Whorling-Sclerosing Variant Meningioma of the Spine: Surgical Management and Outcome of an Extremely Rare Case.

STUDY DESIGN: Case report OBJECTIVE.: To report surgical resection of multiple ventral cervical and thoracic lesion being diagnosed as whorling-sclerosing variant meningioma (WSM) and subsequent stabilization of cervicothoracic spine.

SUMMARY OF BACKGROUND DATA: WSM is a rare histopathology variant of meningioma which is mostly reported in brain and only one case of cervical spine WSM has been reported. Because of the rarity of WSM, there is a paucity of information regarding the clinical, radiological, and histopathologic characteristics.

METHODS: A 57-year-old lady presented with progressive quadriparesis and myelopathy since 10 days. The spine magnetic resonance imaging (MRI) revealed multiple ventral cervical and thoracic lesions which were hypointense in T2-weighted images with heterogeneous and scant enhancement with gadolinium. The patient underwent anterior cervical corpectomy and gross total resection of the lesion along with 360° fixation in the first stage. The thoracic lesions were also resected totally through laminectomy and right lateral extracavitary approach followed by pedicular screw fixation.

RESULTS: The patient improved muscle powers and spasticity and is not independent (Frankel D). The histopathology examination revealed hypocellular lobulated sclerotic mass with whorling pattern of growth in the sclerotic part suggestive of WSM. Immunohistochemical evaluation demonstrated negative staining for epithelial membrane antigen, cytokeratin, Olig-2 and S-100 and immunoactivity for progesterone receptor (PR) and glial fibrillary acidic protein in the tumoral cells located at the periphery. Ki-67 indexing was low.

CONCLUSION: The spine multiple WSM is an extremely rare condition, which has a malignant behavior and it seems that it is spread by cerebrospinal fluid seeding. Surgical removal is hard and requires experience and appropriate equipment.

LEVEL OF EVIDENCE: 4.