PMC3928348_01
A : Operative scar on her back and cafe-au-lait spot (arrow). B : Operative scar on her left thigh and widespread cafe-au-lait spots (arrow). C : Widespread frecklings axillary. D : Cutaneous neurofibromas and plexiform neurofibroma (arrow). E : Patient's pedigree. The circles represent females and squares represent males. Black represents affected individuals. Oblique line represents dead individuals. The patient is signed in arrow, her mother and maternal grandmother have similar manifestations of neurofibromatosis 1.
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F1
Among the family members her mother and maternal grandmother had similar manifestations of NF1, but her daughter and sons had no clinical evidence of NF1 (Fig. 1).
A : Spinal MRI shows a relative-demarcated, heterogeneously enhanced mass lesion accompanied by perifocal edema in C5-7 level (arrow). B : A left-sided T11 spinous process heterogeneously enhanced mass in soft tissue (thick arrow), and intervertebral disk hernia in L2-5 level (thin arrows). C, E, F, and G : Post-gadolinium T1 weighted sagittal imaging revealed widespread punctum enhancing lesion in her scalp and in T11-L5 level. D : Follow-up MRI revealed stable postsurgical changes in the C4-7 level with no evidence of tumor recurrence one year later. H, I, and J : Head MRI suggests no evidences of optic pathway glioma and neurofibromatosis 2.
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F2
No evidences of optic pathway glioma and neurofibromatosis 2 (NF2) had been found in head MRI (Fig. 2). Follow-up magnetic resonance imaging revealed stable postsurgical changes in the C5-C7 level with no evidence of tumor recurrence and dissemination (Fig. 2).
A : A yellowish mass with abundance blood supply in the spinal cord is exposed. B : The spinal cord is slit and the tumor was gross-totally resected safely under an operative microscope.
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F3
Under an operative microscope, the tumor was gross-totally resected safely, and a laminoplasty from C5-7 was performed at the end of the surgery (Fig. 3).
A : In the H&E staining, the cyst wall consists of glial cells characterized by round, irregular nucei and eosinophilic cytoplasm, and perivascular pseudorosettes formation can been noted. B, C and D : The immunohistochemical examination of the cells is positive for glial fibrillary acidic protein (B), epithelial membrane antigen (C), and S-100 protein (D). These findings are consistent with an ependymal cyst diagnosis. E : Biopsy of a scalp neurofibroma, a typical cutaneous neurofibroma without malignant features.
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F4
The biopsy of a scalp neurofibroma showed a typical cutaneous neurofibroma (Fig. 4).