A rare case of intramedullary ‘whorling-sclerosing’ variant meningioma

A 52-year-old man with a seven-year history of progressive weakness, gait problems, and pain in his extremities presented with subacute worsening of his symptoms. Examination revealed weakness in all four extremities, increased tone, hyperreflexia, and sensory deficits. MRI of the cervical spine showed an area of signal abnormality and abnormal enhancement within the cervical cord at the C5-C6 level. The patient initially underwent biopsy followed a few days later by a debulking surgery. Postoperatively, the patient showed improvement in strength as well as ambulation. Intraoperatively, the lesion was confirmed to be intramedullary without any dural attachments. Histopathological examination revealed an extensively hyalinized tumor with sparse collections of cells that were immunopositive for both cytokeratin and GFAP, and immunonegative for EMA and progesterone receptor. This is an unusual pattern of expression, with cytokeratin immunopositivity suggesting a meningioma and GFAP immunopositivity suggesting a glioma. Considering the combination of extensive hyalinization with cytokeratin positivity the tumor was thought to be most consistent with a hyalinized meningioma with GFAP positivity. GFAP-positive meningiomas are rare, and these include the recently described 'whorling-sclerosing' variant. Only three cases of this tumor have been previously reported, all of which were intracranial. This is the first reported case of an intramedullary whorling-sclerosing meningioma.