Diffuse scalp malignant peripheral nerve sheath tumor with intracranial extension in a patient with neurofibromatosis type 1.
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 22cm×18cm, 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.
Ge
Pengfei
P
Department of Neurosurgery, First Affiliated Hospital of Jilin University, 71 Xinmin Street, Changchun 130021, China.
Fu
Shuanglin
S
Lu
Laijin
L
Zhong
Yanping
Y
Qi
Bin
B
Luo
Yinan
Y
D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness / Q000175:diagnosis / Q000473:pathology*; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000175:diagnosis; D019635:Neurosurgical Procedures / Q000379:methods; D010525:Peripheral Nerves / Q000473:pathology*; D010524:Peripheral Nervous System Neoplasms / Q000175:diagnosis / Q000473:pathology*; D019651:Plastic Surgery Procedures / Q000379:methods; D012535:Scalp / Q000294:innervation / Q000473:pathology*; D012886:Skull / Q000473:pathology*; D013524:Surgical Flaps; D014057:Tomography, X-Ray Computed / Q000379:methods; D016896:Treatment Outcome