Primary ectopic intraosseous meningioma associated with multiple meningioma in a patient with central neurofibromatosis
We present a case of multiple meningiomas (MM) as initial manifestation of central neurofibromatosis (NF2). A 19 y. o. woman was admitted with increasing signs of spinal cord compression at Th5-7 level. At CT assisted myelography, an entirely extradurally situated meningioma was detected and totally removed. Two years later increasing right-sided exophthalmos, without visual impairment was observed. The CT scan revealed thickening and abnormal shape of the sphenoid and the bony structures of the orbit. The retrobulbar space was narrowed and the optic nerve was shifted. At operation, ala parva of the sphenoid bone, the roof and the lateral orbital wall appeared thickened, laminated, fragile and exceedingly vascularized. The subdural space was found intact. The histopathological study revealed primary intraosseous meningioma of the sphenoid. There was no family history for neurofibromatosis (NF). The clinical findings at this time could not fulfil the criteria for NF2 and the case was considered as "true" multiple meningiomas occurring in different compartments of the neuraxis. The cytogenetical examination demonstrated a deletion of the long arm of the chromosome 22: 46 XX, del 22 (q 11.2). She was followed up and five years after the initial symptoms asymptotic bilateral vestibular schwannomas were found at CT and MRI. These findings finally led to the diagnosis NF2. There are no specific features distinguishing true MM from those associated with NF2, but we recall the importance of such manifestations for this diagnosis. We have not found any comparable case with these features in the medical literature.