Cases reported "Cranial Nerve Neoplasms"

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1/137. Pediatric eighth cranial nerve schwannoma without evidence of neurofibromatosis.

    Schwannomas of the eighth cranial nerve are rare in children. We report a 4 10/12 - year-old girl with no evidence of neurofibromatosis who presented with facial droop. Radiographic studies revealed a large cerebellopontine angle tumor. At surgery, the tumor was attached to the eighth cranial nerve and histologically was a schwannoma. This is the youngest reported case of unilateral eighth cranial nerve schwannoma in a patient without the stigmata of neurofibromatosis.
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keywords = neurofibroma
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2/137. Spontaneous remission of chiasmatic/hypothalamic masses in neurofibromatosis type 1: report of two cases.

    We report two children with neurofibromatosis type 1 showing enhancing masses on MRI suggesting neoplasms in the chiasm and hypothalamic region. In both patients no visual or endocrinal dysfunction was present. On serial MRI spontaneous partial remission was found, implying that a cautious approach to therapeutic management of similar cases should be taken.
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ranking = 0.83333333333333
keywords = neurofibroma
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3/137. 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.
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ranking = 0.16666666666667
keywords = neurofibroma
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4/137. Dumbbell-shaped trigeminal neurinoma in a child.

    We experienced a case of solitary trigeminal neurinoma in a 10-year-old boy without stigmata of neurofibromatosis. This boy presented with progressive cerebellar ataxia, right abducens palsy and right hemiparesis. Magnetic resonance images (MRI) demonstrated a tumour extending into both the middle and posterior fossas forming a dumbbell-shaped mass lesion with a size of 35 x 30 x 45 mm. The tumour was successfully removed free of complications by a two-stage operation composed of right subtemporal and suboccipital retromastoid approaches. It is extremely rare for a solitary neurinoma to occur at this site in a child without neurofibromatosis. Surgical approaches in this type of tumour are discussed with a review of the literature.
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ranking = 0.33333333333333
keywords = neurofibroma
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5/137. Thoracoscopic resection of intrathoracic vagus neurofibroma.

    A thoracoscopic resection of mediastinal neurofibroma originating from the intrathoracic vagus nerve was successfully performed in an 18-year-old female. This report reviews the clinical and morphological features of intrathoracic vagus neurofibroma. Often neurofibroma has multiple lesions and/or multinodular shape. Thoracoscopic resection is a useful treatment for patients with intrathoracic neurofibroma, although careful observation is necessary when performing a radical resection.
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ranking = 1.3333333333333
keywords = neurofibroma
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6/137. Spontaneous partial regression of low-grade glioma in children with neurofibromatosis-1: a real possibility.

    At the age of 41 and 31 months, respectively, a boy and a girl affected by neurofibromatosis-1 were diagnosed with a visual pathway glioma during surveillance contrast-enhanced head magnetic resonance imaging (MRI). In the first child, the initial MRI showed that the entire optic chiasm, the intracranial tract of the left optic nerve, and hypothalamus were grossly enlarged and enhanced in the post-gadolinium T1-weighted images. Ten months later, the hypothalamic component of the lesion had regressed markedly and there were no more areas of contrast enhancement. In the second child, the initial MRI showed that the optic chiasm, the right optic tract, and geniculate body were enlarged and enhanced after gadolinium injection. At 6-month follow-up, the MRI showed that the right optic tract and the anterior aspect of the optic chiasm decreased in size and the contrast enhancement of the entire lesion was reduced dramatically. These findings, as indicated by other similar reports, confirm that spontaneous regression of visual pathway glioma is a rare but real possibility in children with neurofibromatosis-1. Therefore, clinicians need to be aware of visual pathway glioma's erratic behavior in children with neurofibromatosis-1 with special attention given to the importance of a very conservative attitude toward any type of treatment for such patients.
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ranking = 1.1666666666667
keywords = neurofibroma
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7/137. Isolated metastases of adenocarcinoma in the bilateral internal auditory meatuses mimicking neurofibromatosis type 2--case report.

    A 56-year-old male with a history of lung cancer presented with isolated metastases of adenocarcinoma in the bilateral internal auditory meatuses (IAMs), mimicking the bilateral acoustic schwannomas of neurofibromatosis type 2, and manifesting as rapidly worsening tinnitus and bilateral hearing loss. magnetic resonance imaging showed small tumors in both IAMs with no sign of leptomeningeal metastasis. The preoperative diagnosis was neurofibromatosis type 2. Both tumors were removed and the histological diagnoses were adenocarcinoma. neuroimaging differentiation of a solitary metastatic IAM tumor from a benign tumor is difficult, although rapidly progressive eighth cranial nerve dysfunction suggests a malignant process. Metastases should be considered as a rare diagnostic possibility in a patient with small tumors in both IAMs.
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ranking = 1
keywords = neurofibroma
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8/137. Benign glandular peripheral nerve sheath tumor of the seventh and eighth cranial nerve complex.

    A unique, deep-seated, benign, glandular peripheral nerve sheath tumor (PNST) of the cerebellopontine angle is described. The tumor arose from the seventh and eighth cranial nerve complex in a 15-year-old boy without neurofibromatosis. Histologically, benign glands were embedded in a bland spindle cell stroma. The epithelial cells were immunoreactive for CAM 5.2, and focally for chromogranin. The spindle cells were positive for S100P. The benign glandular PNST unassociated with neurofibromatosis is a controversial entity. The superficial location of most reported cases has made it difficult to exclude entrapped adnexae as a source for the glands. This tumor was separate from the internal auditory meatus on MRI scan, the most likely source of entrapped glands at this site. This case is the first report of a deep-seated, benign, glandular peripheral nerve sheath tumor. It suggests that glandular differentiation in PNSTs, while unusual, is not synonymous with neurofibromatosis or malignancy.
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ranking = 0.5
keywords = neurofibroma
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9/137. 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.
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ranking = 0.83333333333333
keywords = neurofibroma
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10/137. 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.
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ranking = 0.33333333333333
keywords = neurofibroma
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