Diffuse scalp malignant peripheral nerve sheath tumor with intracranial extension in a patient with neurofibromatosis type 1

作者：Ge Pengfei; Fu Shuanglin; Lu Laijin; Zhong Yanping; Qi Bin; Luo Yinan^*

来源：Journal of Clinical Neuroscience, 2010, 17(11): 1443-1444.

DOI：10.1016/j.jocn.2010.01.055

• Malignant peripheral nerve sheath tumor
• Neurofibromatosis Type 1
• Scalp tumor

We describe a rare scalp malignant peripheral nerve sheath tumor (MPNST) with cranial destruction and intracranial extension in a 52-year-old male with neurofibromatosis type 1 (NF1). The scalp tumor measured 22 cm x 18 cm, with local surface ulceration. Skin examination revealed many cafe-au-lait spots and small, hard dermal nodules on the trunk. CT scans revealed the scalp tumor to have heterogeneous density with partial destruction of the right parietal cranium; on T1-weighted MRI the scalp tumor displayed heterogeneous hypointensity, whereas on T2-weighted and fluid-attenuated inversion recovery MRI it was hyperintense. The tumor was excised totally and the scalp reconstructed using a skin flap isolated from the lateral aspect of the left thigh. Histological examination confirmed that the tumor was an MPNST. The patient recovered uneventfully and was well at the 6-month follow-up, with no local or other tumor recurrence noted.

• 出版日期2010-11
• 单位吉林大学