NF-2 is an autosomal dominant hereditary disorder associated with the development of multiple nervous system tumors. Meningiomas in this aggressive or diffuse group of tumors are poorly treated at present. Focal lesions may be removed surgically or treated with stereotactic radiation. However, diffuse tumor growth may invade bone, skull base, or venous sinuses, and in the case of malignant meningioma, may metastasize outside the central nervous system. At present, neither radiotherapy nor chemotherapy is effective for all of these tumors and other modalities are being investigated.
Spinal meningiomas are intradural extramedullary masses.
They arise from mesothelial cells rests found where the arachnoid joins the dura of the nerve root sheath. As they progress, these tumors produce signs of spinal cord compression. Their highest distribution in the spinal canal is thoracic, followed by cervical and rarely, lumbar-sacral region. Meningiomas have been examined from a molecular and cellular perspective. Estrogen, progesterone, androgen, corticosteroid and somatostatin binding sites are present in meningioma cells, but their growth stimulatory effects are unclear.