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1/9. 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 = 1
keywords = complex
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2/9. A case of multiple schwannomas of the trigeminal nerves, acoustic nerves, lower cranial nerves, brachial plexuses and spinal canal: schwannomatosis or neurofibromatosis?

    In most cases, while schwannoma is sporadically manifested as a single benign neoplasm, the presence of multiple schwannomas in one patient is usually indicative of neurofibromatosis 2. However, several recent reports have suggested that schwannomatosis itself may also be a distinct clinical entity. This study examines an extremely rare case of probable schwannomatosis associated with intracranial, intraspinal and peripheral involvements. A 63-year-old woman presented with a seven-year history of palpable lumps on both sides of the supraclavicular area and hearing impairment in both ears. On physical examination, no skin manifestations were evident. Facial sensory change, deafness in the left ear and decreased gag reflex were revealed by neurological examination. magnetic resonance imaging revealed multiple lesions of the trigeminal nerves, acoustic nerves, lower cranial nerves, spinal accessory nerve, brachial plexuses, and spinal nerves. Pathological examination of tumors from the bilateral brachial plexuses, the spinal nerve in the T8 spinal position and the neck mass revealed benign schwannomas. Following is this patient case report of multiple schwannomas presenting with no skin manifestations of neurofibromatosis.
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ranking = 1.3990026575891
keywords = neoplasm
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3/9. MRI findings of vestibulocochlear hemorrhage in a leukemic patient with sensorineural hearing loss.

    This case report presents a patient with M4 leukemia with signs and symptoms of acute sensorineural hearing loss. The patient's MRI demonstrated high signal on unenhanced T1-weighted images within the left vestibulocochlear complex that was consistent with subacute hemorrhage. Follow-up MRI showed clearing of the previously seen high T1-weighted signal from the left vestibulocochlear complex. This case report documents for the first time the MRI findings of vestibulocochlear complex hemorrhage in a leukemic patient.
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ranking = 0.6
keywords = complex
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4/9. Direct infiltration of brainstem glioma along the cranial nerves.

    The authors describe a case of a low-grade brainstem glioma extending along the cranial nerves without any evidence of leptomeningeal spread. The tumor extended directly along the VII-VIIIth cranial nerve complex and also along the trigeminal nerve, which is quite an unusual characteristic of the glial tumors.
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ranking = 0.2
keywords = complex
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5/9. Melanotic schwannoma of the acoustic nerve.

    Melanotic schwannoma is a rare neoplasm, with only 23 previously reported cases. We report an additional case of a melanotic schwannoma arising from the acoustic nerve in a 74-year-old man who had a probable forme fruste of neurofibromatosis. We briefly review the literature of this neoplasm and discuss theories regarding the histogenesis of these tumors.
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ranking = 2.7980053151781
keywords = neoplasm
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6/9. Psychoacoustic and electrophysiologic effects of partial eighth nerve damage.

    The authors present psychoacoustic and electrophysiologic data concerning a patient with partial damage to the auditory nerve, presumably from a dilated arterial vessel. The lesion was described and documented during neurosurgery for vestibular nerve decompression. Pure tone thresholds, psychoacoustical tuning curves, speech reception ability for spondaic words, maximum word recognition performance for monosyllabic NU-6 word lists, and performance on the synthetic sentence identification test in the ipsilateral and contralateral competing message modalities were normal. Findings consistent with eighth nerve dysfunction, absent contralateral acoustic reflexes, absent or unrecognizable early evoked potentials occurring in the first 10 msec, brainstem auditory evoked potentials (BAEPs), and slight rollover of the performance intensity function for monosyllabic words were obtained on the involved side. In spite of the grossly abnormal BAEPs, two late, presumably cortical, auditory evoked potential components, which occur in the 60 to 250-msec range--vertex potential and T complex--were present and normal. The findings indicate that the perceptual processes needed for speech reception and recognition and for the appearance of later, presumably cortical-evoked potentials can be preserved despite partial damage to the auditory nerve.
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ranking = 0.2
keywords = complex
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7/9. "Onion bulb" formation associated with a solitary neoplasm of the eighth nerve sheath.

    Audiometric patterns associated with retrocochlear disorders include abnormal adaptation, delay of auditory evoked potential latencies, and characteristic abnormalities of speech intelligibility functions. The anatomic substrate of these psychoacoustic and electrophysiologic abnormalities is unknown. This paper describes the incidental observation of "onion bulbs" in a solitary neoplasm involving the eighth nerve sheath and in the cochlear nerve lateral to the neoplasm. Such hypertrophic neuropathy in the eighth nerve has not been described previously. This occurrence raises the question of sequential demyelination and remyelination in a neoplasm-bearing nerve as a possible histopathologic correlate to retrocochlear dysfunction.
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ranking = 9.7930186031234
keywords = neoplasm
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8/9. glioma of the acoustic nerve.

    We report a case of astrocytoma of the acoustic nerve. Most gliomas arise from the brainstem, and seldom originate in the acoustic or other "true" cranial and spinal nerves. Clinical features of this rare acoustic tumor differ from those of brainstem gliomas, but are indistinguishable from typical acoustic neurilemoma. We discuss the diagnosis and histogenesis of glioma arising in the eighth cranial nerve. Demonstration of glial fibrillary acid protein, an antigen specific for astrocytes, is a new method of verifying the diagnosis. review of the literature indicates that a few cases of epithelial-like tumors of peripheral nerves may have been of neuroepithelial origin. The evidence, however, generally is not sufficient to exclude the possibility of metastatic neoplasms or other tumors such as malignant schwannoma and melanoma. Most of these putative gliomas contained gland-like tissue, and did not have the morphologic appearance of astrocytoma, as in approximately five reported examples, and in our case.
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ranking = 1.3990026575891
keywords = neoplasm
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9/9. Solitary primary lymphoma of the cerebellopontine angle: case report.

    A 27-year-old woman had a solitary primary B-cell lymphoma that involved the right cerebellopontine angle. The tumor invaded the ipsilateral acoustic nerve and produced profound sensory neural hearing loss. Surgical resection was promptly followed by radiotherapy and a concluding brief chemotherapy treatment. The patient survived 34 months after the initial onset of the disease or 22 months after the combined treatments. Only six lymphomas affecting the cerebellopontine angle, three primary and three secondary, have been recorded. The combination of surgical treatment and radiotherapy appeared to offer encouraging results for primary lymphomas. The differential diagnosis of lymphoma of the cerebellopontine angle includes common acoustic neurilemoma, meningioma, epidermoid tumor, and other rare neoplasms involving this region that required pathological verification to distinguish them.
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ranking = 1.3990026575891
keywords = neoplasm
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