Cases reported "Neurofibromatoses"

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1/12. 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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2/12. Malignant schwannoma and late-onset form of neurofibromatosis (NF-VII type) in a patient with skeletal manifestations.

    INTRODUCTION: Von Recklinghausen's neurofibromatosis is known to occur with markedly variable expressivity. Nevertheless, cases that do not feature characteristic findings are uncommon. CASE: We report an extremely rare, sporadic case of neurofibromatosis, exclusively represented in the skeleton of a 49-year-old woman. The late onset of the disease and the absence of common diagnostic criteria permit us to classify it as neurofibromatosis type VII. Additionally, the disease was complicated by early malignant transformation of a thoracic neurinoma, which was removed by a wide local resection. Two years after surgery, the patient developed local recurrence and liver metastases. She died a few months later despite aggressive chemotherapy.
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3/12. 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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4/12. Treatment of a large high-grade neurofibrosarcoma with concomitant vinblastine, doxorubicin, and radiotherapy.

    A patient with neurofibromatosis developed a large inoperable malignant schwannoma on the posterior neck. The tumor underwent complete local regression following combined-modality treatment with radiotherapy, vinblastine, and doxorubicin. vinblastine may be effective in combined-modality therapy.
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5/12. Malignant schwannoma of the liver in a patient without neurofibromatosis: a case report and review of the literature.

    Primary schwannomas of the liver are extremely rare. We report a case of malignant schwannoma of the liver occurring in a 49-year-old man, who did not have neurofibromatosis, and review the literature. The clinical and histologic findings of benign and malignant schwannomas of the liver are compared.
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6/12. Multiple recurrent benign schwannomas of deep and superficial nerves of the upper extremity: a new variant of segmental neurofibromatosis.

    Benign schwannomas of the brachial plexus are uncommon tumors, first described in the late 19th century. These lesions, which are histologically benign, can generally be excised without sacrifice of neural elements. We present the first known case of multiple concurrent and recurrent benign schwannomas of the upper extremity in an individual who demonstrated no other evidence of neurofibromatosis, and we suggest that this case may represent a new subtype of type V neurofibromatosis.
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7/12. Acoustic neuroma in an adolescent without neurofibromatosis: case study.

    When acoustic neuromas are found in younger age groups, they are typically bilateral and associated with neurofibromatosis (NF). A unilateral acoustic neuroma in a child or adolescent without NF is rare. We report audiometric, auditory brainstem response (ABR) and magnetic resonance imaging (MRI) data for a 15-year-old male with an acoustic neuroma but without associated NF. The patient was seen for audiologic assessment on three separate occasions over a period of 5 years. The first two assessments produced unremarkable audiometric and immittance data. The third assessment, subsequent to a failed school hearing screening, demonstrated a flat unilateral sensorineural hearing loss, unilateral absent acoustic reflexes, and abnormal bilateral ABR recordings. MRI with contrast demonstrated a unilateral mass extending out of the internal auditory meatus. Microscopic examination of the removed tumor confirmed a vestibular schwannoma. An enhanced MRI 3 years post neuroma removal demonstrated no evidence of tumor regrowth and no evidence of other neoplasms. ABR recordings for the uninvolved ear continued to demonstrate neural synchrony as evidenced by normal absolute wave latencies; however, the III-V and I-V interwave latencies remained extended beyond the 99th percentile. Postoperatively, the young man was fitted with a high gain in-the-ear hearing aid in the involved ear, which he has continued to wear on a daily basis for the past 3 years.
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8/12. Neurofibromatosis and malignant schwannomas in tanzania.

    In a five-year-period, twenty three cases of peripheral nerve tumours were seen at the pathology department of Muhimbili Medical Centre. Nine of these had von-Recklinghausen's disease (neurofibromatosis). One of these patients developed malignant schwannomas at two different sites simultaneously. patients with malignant disease in tanzania as in other sub-Saharan African countries usually get to the large referral hospitals late in the course of their disease. Despite this delay and the advanced nature of their illness, attempts should be made to achieve satisfactory palliative results.
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9/12. Malignant schwannoma of the parapharyngeal space in von Recklinghausen's disease: a case report and review of the literature.

    Neurofibromatosis (NF) or von Recklinghausen's disease is frequently accompanied by malignant tumours, which can occur at any site in the body. These malignancies are mainly of soft tissue origin. Of all head and neck malignancies, the number of soft tissue sarcomas is limited and in combination with NF this type of tumour is a rare event. In this report we describe the clinical course of a young female patient with NF, who presented with a massive malignant schwannoma in the parapharyngeal space, and review the pertinent literature.
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10/12. Multiple cutaneous plexiform schwannomas. Report of a case and review of the literature with particular reference to the association with types 1 and 2 neurofibromatosis and schwannomatosis.

    Plexiform schwannomas are relatively rare, benign peripheral nerve sheath tumors, which usually arise in either the dermis or subcutaneous tissue, although rare cases originate in skeletal muscle or other deep somatic soft tissue sites. These tumors may occur singly or as multiple lesions and may be localized to one anatomic site or diffusely distributed. Rare cases have been associated with "schwannomatosis" as well as type 1 neurofibromatosis (von Recklinghausen's disease). We report an unusual case of multiple cutaneous plexiform schwannomas associated with bilateral acoustic neuromas as well as other intracranial and intraspinal neoplasms. In addition, we examine the relationship between the various forms of cutaneous schwannoma, particularly the plexiform variant, and both types 1 and 2 neurofibromatosis; we also examine several purported cases of schwannomatosis.
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