Spinal Cord Ependymoma Associated with Neurofibromatosis 1 : Case Report and Review of the Literature
A 49-year-old female patient was admitted to our department because of numbness in her fingers that progressed to her entire body above the bellybutton for half a year. In his past medical history there were expeditiously increscent cutaneous neurofibromas respectively on her back and left thigh for five years, and a total resection for tumors had been performed in a local hospital. Histological examination revealed both of them were neurofibromas with malignant features. On physical examination, widespread café-au-lait spots, axillary and groin frecklings, cutaneous neurofibromas, plexiform neurofibromas and operative scar on her back and left thigh were present, and a sensory deficit was present between the C4 level and bellybutton. No iris hamartomas had been found and mammary gland were normal. 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 ().\nMagnetic resonance imaging showed a relative-demarcated, heterogeneously enhanced mass lesion accompanied by perifocal edema in C5-7 level, a left-sided T11 spinous process heterogeneously enhanced mass in soft tissue, intervertebral disk hernia in L2-5 level and widespread punctum enhancing lesion in her scalp and in T11-L5 level. No evidences of optic pathway glioma and neurofibromatosis 2 (NF2) had been found in head MRI ().\nThe patient was positioned prone after placement of MEP and SEP monitoring devices, then a posterior laminectomy was performed from C5-7. After confirming the tumor location at the C5-7 level by ultrasonography, we incised the dura at the midline and slit the spinal cord, a yellowish mass with abundance blood supply in the spinal cord was observed. 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 ().\nHistological examination revealed it was a moderately cellular glial tumor characterized by round, irregular nucei and eosinophilic cytoplasm, and perivascular pseudorosettes formation was noted. Immunohistochemical analysis showed the tumor cells have immunoreactivity for glial fibrillary acidic protein, epithelial membrane antigen, neuron specific enolase and S-100, but were negative for reticulin. 1% of the cells were positive for monoclonal antibody to Ki-67 antigen. The findings were consistent with ependymoma, grade II in the World Health Organization classification. The biopsy of a scalp neurofibroma showed a typical cutaneous neurofibroma ().\nPostoperatively, no adjunctive therapy was given and the patient manifested normal physical development. One year after operation, the neurological disorders had recovered and no neurological deficits were examined. Follow-up magnetic resonance imaging revealed stable postsurgical changes in the C5-C7 level with no evidence of tumor recurrence and dissemination ().