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A Unique Case of Synchronous Extramedullary Spinal Masses – Is Surgery an Answer?

Rachana Yendala , Nauman Shahid , Ahmed Alnemari , Robert Mrak , Mark Buehler , Daniel Gaudin Muhammad Hameed

Multiple spinal tumors are known to occur in association with phacomatosis like neurofibromatosis. Rarely, sporadic occurrences have been described in the absence of a clear underlying pathogenesis. The majority of intradural extramedullary spinal tumors are meningiomas or nerve sheath tumors like schwannoma and neurofibroma. Ependymomas, which are the most common intramedullary spinal tumors in adults, can also occur in the extramedullary intradural space and impose a diagnostic challenge. Only a few such cases have been described in the literature. We report the first sporadic occurrence of an intradural extramedullary ependymoma synchronously presenting with a different neoplasm, a schwannoma. A 43-year-old woman presented to the authors’ institution with lumbosacral pain and neurological deficits lasting a decade. On magnetic resonance imaging, she was found to have two stable, contrast-enhancing intradural extramedullary spinal lesions in the L2-3 and L4-5 vertebral areas. She subsequently underwent L2-L5 laminectomy with total resection of the former lesion and subtotal resection of the later lesion. Histological and immunohistochemical analysis revealed two distinct tumors: a cellular ependymoma and a schwannoma at the two respective locations. Ependymomas are low grade, slowly growing tumors that mimic other, benign tumors clinically as well as radiologically. However, the management of these tumors differs since ependymomas have a potential for recurrence and metastasis. The underlying etiology and pathogenesis of these tumors is not clearly established. However, an association with an underlying phacomatosis needs to be considered. Our purpose is to create awareness that an intradural extramedullary ependymoma can coexist with other spinal tumors and should be included in the differential diagnosis of a multifocal spinal lesion presentation. Owing to difficulties in differentiating these closely mimicking spinal tumors clinically and radiologically, early identification with surgical resection, when possible, might be a recommended strategy