The response of spinal cord ependymomas to bevacizumab in patients with neurofibromatosis Type 2 (original) (raw)
Related papers
Bevacizumab treatment for symptomatic spinal ependymomas in neurofibromatosis type 2
Acta neurologica Scandinavica, 2015
Neurofibromatosis type 2 (NF2) is a tumor suppressor syndrome associated with vestibular schwannomas, meningiomas, and spinal ependymomas. There have been anecdotal reports of radiographic response of spinal ependymomas in NF2 patients being treated for progressive vestibular schwannomas with bevacizumab, a monoclonal antibody against vascular endothelial growth factor (VEGF). The aim of this study was to review the clinical effects of bevacizumab treatment for symptomatic, NF2-associated ependymomas METHODS: We conducted a retrospective review of all patients with NF2 treated with bevacizumab for symptomatic ependymoma at three NF2 specialty centers. Tumor size was evaluated by linear measurements; radiographic response was defined as >20% reduction in tumor size. We also performed immunohistochemical evaluation of NF2-associated symptomatic ependymomas from five patients, including two from this clinical series. Eight patients with NF2 and symptomatic ependymoma were treated wi...
Neuropathology, 2012
Neurofibromatosis type 2 (NF2) is a hereditary tumor syndrome. The hallmark of NF2 is bilateral vestibular schwannoma. In addition, glioma is one of the diagnostic criteria of NF2. In this retrospective study the clinical presentation and histopathological features of 12 spinal gliomas from NF2 patients were assessed. Ten tumors were previously diagnosed as ependymomas and two as astrocytomas. However, upon re-evaluation both astrocytomas expressed epithelial membrane antigen in a dot-like fashion and in one case it was possible to perform electron microscopy revealing junctional complexes and cilia typical for ependymoma. The findings suggest that NF2-associated spinal gliomas are ependymomas. Based on the fact that NF2-associated gliomas are almost exclusively spinal and that no NF2 mutations have been found in sporadic cerebral gliomas, we suggest that "glioma" in the current diagnostic criteria for NF2 should be specified as "spinal ependymoma".
Child's Nervous System, 2011
Introduction Neurofibromatosis type 2 (NF2) is an inherited, rare autosomal dominant syndrome characterised by the development of multiple benign cranial and spinal tumours, peripheral neuropathy, ophthalmological and cutaneous lesions. Herein, we report one case of NF2 treated with multivariate chemotherapy. Material and methods A 13-year-old female presented with multiple cranio-spinal tumours in MRI. First symptoms were progressive changes in vision, left-sided paresis, unilateral sensorineural hearing loss, and left hypoglossal nerve paresis. The patient underwent palliative, partial surgical resection of the tumour which was located in a posterior fossa. Histopathological examination showed a psammomatous meningioma located near the great foramen and schwannomas of VIII nerve in the cerebello-pontine angle. Clinical and radiological examination revealed a rapid progression of the disease. As such, multivariate chemotherapy was used. The patient died 4 years after diagnosis. Conclusion NF2 patients with multiple tumours at diagnosis may not be treatable with surgery alone and, as a result, presentation with such a disease in childhood results in poor prognosis. The unification of management strategies in NF2 patients is highly desirable.
Long-term natural history of neurofibromatosis Type 2–associated intracranial tumors
Journal of Neurosurgery, 2012
Object Neurofibromatosis Type 2 (NF2) is a heritable tumor predisposition syndrome that leads to the development of multiple intracranial tumors, including meningiomas and schwannomas. Because the natural history of these tumors has not been determined, their optimal management has not been established. To define the natural history of NF2-associated intracranial tumors and to optimize management strategies, the authors evaluated long-term clinical and radiographic data in patients with NF2. Methods Consecutive NF2 patients with a minimum of 4 years of serial clinical and MRI follow-up were analyzed. Results Seventeen patients, 9 males and 8 females, were included in this analysis (mean follow-up 9.5 ± 4.8 years, range 4.0–20.7 years). The mean age at initial evaluation was 33.2 ± 15.5 years (range 12.3–57.6 years). Patients harbored 182 intracranial neoplasms, 164 of which were assessable for growth rate analysis (18 vestibular schwannomas [VSs], 11 nonvestibular cranial nerve [CN]...
Successful step-by-step treatment of multiple tumours in neurofibromatosis type 2
Interdisciplinary Neurosurgery, 2019
A clinical study on successful treatment of multifocal brain and spinal cord lesions in neurofibromatosis type 2 in a woman aged 35 years is presented. Magnetic resonance imaging showed bilateral vestibular schwannomas, brain meningiomatosis and multiple spinal intramedullary tumours. The treatment comprised four stages: removal of giant dextral vestibular schwannomas, removal of convexital cerebral meningiomatosis, whole-brain radiation therapy and follow-up. The spinal cord mass was asymptomatic during the entire follow-up. These steps enabled achievement of a long relapse-free period and resulted in good functional outcomes.
Clinical Radiology, 2008
To assess the magnetic resonance imaging (MRI) features and natural history of intramedullary tumours in patients with neurofibromatosis type 2 (NF2). MATERIALS AND METHODS: Eleven NF2 patients with intramedullary spinal cord tumours were identified from the database of the multidisciplinary NF2 clinic. All the imaging studies of these patients were individually reviewed by two neuroradiologists to evaluate the size, number, location, imaging characteristics, and interval growth of the intramedullary tumours. RESULTS: Two of the 11 patients had lesions that required surgery. Both these lesions were in the cervical region, and extended over three and five segments respectively. Nine patients with a mean imaging follow-up period of 77 months had lesions that remained stable, apart from the development of small peritumoral cysts in three. The lesions were well circumscribed, often multiple, usually less than 1 cm in diameter, and were most frequently found in the cervical cord. CONCLUSION: The majority of intramedullary tumours in NF2 patients are very slow growing and share certain MRI features that differ from those of progressive or symptomatic lesions.
Neuro-Oncology Advances
Background Neurofibromatosis type 2 (NF2)-related schwannomatosis is an autosomal dominant tumor-predisposition syndrome characterized by bilateral vestibular schwannomas (VS). In patients with VS associated with NF2, vascular endothelial growth factor A inhibitor, bevacizumab, is a systemic treatment option. The aim of this study is to retrospectively evaluate NF2 patient responses to bevacizumab on VS growth and symptom progression. Methods This is a retrospective analysis of patients seen at the Mayo Clinic Rochester Multidisciplinary NF2 Clinic. Results Out of 76 patients with NF2 evaluated between 2020 and 2022, we identified 19 that received treatment with bevacizumab. Thirteen of these patients discontinued bevacizumab after median treatment duration of 12.2 months. The remaining 6 patients are currently receiving bevacizumab treatment for a median duration of 9.4 months as of March, 2023. Fifteen patients had evaluable brain MRI data, which demonstrated partial responses in ...
Neurologia medico-chirurgica
We conducted a feasibility study to investigate the therapeutic effect of bevacizumab on vestibular schwannomas (VS) associated with neurofibromatosis type 2 (NF2) in a sample of Japanese patients. Ten NF2 patients were selected between 2013 and 2018: nine women and one man, with ages ranging from 12 to 45 years (mean: 29.4). Bevacizumab was administered intravenously in 5 mg/kg doses four times, with an inter-dose interval of 2 weeks. Seventeen tumors were followed for 3-72 months (mean: 39). A reduction from baseline tumor volume of at least 20% was considered a therapeutic radiologic response. Maximum reduction in tumor volume was identified in the 3rd month in 11 tumors, and in the 6th month in three tumors. Three tumors did not show any response to bevacizumab. A radiologic response was detected in seven tumors (41%). There was a significantly lower tumor volume mean in the 3rd month in comparison to the baseline for the entire sample. Tumors in patients aged 25 and above showed a significant reduction in volume in the 3rd month and significantly lower tumor-volume-to-baseline ratio than younger patients in both the 3rd and 6th months. The interaction between 'time' and 'age group' factors significantly affected the therapeutic outcome of bevacizumab on tumor volume. This study investigated the therapeutic effects of bevacizumab on NF2-associated vestibular schwannomas in Japanese patients. Bevacizumab appears to be a useful therapeutic choice in NF2 cases to control the growth of VS. Therefore, a randomised control trial to prove this assumption is necessary.
Sporadic spinal schwannomas and neurofibromas - a review
Folia medica, 2021
Tumours arising from the spinal nerve roots are more common in men between the third and fourth decades of life, located mainly in the intradural space of the lumbar region. The clinical symptoms of spinal schwannomas and neurofibromas are similar and depend on the location of the tumour and its relationship to the spinal cord and nerve roots. The pain (radiculalgia and/or vertebralgia) is the earliest symptom, followed by sensory deficit, while motor deficits and sphincter disorders are late symptoms. Magnetic resonance imaging is the diagnostic tool of choice and should be performed when the disease is suspected, as early and accurate diagnosis is essential for treatment outcome. The treatment of these lesions is surgical aiming at total tumour resection that promotes neurological recovery and prevents from recurrences. Subtotal removal also provides favourable outcome but at higher recurrence rates. The aim of this literature review was to study some important characteristics of ...