Abstract

Most previous reports on neurilemmoma of the head and neck region describe a solitary tumor. Although most reports on multiple neurilemmoma considered these lesions to belong to von Recklinghausen disease, atypical cases of von Recklinghausen disease were also included in some reports. Recently, these multiple neurogenic tumors have been classfied into neurofibromatosis type 1 and 2 (NF 1, 2). NF 1 is considered as original von Recklinghausen disease, whereas NF 2 refers to atypical von Recklinghausen disease, which has been reported along with multiple neurilemmoma, including other neurogenic tumors. It is well known that NF 2 is distinguished from NF 1 both clinically and genetically. The clinical criteria for NF 1 and NF 2 have also been described. NF 2 is often associated with meningioma and neurilemmoma. The present case had both meningioma and neurilemmoma, which occurred at multiple sites, such as the tongue, mediastinum, and brain. Considering recent reports that the gene of atypical multiple neurilemmoma may be identical to that of NF 2, we regarded the present case to be NF 2.

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