Central Neurofibromatosis

NF-2 is an extremely rare form of neurofibromatosis (NF) characterized by central system (CNS) neural crest-derived tumors and frequently cafe au lait spots (CLS). The purpose of this study was to report the clinical and imaging findings of seven patients with this disorder and to stress that value of surgical treatment in its management. Seven patients between 8 and 32 years of age who had NF-2 were included in the study. Clinical charts, surgical and pathological findings, and imaging studies were reviewed retrospectively. Patients were followed up for to 142 months. Clinical evaluation and neuroimaging studies detected the clinical criteria of NF-2 in all patients. Two deaths occurred after surgical intervention in our series. None of the patients suffered from recurrent tumor following surgery. Our results show that NF-2 is an uncommon entity which has a good prognosis after surgical intervention, in spite of the presence of multiple cranial and/or spinal lesions.

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Multiple meningiomas of the central nervous system without the stigmata of neurofibromatosis. Clinical and therapeutic study

Turğut

Palaoğlu

Özcan

et al. 1997

Neurosurg. Rev.

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Multiple meningiomas are relatively rare tumors without known neurofibromatosis. In this paper, such eight cases of multiple meningiomas as described by CUSHING and EISENHARDT are presented. Certain aspects of diagnosis and surgical management of this rare condition are discussed with particular emphasis on the importance of the distinction among multiple meningioma, meningiomatosis, or recurrences of these tumors. Fortunately, many of these patients tolerate multiple surgical interventions well, although the removal of these tumors in critical areas is a difficult problem. Thus, we think that it is important to examine and supervise all patients who have had a meningioma for a possible occurrence of a second meningioma.

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Central Neurofibromatosis

Cited by 4 publications

References 12 publications

Neurocutaneous Syndromes and Associated CNS Tumors

Neurocutaneous Syndromes and Associated CNS Tumors

The neurosurgical aspects of neurofibromatosis 2: diagnosis and management

Multiple meningiomas of the central nervous system without the stigmata of neurofibromatosis. Clinical and therapeutic study

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