Malignant peripheral nerve sheath tumor of the trigeminal nerve: Clinicopathologic features in a young adult patient

Abstract

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 Meckel'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 Smarcb1. 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.

Malignant Peripheral Nerve Sheath Tumor of the Cerebellar Hemisphere: An Unusual Location and Multiple Intracranial Parenchyma Metastases

“…The main manifestations of MRI are mixed-signal intensity, irregular contour with necrosis, compression of adjacent brain tissue by the lesions, significant local invasion, and signal enhancement in almost all cases. A computed tomography scan may show tumor invasion of surrounding bone [11]. Chen L et al reported two MRI scans of MPNST in the cerebellopontine angle area: low signal on T1WI, mixed high signal on T2WI, and significant inhomogeneous enhancement [12].…”

“Gliomatosis encephali” as a novel category of brain tumors by the first autopsy case report of gliomatosis cerebelli

“…The designs of the primers were set to detect TP53 hotspot mutations (R175, G245, R248, R273 and R306). The PCR products were then sequenced on a 3130xl Genetic Analyzer (Applied Biosystems, Foster City, CA, USA) with a Big Dye Terminator v1.1 Cycle Sequencing Kit (Applied Biosystems) in accordance with the manufacturer's instructions …”

“…The PCR products were then sequenced on a 3130xl Genetic Analyzer (Applied Biosystems, Foster City, CA, USA) with a Big Dye Terminator v1.1 Cycle Sequencing Kit (Applied Biosystems) in accordance with the manufacturer's instructions. 6 Immunohistochemical staining was performed by the avidin-biotin-peroxidase complex (ABC) method (Vectastain ABC Elite kit, Vector, Burlingame, CA, USA). Non-specific binding of the biotin-avidin system reagents was blocked by pretreating the sections with 0.3% hydrogen peroxide in methanol and a normal blocking serum, and then incubating them with the required primary antibody overnight (for 17 h) at 4°C.…”

Nuances in the Treatment of Malignant Tumors of the Clival and Petroclival Region

“…Malignant nerve sheath tumors (MNSTs) are rare entities that usually affect adult patients in the third to sixth decade of life, although children as young as 5 years have also harbored these tumors. 31 Incidence in the overall population is 0.001% and intracranial lesions are even less common. 31 Although these lesions are found in both neurofibromatosis types (NF1 and NF2), they are most often found in von Recklinghausen disease (NF1).…”

“… 31 Incidence in the overall population is 0.001% and intracranial lesions are even less common. 31 Although these lesions are found in both neurofibromatosis types (NF1 and NF2), they are most often found in von Recklinghausen disease (NF1). 31 32 33 …”