据和表现.手术切除效果满意.     

Management of multiple tumors in neurofibromatosis type 2 patients

Introduction

Neurofibromatosis type 2 is characterized by the presence of bilateral vestibular schwannomas. However, other nervous system tumors may also occur. Therefore, the management of NF2 patients is complex and requires a multidisciplinary discussion in a specialized center.

Microsurgical management of spinal schwannomas: evaluation of 128 cases

OBJECT: The authors conducted a study to evaluate the clinical characteristics and surgical outcomes in patients with spinal schwannomas and without neurofibromatosis (NF). METHODS: The data obtained in 128 patients who underwent resection of spinal schwannomas were analyzed. All cases with neurofibromas and those with a known diagnosis of NF Type 1 or 2 were excluded. Karnofsky Performance Scale (KPS) scores were used to compare patient outcomes when examining the anatomical location and spinal level of the tumor. The neurological outcome was further assessed using the Medical Research Council (MRC) muscle testing scale. RESULTS: Altogether, 131 schwannomas were treated in 128 patients (76 males and 52 females; mean age 47.7 years). The peak prevalence is seen between the 3rd and 6th decades. Pain was the most common presenting symptom. Gross-total resection was achieved in 127 (97.0%) of the 131 lesions. The nerve root had to be sacrificed in 34 cases and resulted in minor sensory deficits in 16 patients (12.5%) and slight motor weakness (MRC Grade 3/5) in 3 (2.3%). The KPS scores and MRC grades were significantly higher at the time of last follow-up in all patient groups (p = 0.001 and p = 0.005, respectively). CONCLUSIONS: Spinal schwannomas may occur at any level of the spinal axis and are most commonly intradural. The most frequent clinical presentation is pain. Most spinal schwannomas in non-NF cases can be resected totally without or with minor postoperative deficits. Preoperative autonomic dysfunction does not improve significantly after surgical management.     

Spinal schwannomatosis in the absence of neurofibromatosis: A very rare condition

Schwannomatosis is defined as an extremely rare tumors syndrome characterized by the presence of multiple schwannomas in the absence of typical signs of NF1 and NF2 syndromes. The genetic and molecular analysis performed on these tumors makes it possible to name schwannomatosis as distinct clinical and genetic syndrome. The treatment in the case of symptomatic lesions is surgical removal; if the lesions are asymptomatic it is better to perform serial MRI studies. Given the high incidence of developing additional lesions in patients with schwannomatosis, it remains imperative to perform serial brain and spinal cord MRI studies during follow-up. The differential diagnosis is important including clinical and radiological criteria plus molecular genetic analysis of tumor cells and lymphocyte DNA. We report a rare case of spinal schwannomatosis in which genetic analysis performed on surgical samples showed two different mutations in the cells of the two lesions.     

Multiple schwannomas: report of two cases

In this paper authors present two cases of multiple schwannomas without the features of neurofibromatosis (NF). The authors retrospectively reviewed the hospital charts, radiology films, operative notes and pathology slides of these two patients. There was no family history of neurofibromatosis. The two patients had contrast enhanced MRI, which was negative for vestibular schwannomas. Both underwent surgical excision of symptomatic lesions. Histopathology confirmed these lesions as schwannomas. Molecular genetic analysis in case 1 demonstrated two distinct mutations of the NF2 gene in two different schwannomas, with concomitant loss of heterozygosity in both tumours. In contrast peripheral blood lymphocytes did not reveal mutations of NF2. The authors recommend surgery for symptomatic lesions. Asymptomatic tumours can be monitored. Regular follow up is essential as they may develop fresh lesions at any time. The relevant literature is discussed.

     

A Linear Variant of Segmental Schwannomatosis Localized to the Upper Extremity

Introduction  Schwannomas represent benign peripheral nerve sheath tumors. Their phenotypic presentations in schwannomatosis and segmental schwannomatosis have been well described. To date, however, cases of schwannomatosis or multiple schwannomas localized to a single nerve fascicle have been limited in the literature. Case Presentation  In this study, we identify a case of a 48 year-old non-neurofibromatosis male who presented with symptomatic schwannomas localized to a single nerve fascicle of the upper extremity. Intraoperative exploration revealed four schwannomas arising from a 15-cm segment of ulnar nerve fascicle. Surgical excision was successful, without neurological deficit or recurrence. Conclusion  This study identifies a case of schwannomatosis localized to a single nerve fascicle that may represent a linear variant of segmental schwannomatosis. The presentation may represent a temporary linear appearance in progression to “nonlinear” segmental variant; however, a molecularly distinct subset of schwannomas cannot be excluded.     

A non-NF2 case of schwannomas of vestibular and trigeminal nerves with different genetic alterations of NF2 gene: case report

BACKGROUND: We report a patient with 2 separate schwannomas, a vestibular schwannoma and a trigeminal schwannoma, that were attached to each other and appeared to be a single tumor on imaging studies. CASE DESCRIPTION: The patient, without any family history of neurofibromatosis, presented with a progressive hearing loss and mild left facial nerve palsy. Magnetic resonance imaging showed a snowman-like tumor in the left cerebellopontine angle. Surgical exposure revealed that the tumor consisted of 2 "kissing" schwannomas, a trigeminal and vestibular schwannoma. Molecular genetic analysis detected a 1-base pair deletion at exon 10 of the neurofibromatosis type 2 (NF2) gene in the trigeminal schwannoma, but not in the acoustic schwannoma. However, loss of heterozygosity at chromosome 22q (D22S282 and D22S929) was detected in both tumors, losing the same allele. CONCLUSION: Multiple schwannomas in non-NF2 patients are extremely rare, and possible causes include simple coincidence or germline genetic alteration of adjacent gene on chromosome 22q, similar to the cause recently suggested in familial schwannomatosis. Although not always possible, molecular genetic examination may help to understand the underlying mechanism and would be warranted in such cases.     

Reduced-dose radiosurgery for vestibular schwannomas

OBJECTIVE: To evaluate tumor control and complications associated with low-dose radiosurgery for vestibular schwannomas. METHODS: Between December 1993 and January 2000, 47 patients with vestibular schwannomas were treated at our center with gamma knife radiosurgery. The marginal tumor doses ranged from 7.5 to 14.0 Gy (median, 12.0 Gy) for patients treated after microsurgery and from 10.0 to 15.0 Gy (median, 12.0 Gy) for patients in whom radiosurgery was the primary treatment. The median maximum tumor diameter was 18 mm (range, 3-50 mm). Evaluation included audiometry, neurological examination, and serial imaging tests. A survey was conducted at the time of analysis. RESULTS: Follow-up data were available for 45 patients and ranged from 1 to 7 years (median, 3.6 yr). In 43 patients (96%), tumor control (no radiographic progression or surgical resection) was observed. All 33 previously untreated patients had tumor control. Transient facial weakness, experienced in two patients (4%), had resolved completely within 6 months. No patient developed trigeminal neuropathy. Hearing was diminished from baseline in 12% of patients with useful hearing (Gardner-Robertson Class III). However, all patients with pretreatment hearing Gardner-Robertson Class I or II maintained testable hearing (Class I to III) at the most recent examination. CONCLUSION: Low-dose radiosurgery in this series provided comparable local control and decreased incidences of complications in relation to other reports. Additional follow-up will allow more definitive conclusions to be reached regarding the ultimate rates of tumor control and hearing preservation. Nevertheless, the current dose used for vestibular schwannomas at the University of Maryland Medical Center is 12.0 Gy to the tumor periphery.     

Hybrid neurofibroma/schwannoma is overrepresented among schwannomatosis and neurofibromatosis patients

We analyzed the histologic features of peripheral nerve sheath tumors occurring in 14 patients with schwannomatosis. Among a total of 31 tumors, 19 tumors (61%) showed schwannoma-like nodules within a neurofibroma-like tumor, corresponding to hybrid neurofibroma/schwannoma. At least 1 hybrid tumor occurred in 10 of 14 (71%) schwannomatosis patients. We then retrieved cases of hybrid tumors without documented relation to schwannomatosis from our database and identified 41 tumors arising in 23 patients. More than half of these patients (14/23) were reported to suffer from multiple peripheral nerve sheath tumors, favoring a tumor syndrome. Indeed, analysis of clinical records revealed the diagnosis of neurofibromatosis type 2 (NF2) in 26% (6/23), neurofibromatosis type 1 (NF1) in 9% (2/23), definite schwannomatosis in 4% (1/23), and possible schwannomatosis in 13% (3/23) of patients with multiple nerve sheath tumors. Our findings suggest that hybrid neurofibroma/schwannoma represents a common tumor type in schwannomatosis and shows a striking association with neurofibromatoses.     

Unilateral vestibular schwannoma with other neurofibromatosis type 2-related tumors: clinical and molecular study of a unique phenotype

OBJECT: Although the manifestations of neurofibromatosis Type 2 (NF2) vary, the hallmark is bilateral vestibular schwannomas (VSs). The authors studied the clinical course and genetic basis of unilateral VSs associated with other NF2-related tumors. METHODS: Forty-four adults presenting with unilateral VSs and other NF2-related tumors were identified. A comprehensive review of patient records and cranial imaging was conducted. Molecular analysis of the NF2 locus was performed in available tumors and paired blood specimens. Patient age at symptomatic onset ranged from 11 to 63 years (mean 32 years). Twenty-two patients (50%) presented with eighth cranial nerve dysfunction. Twenty-six presented with multiple lesions. Thirty-eight harbored other intracranial tumors and 27 had spinal tumors, with most lesions situated ipsilateral to the VS. No patient had a relative with NF2, although two of 63 offspring had isolated NF2-related findings. A contralateral VS developed in four patients 3 to 46 years after the symptomatic onset of a unilateral VS, and two of these patients experienced rapid progression to total deafness. Results of a Kaplan-Meier analysis identified actuarial chances of developing contralateral VS: 2.9% (3-17 years after the VS symptomatic onset of unilateral VS), 11% (18-24 years), and 28.8% (25-40 years). Mosaicism for the NF2 gene mutation was proven in eight patients. CONCLUSIONS: The authors describe the clinical features of this unique phenotype--unilateral VS with other NF2-related tumors. Persons with this phenotype should undergo evaluation and monitoring similar to that conducted in patients with NF2, and the possibility of aggressive contralateral VS formation should be considered in their treatment. Molecular genetic analysis is best performed using resected tumor specimens and will enable future studies to determine the genetic risks of individuals with mosaicism.     

The comparison between the growth fraction of bilateral vestibular schwannomas in neurofibromatosis 2 (NF2) and unilateral vestibular schwannomas using the monoclonal antibody MIB 1

Summary Formalin-fixed paraffin sections of 55 consecutive bilateral vestibular schwannomas in 46 patients with neurofibromatosis 2 (NF2), and 50 patients with unilateral vestibular schwannomas were investigated immunohistochemically with the monoclonal antibody MIB 1 directed against recombinant parts of Ki-67 antigen.The immunohistochemical staining was carried out on dewaxed microwave oven-processed paraffin sections of formalin-fixed tumour tissues. The labelling index (LI) obtained was compared to clinical and histological findings in both groups.There was no correlation between the LI and age of the patients, tumour size, or histological type of tumour (Antoni A or B). Vestibular schwannomas in NF2 showed higher LI than unilateral vestibular schwannomas: the maximal LI found per section (LI max) ranged from 0.4 to 17.6% (mean, 2.7%) in NF2 schwannomas, and from 0 to 9% (mean, 2.2%) in unilateral schwannomas.These differences may express immunohistochemically some clinical and morphological differences between bilateral and unilateral vestibular schwannomas.     

Resection of large vestibular schwannomas: facial nerve preservation in the context of surgical approach and patient-assessed outcome

OBJECT: Vestibular schwannoma surgery has evolved as new therapeutic options have emerged, patients' expectations have risen, and the psychological effect of facial nerve paralysis has been studied. For large vestibular schwannomas for which extirpation is the primary therapy, the goals remain complete tumor resection and maintenance of normal neurological function. Improved microsurgical techniques and intraoperative facial nerve monitoring have decreased the complication rate and increased the likelihood of normal to near-normal postoperative facial function. Nevertheless, the impairment most frequently reported by patients as an adverse effect of surgery continues to be facial nerve paralysis. In addition, patient assessment has provided a different, less optimistic view of outcome. The authors evaluated the extent of facial function, timing of facial nerve recovery, patients' perceptions of this recovery and function, and the prognostic value of intraoperative facial nerve monitoring following resection of large vestibular schwannomas; they then analyzed these results with respect to different surgical approaches. METHODS: The authors retrospectively reviewed a database of 67 patients with 71 vestibular schwannomas measuring 3 cm or larger in diameter. The patients had undergone surgery via translabyrinthine, retrosigmoid, or combined approaches. Clinical outcomes were analyzed with respect to intraoperative facial nerve activity, responses to intraoperative stimulation, and time course of recovery. Eighty percent of patients obtained normal to near-normal facial function (House-Brackmann Grades I and II). Patients' perceptions of facial nerve function and recovery correlated well with the clinical observations. CONCLUSIONS: Trends in the data lead the authors to suggest that a retrosigmoid exposure, alone or in combination with a translabyrinthine approach, offers the best chance of facial nerve preservation in patients with large vestibular schwannomas.     

Neurogenic tumors of the cervical vagus nerve: report of four cases and review of the literature

OBJECTIVE AND IMPORTANCE: Nerve sheath tumors arising from the cervical vagus nerve are extremely rare. These tumors most often present as asymptomatic, slowly enlarging, lateral neck masses and therefore often come initially to the attention of otolaryngologists and general surgeons. Because they are nerve tumors, however, neurosurgeons must be able to recognize and treat these rare entities. We report three cases of schwannoma and one case of neurofibroma of the cervical vagus nerve that were encountered at our center (Louisiana State University Medical Center) during a 31-year period. CLINICAL PRESENTATION: The patients ranged from 31 to 61 years of age at the time of presentation to Louisiana State University Medical Center. Presenting complaints included hoarseness, Horner's syndrome, and palpation of an enlarging, asymptomatic, cervical mass. Reviews of systems revealed episodes of aspiration for one patient and frequent respiratory illnesses for two patients. These episodes were possibly related to their tumors. Imaging studies demonstrated well-circumscribed masses in the region of the carotid sheath. INTERVENTION: Using microsurgical techniques, gross total resection of all four tumors was accomplished. For one patient, the vagus nerve needed to be divided and an end-to-end anastomosis was performed. For the other three patients, resection of the tumor was achieved with the vagus nerve in continuity. CONCLUSION: Vagal nerve schwannomas and neurofibromas in the neck are rare neoplasms. We present four cases of these benign tumors. The pathological features, epidemiological characteristics, presentation, differential diagnosis, and management are discussed. Gross total resection with preservation of the vagus nerve remains the treatment of choice.     

Soft tissue perineurioma in a patient with neurofibromatosis type 2: a tumor not previously associated with the NF2 syndrome

Neoplasms that commonly affect patients with neurofibromatosis type 2 (NF2) include schwannomas, meningiomas, astrocytomas, ependymomas, and neurofibromas. Perineuriomas are rare tumors of the peripheral nerve sheath that share some characteristics with meningioma. As in both NF2-associated and sporadic cases of schwannoma and meningioma, perineuriomas often harbor mutations or deletions of the NF2 gene. However, perineuriomas have not previously been reported in the clinical setting of NF2. A 30-year-old man with a history of bilateral vestibular schwannomas, a parasagittal meningioma, an intraspinal ependymoma, and multiple other neoplasms involving both cranial and peripheral nerves (thereby fulfilling the diagnostic criteria for NF2) presented with an enlarging thigh mass. The diagnosis of cellular soft tissue perineurioma was confirmed by both immunohistochemical and ultrastructural analysis. This case represents the first report of a soft tissue perineurioma arising in the setting of NF2.     

Treatment of orbital schwannomas and neurofibromas

We present an overview of the treatment and clinical outcome of five orbital peripheral nerve tumours, carried out in our centre from 1999 to 2003. The surgical approach was determined by the location and extension of the lesion. Supraorbital orbitotomy was performed in two superiorly located lesions, a transconjunctival approach in one medial, basal, extraconal lesion. A pterional extradural approach was used in two cases with involvement of the apex, superior orbital fissure and cavernous sinus. Three patients were diagnosed as having schwannoma, one as neurofibroma, and one as cystic mixed neurofibroma and schwannoma. One patient suffered from multiple schwannomas [bilateral acoustic schwannomas, cervical schwannomas (NF2)]. One patient showed bilateral orbital neurofibromas, plexiform cutaneous neurofibroma (NF1) and glaucoma due to a coexisting Marfan's syndrome. Local recurrences were not seen after complete resection in all patients. Surgery is the therapeutic goal.     

Therapeutics for Childhood Neurofibromatosis Type 1 and Type 2

Neurofibromatosis type 1 (NF1) and type 2 (NF2) are genetically and medically distinct neurocutaneous disorders that are both associated with tumors affecting the central and peripheral nervous systems. NF1 has a frequency of 1 in 3,000, compared with 1 in 30,000 for NF2. Careful surveillance is important for both conditions, to allow early identification and treatment of complications. The most common and important problems in NF1 are cognitive impairment, optic pathway gliomas, plexiform neurofibromas, and orthopaedic issues. Early intervention and tailored educational programs are indicated for learning difficulties. Attention deficit hyperactivity disorder may be amenable to treatment with stimulant medication. A clinical trial is under way to evaluate lovastatin in the treatment of cognitive problems in children with NF1. Chemotherapy with vincristine and carboplatin is the current standard of care for symptomatic optic pathway gliomas, but new agents with improved efficacy are needed. Plexiform neurofibromas may be treated with surgery, but often recur. To date, no medical therapy has proven effective in limiting plexiform neurofibroma growth, but several candidate medications are under consideration in clinical trials. Malignant peripheral nerve sheath tumors may arise in preexisting plexiform neurofibromas, so changes in tumor growth or an increase in pain or focal neurologic deficit should prompt further investigation and early treatment with wide surgical resection, with or without adjuvant chemotherapy or radiotherapy. Specialist surgical intervention may be needed for scoliosis and tibial pseudoarthrosis. In NF2, surgical treatment remains a cornerstone of management for symptomatic progressive vestibular schwannomas, meningiomas, and spinal tumors. Vascular endothelial growth factor inhibitors show promise for the treatment of vestibular schwannomas, with the aim of delaying surgery, and other targeted molecular therapies are becoming available as investigational options. Hearing aids and brainstem and cochlear implants have a role in optimizing functional hearing in some patients. Specialist ophthalmology input should be arranged to monitor for ophthalmologic complications. A coordinated effort is needed to enroll NF1 and NF2 patients in international multicenter clinical trials of promising new pharmacologic agents. Genetic testing is useful for prenatal diagnosis and may be important in understanding individual responses to novel medical therapies in the future. Effective transition to adult services is important, considering the likelihood of further complications in the adult years.     

Endoscopic Resection of Vestibular Schwannomas

Objective To report our results and the technical details of fully endoscopic resection of vestibular schwannomas. Design Prospective observational study. Setting A single academic institution involving neurosurgery and neurotology. Participants Twelve consecutive patients who underwent fully endoscopic resection of a vestibular schwannoma. Main Outcome Measures Hearing preservation, based on the American Association of Otolaryngology-Head and Neck Surgeons (AAO-HNS) score as well as the Gardener and Robertson Modified Hearing Classification (GR). Facial nerve preservation based on the House-Brackmann (HB) score. Results All patients successfully underwent gross total resection. Facial nerve preservation rate was 92% with 11 of 12 patients retaining an HB score of 1/6 postoperatively. Hearing preservation rate was 67% with 8 of 12 patients maintaining a stable AAO-HNS grade and GR score at follow-up. Mean tumor size was 1.5 cm (range: 1–2 cm). No patients experienced postoperative cerebrospinal fluid leak, infection, or cranial nerve palsy for a complication rate of 0%. Mean operative time was 261.6 minutes with an estimated blood loss of 56.3 mL and average length of hospital stay of 3.6 days. Conclusion A purely endoscopic approach is a safe and effective option for hearing preservation surgery for vestibular schwannomas in appropriately selected patients.     

Stereotactic radiosurgery in the management of acoustic neuromas associated with neurofibromatosis Type 2

OBJECT: Stereotactically guided radiosurgery is one of the primary treatment modalities for patients with acoustic neuromas (vestibular schwannomas). The goal of radiosurgery is to arrest tumor growth while preserving neurological function. Patients with acoustic neuromas associated with neurofibromatosis Type 2 (NF2) represent a special challenge because of the risk of complete deafness. To define better the tumor control rate and long-term functional outcome, the authors reviewed their 10-year experience in treating these lesions. METHODS: Forty patients underwent stereotactic radiosurgery at the University of Pittsburgh, 35 of them for solitary tumors. The other five underwent staged procedures for bilateral lesions (10 tumors, 45 total). Thirteen patients (with 29% of tumors) had undergone a median of two prior resections. The mean tumor volume at radiosurgery was 4.8 ml, and the mean tumor margin dose was 15 Gy (range 12-20 Gy). The overall tumor control rate was 98%. During the median follow-up period of 36 months, 16 tumors (36%) regressed, 28 (62%) remained unchanged, and one (2%) grew. In the 10 patients for whom more than 5 years of clinical and neuroimaging follow-up results were available (median 92 months), five tumors were smaller and five remained unchanged. Surgical resection was performed in three patients (7%) after radiosurgery; only one showed radiographic evidence of progression. Useful hearing (Gardner-Robertson Class I or II) was preserved in six (43%) of 14 patients, and this rate improved to 67% after modifications made in 1992. Normal facial nerve function (House-Brackmann Grade 1) was preserved in 25 (81%) of 31 patients. Normal trigeminal nerve function was preserved in 34 (94%) of 36 patients. CONCLUSIONS: Stereotactically guided radiosurgery is a safe and effective treatment for patients with acoustic tumors in the setting of NF2. The rate of hearing preservation may be better with radiosurgery than with other available techniques.     

Treatment of large and giant residual and recurrent vestibular schwannomas.

This report is a retrospective analysis of the surgical outcome of 15 patients (8 females, 7 males; mean age, 37.8 years) with residual or recurrent vestibular schwannomas operated on between 1987 and 2005. These 15 patients were part of a larger series of 252 consecutive vestibular schwannoma excisions. Tumors were classified as large (10) when their diameter exceeded 3.5 cm and giant (5) when their diameter exceeded 4.5 cm. All patients had previously undergone surgery. Hearing was lost in all cases, 8 had complete facial nerve palsy, 6 had trigeminal nerve deficits, 5 had cranial nerve IX and X palsy, and 10 had ataxic gait. Twelve patients had hydrocephalus. The tumors were reoperated through the retrosigmoid-transmeatal approach. The mean postoperative follow-up was 4.9 years. Complete resection was achieved in all patients. The facial nerve was preserved in 6 of the 7 patients with preoperative facial function. Transient worsening of bulbar cranial nerves palsy occurred in 2 cases. Cerebrospinal fluid leakage occurred in 3 patients. There were no deaths, and the tumors were histologically benign. Surgical removal is the only treatment for these lesions. Total resection associated with a low morbidity rate is possible. Preservation of the facial nerve is difficult due to severe scar tissue.