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

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• 作者：Pengfei Ge ; Shuanglin Fu ; Laijin Lu ; Yanping Zhong ; Bin Qi ; Yinan Luo
• 关键词：Malignant peripheral nerve sheath tumor ; Neurofibromatosis Type 1 ; Scalp tumor
• 刊名：Journal of Clinical Neuroscience
• 出版年：2010
• 出版时间：November 2010
• 年：2010
• 卷：17
• 期：11
• 页码：1443-1444
• 全文大小：259 K

文摘

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 × 18 cm, with local surface ulceration. Skin examination revealed many café-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.