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Malignant peripheral nerve sheath tumor of the trigeminal nerve: Clinicopathologic features in a young adult patient
Author(s) -
Nakayama Yoko,
Watanabe Masatoshi,
Suzuki Kenji,
Usuda Hiroyuki,
Emura Iwao,
Ogura Ryosuke,
Shiga Atsushi,
Toyoshima Yasuko,
Takahashi Hitoshi,
Kawaguchi Tadashi,
Kakita Akiyoshi
Publication year - 2013
Publication title -
neuropathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.701
H-Index - 61
eISSN - 1440-1789
pISSN - 0919-6544
DOI - 10.1111/neup.12004
Subject(s) - medicine , trigeminal nerve , schwannoma , pathology , malignant peripheral nerve sheath tumor , neurofibromatosis , anatomy
Malignant peripheral nerve sheath tumors ( MPNSTs ) arising from cranial nerves are rare and usually affect adults. Here we report the clinicopathologic features of a young adult patient with a trigeminal nerve MPNST , in whom another tumor involving the oculomotor nerve on the contralateral side was evident. The patient, an 18‐year‐old woman, had suffered recurrent paroxysmal sharp stabbing pain over her cheek and forehead on the right side for 1 month. A brain MRI study disclosed a mass, 35 mm in diameter, in the right M eckel's cave, and another mass, 10 mm in diameter, involving the intracranial portion of the left oculomotor nerve. Following gadolinium administration, the former and latter tumors exhibited strong and weak enhancement, respectively. The patient had no clinical stigmata characteristic of neurofibromatosis type 1. Following a tentative diagnosis of schwannoma, total resection of the trigeminal nerve tumor was performed. Histologically, the tumor consisted of highly cellular, spindle‐shaped cells arranged in a fascicular pattern, with occasional mitotic figures, nuclear pleomorphism and necrosis. Immunohistochemically, the tumor cells showed variable intensities and frequencies of reactivity for S ‐100 protein, myelin basic protein, CD34 , podoplanin and p53, but no reactivity for S marcb1. Thus, the tumor exhibited features of MPNST . This case appears to provide information that is useful for accurate diagnosis and surgical planning in patients with bilateral or multiple cranial nerve tumors.