1/38. A case of laryngeal neurinoma with neurofibromatosis 2.We present a case of a laryngeal neurinoma in a patient with neurofibromatosis 2. A 39-year-old man presented to our hospital with multiple complaints including progressive bilateral hearing loss, dizziness, dyspnea, dysphagia, and a 9-year history of right lower leg weakness. magnetic resonance imaging demonstrated multiple lesions including bilateral cerebellopontine angle tumors, a foremen magnum tumor, multiple tumors of the spinal cord, a laryngeal tumor, and several retrocervical tumors. Fiberoptic laryngoscopy revealed a large submucosal supraglottic tumor. The laryngeal tumor was visualized through microlaryngoscopy and excised with a KTP laser directed through a quartz fiber.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
2/38. spinal cord ganglioglioma in a child with neurofibromatosis type 2. Case report and literature review.Gangliogliomas of the spinal cord are rare disease entities that occur in early childhood. Their occurrence in association with neurofibromatosis Type 2 (NF2) has not been described. The authors describe the unique case of a 2-year-old child with stigmata of NF2 who harbored a spinal cord ganglioglioma that presented as a rapidly growing, exophytic intramedullary mass lesion at the cervicomedullary junction. Treatment consisted of complete surgical resection. Histopathological analysis of the lesion demonstrated a mixed population of neoplastic cells, of both neuronal and glial lineage, that supported the diagnosis of ganglioglioma.- - - - - - - - - - ranking = 2keywords = spinal (Clic here for more details about this article) |
3/38. Bilateral facial nerve schwannomas.facial nerve schwannoma is an uncommon tumor and bilateral facial nerve tumors are extremely rare. A case is presented in which neuromas affecting the intra-canalicular and labyrinthine portions of both facial nerves occurred. Radiologic assessment demonstrated the origin of these tumors. Eventual tumor involvement of the sole functioning cochlea resulted in the development of total hearing loss. Management entailed symptomatic care and surgical resection. Auditory rehabilitation was attempted using cochlear implantation, but results have not been satisfactory. Genetic screening identified a mutation in the NF2 gene. It is proposed that this patient's condition should be considered a variant of neurofibromatosis 2 and that bilateral facial neuromas should be included in the clinical criteria for this condition.- - - - - - - - - - ranking = 0.090377628912353keywords = canal (Clic here for more details about this article) |
4/38. Neurofibromatosis type 2 with multiple primary brain tumors in monozygotic twins.BACKGROUND: Although monozygotic twins with neurofibromatosis complicated by brain tumors rarely have been reported, none of them fulfilled the diagnostic criteria for neurofibromatosis type 2 (NF2). METHOD: We describe here the first pair of monozygotic twins with NF2, and the result of the molecular analysis of their NF2 gene. RESULTS: One of the brothers (Case 1) developed tetraparesis and cerebellar truncal ataxia at age 12. He had no skin lesions. Radiological examinations revealed, at one time or another, bilateral vestibular schwannomas, a foramen magnum meningioma, five supratentorial meningiomas, and multiple spinal cord tumors. He underwent three operations over a 10-year period to remove tumors. The patient is now 23 years old and is in college. Although asymptomatic when examined at age 12, CT scan revealed that his brother (Case 2) also had multiple brain tumors, including meningiomas, schwannomas, and multiple spinal tumors. Tumors were removed in eight operations over a 10-year period. The patient is now deaf and confined to a wheelchair. An identical nonsense mutation caused by a C to T transition (C169) in a CpG dinucleotide of the NF2 gene was identified in both patients. CONCLUSION: These results led us to speculate that dissimilarities with respect to time of appearance, distribution, and extent of symptoms and tumors between the twins were dependent on the influence of other genetic factors.- - - - - - - - - - ranking = 2keywords = spinal (Clic here for more details about this article) |
5/38. association of lower cranial nerve schwannoma with spinal ependymoma in ? NF2.A 15 year old male, who had earlier been operated for intraspinal intramedullary ependymoma, subsequently developed a right cerebello pontine (CP) angle mass. A diagnosis of right CP angle ependymoma was considered, in view of established histology of previously operated spinal lesion. Histopathological examination of the well defined extra-axial mass, which was attached with ninth cranial nerve, however revealed a schwannoma. A diagnosis of Neurofibromatosis-2 (NF2) is strongly suspected, because of well established fact, that the spinal ependymomas may have association with lower cranial nerve schwannomas in NF2. Cranial and spinal MRI screening for early diagnosis of associated, asymptomatic lesions, in suspected cases of NF2, particularly in children, is recommended.- - - - - - - - - - ranking = 8keywords = spinal (Clic here for more details about this article) |
6/38. Intracanalicular schwannoma of the facial nerve: a manifestation of neurofibromatosis type 2.Primary facial nerve tumors, which are relatively uncommon, can present a diagnostic dilemma based on their location and variable pattern of symptoms. Of primary cranial nerve tumors, schwannomas of the facial nerve rank third in frequency after those of the eighth and fifth cranial nerves. We report an illustrative case of an intracanalicular schwannoma associated with several central nervous system tumors, consistent with neurofibromatosis type 2. Initially assumed to be an eighth cranial nerve tumor, the schwannoma was found intraoperatively to arise from the facial nerve. early diagnosis and treatment enabled excision of the tumor without sacrifice of the facial nerve. facial nerve schwannomas can resemble acoustic schwannomas in their clinical presentation. Only a heightened level of clinical vigilance will point to the correct diagnosis and result in an optimal therapeutic outcome for patients with these rare tumors.- - - - - - - - - - ranking = 0.45188814456176keywords = canal (Clic here for more details about this article) |
7/38. Focal amyotrophy in neurofibromatosis 2.Neurofibromatosis type 2 (NF2) is an autosomal dominant disorder characterised by bilateral vestibular schwannomas and other CNS tumours including meningiomas and spinal schwannomas. Occasionally, peripheral neuropathy occurs in these patients but this is the first report of focal amyotrophy. Clinical, electrophysiological, and imaging data from four NF2 patients seen at a specialist neurofibromatosis clinic over a 4 year period are described in whom symptomatic focal amyotrophy preceded the diagnosis of NF2. Two presented with wasting and weakness of a single muscle group, several years before NF2 was diagnosed. In one patient a mononeuritis multiplex was the presenting feature of NF2, and in one patient focal wasting and weakness developed after the diagnosis of NF2 was made. In none of the four cases could a focal peripheral nerve or root neurofibroma be identified despite extensive imaging with MRI, and the limitations of neuroimaging for identifying a structural cause in patients with NF2 with a focal peripheral nerve lesion is discussed. It is likely that NF2 may affect peripheral nerve structures in a manner distinct from a compressive schwannoma.- - - - - - - - - - ranking = 1keywords = spinal (Clic here for more details about this article) |
8/38. Tanycytic ependymoma in association with neurofibromatosis type 2.A rare case of tanycytic ependymoma associated with neurofibromatosis type 2 (NF2) is presented for the first time, with emphasis on its clinical course and histopathological features. A 30-year-old man had developed gait disturbance in his childhood, and harbored multiple tumors in spinal nerve roots, in the intradural extramedullary and intramedullary spinal cord. The spinal root tumor and intradural extramedullary tumor were histologically diagnosed as schwannoma and meningioma, respectively. magnetic resonance imaging showed two intramedullary cystic lesions, one in the cervical and the other in the thoracic spine. Because his sensorimotor dysfunction in the lower extremities continued to worsen gradually, three of the multiple nodular tumors in the thoracic cystic lesion were removed. All three tumors were composed of eosinophilic piloid cells with modest nuclear pleomorphism. No Rosenthal fibers were found. A concentration of slender eosinophilic cellular processes surrounding the vascular wall was seen. periodic acid Schiff and Masson trichrome-positive balloons were seen in the extracellular space. Detection of ependymal rosettes, although only few in number, led the diagnosis as a tanycytic ependymoma. Recognition of this ependymoma variant should be emphasized to avoid confusion with pilocytic astrocytoma or intramedullary schwannoma.- - - - - - - - - - ranking = 3keywords = spinal (Clic here for more details about this article) |
9/38. Neurofibromatosis type 2--the importance of serial imaging of the brain and spinal cord.Meningiomas and schwannomas are relatively common central nervous system neoplasms, but patients harbouring multiple meningiomas or schwannomas are rare. We present the case of a 27 year-old patient with Neurofibromatosis type 2 with multiple associated intracranial meningiomas and spinal cord neurofibromas at various levels. patients with Neurofibromatosis type 2 should be followed up for life with serial magnetic resonance imaging of the brain and spinal cord to detect new and recurrent tumours at these sites.- - - - - - - - - - ranking = 6keywords = spinal (Clic here for more details about this article) |
10/38. Unusual case of multiple cellular and malignant schwannomas of the cranial and spinal nerves.Schwannomas from cranial nerves and spinal roots are most often benign, malignant schwannomas being uncommon in this location. This report describes a unique case of multiple cellular and malignant schwannomas of the cranial and spinal nerves in a patient with features of neurofibromatosis 2. The tumors were arising from left optic, bilateral oculomotor, trochlear, abducent and vestibular nerves, the left facial and the spinal lumbar nerve roots. The tumor arising from the right trochlear nerve was seen excavating into the hippocampus and the left vestibular nerve into the medulla. In addition, the patient had nodular schwannomas adherent to the inferior surface of the optic chiasm and the cerebellum away from the cranial nerves. Hyperplastic schwannosis was noted in perivascular spaces of thalamus, cerebellum and hippocampus on both sides away from the main lesions. The immunohistochemical and ultrastructural profiles of the tumors suggest that neurofibromas and schwannomas are probably not distinct tumors but lie within a spectrum that differs histologically depending on the predominant cell type.- - - - - - - - - - ranking = 7keywords = spinal (Clic here for more details about this article) |
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