Acoustic Neuromas and Neurofibromatosis Type 2

2013 ◽  
Keyword(s):  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Acoustic Neuromas

Growth Rate Characteristics of Acoustic Neuromas Associated With Neurofibromatosis Type 2

1996 ◽  
Vol 106 (6) ◽  
pp. 694-699 ◽  
Author(s):  
Mona M. Abaza ◽  
Erini Makariou ◽  
Mark Armstrong ◽  
Anil K. Lalwani
Keyword(s):  
Growth Rate ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Acoustic Neuromas

scholarly journals Neurofibromatosis type 2 and central neurofibromatosis

1998 ◽  
Vol 4 (3) ◽  
pp. E2 ◽  
Author(s):  
Leonard I. Malis
Keyword(s):  
United States ◽  
Nervous System ◽  
Genetic Research ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
The Past ◽  
Acoustic Neuromas ◽  
Central Neurofibromatosis ◽  
Considerable Range

Neurofibromatosis type 2 (NF2) is a rare disease, affecting only approximately 1000 patients in the entire United States. The diagnosis requires the presence of bilateral acoustic neuromas, but many other tumors of the nervous system are also present. It is a very different disease from von Recklinghausen's neurofibromatosis, NF1. The remarkable genetic research in recent years has defined the origin of NF2 to be the lack of a specific suppressor protein, known as Merlin. While we await a method to replace this protein, the neurosurgical care of these patients is a formidable problem. The author reviews his personal series of 41 patients with NF2 treated during the past 30 years and presents 10 cases in detail to demonstrate their considerable range of differences and the treatment problems they have posed.

scholarly journals 62 Improvement of hearing with bevacizumab in a patient with neurofibromatosis type 2 and bilateral acoustic schwannomas

2018 ◽  
Vol 45 (S3) ◽  
pp. S11-S11
Author(s):  
Dimas Yusuf ◽  
Cian O’Kelly ◽  
Jacob C. Easaw
Keyword(s):  
Hearing Loss ◽  
Disease Progression ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Invasive Treatment ◽  
Auditory Nerves ◽  
Acoustic Neuromas ◽  
Patient Reported ◽  
The Right

BACKGROUND: Neurofibromatosis type 2 (NF2) is a rare genetic condition caused by mutations in the Merlin gene on chromosome 22. It results in acoustic neuromas (schwannomas) and other CNS tumors including meningiomas and ependymomas. Most patients develop hearing loss as a result of neuroma-driven destruction of auditory nerves. Surgery and radiation therapy remain the two most commonly recommended treatment options. However, there is a risk of further hearing loss with these procedures. There is emerging evidence that bevacizumab, a monoclonal antibody against VEGF-A, can shrink acoustic neuromas and mitigate hearing loss. CASE PRESENTATION: A 34-year-old female with bilateral acoustic neuromas from NF2 suffers partial hearing loss in the left ear and total hearing loss in the right ear after removal of the right-sided neuroma. Baseline MRI showed a left-sided acoustic neuroma (15 x 13 mm) and recurrence of the right-sided neuroma (18 x 14 mm). Bevacizumab was initiated at 5 mg/kg IV every 14 days. After 8 cycles, the patient reported marked improvement in hearing. At lower frequencies (< 1,000 Hz, the range of human voice), auditory thresholds improved by up to 60% of baseline, while at higher frequencies, improvements of up to 46% were seen. Repeat imaging showed no disease progression. CONCLUSIONS: Bevacizumab led to hearing improvement and prevention of disease progression after 8 cycles of therapy. This treatment should be considered in patients with NF2 and acoustic neuromas who wish to pursue a less-invasive treatment option with the potential of delaying progression and mitigating hearing loss.

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

1999 ◽  
Vol 6 (3) ◽  
pp. E1
Author(s):  
Brian R. Subach ◽  
Douglas Kondziolka ◽  
L. Dade Lunsford ◽  
David J. Bissonette ◽  
John C. Flickinger ◽  
...  
Keyword(s):  
Stereotactic Radiosurgery ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Tumor Control ◽  
Nerve Function ◽  
Tumor Control Rate ◽  
Acoustic Neuromas ◽  
The Mean

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 better define 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 (36%) tumors 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.

Surgical Management of Vestibular Schwannomas in Neurofibromatosis Type 2

2012 ◽  
Vol 73 (S 02) ◽  
Author(s):  
J. Tysome ◽  
R. MacFarlane ◽  
J. Durie-Gair ◽  
N. Donnelly ◽  
R. Mannion ◽  
...  
Keyword(s):  
Surgical Management ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Vestibular Schwannomas
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