CERVICAL INTRASPINAL EXTRAMEDULLARY GANGLIONEUROMA COEXISTING WITH NEUROFIBROMATOSIS TYPE 1: A DIAGNOSTIC DILEMMA AND LITERATURE REVIEW - A CASE REPORT

Abstract

Introduction: Spinal ganglioneuroma is a rare, mature peripheral neuroblastic neoplasm. Its cervical intradural extramedullary and multilevel presentation is exceptional, even in patients with neurofibromatosis type 1 (NF1), and it may be mistaken for nerve sheath tumors. Case Presentation: A 48-year-old woman with NF1 and a history of lumbosacral schwannoma presented with cervical pain, progressive myelopathy, and right-predominant tetraparesis. Results: Magnetic resonance imaging revealed a solid, multilobulated, posterior intradural extramedullary lesion extending from C2 to C6, heterogeneously hyperintense on T2-weighted images and associated with severe spinal cord compression. Electromyography demonstrated C4–C7 cervical polyradicular involvement. A right-sided C2–C6 crossover hemilaminectomy was performed, followed by gross total resection of the lesion. Histopathological examination identified mature ganglion cells within a Schwannian spindle-cell stroma, establishing the diagnosis of ganglioneuroma. The postoperative course was complicated by surgical site infection, meningitis, and septic shock, with persistent tetraparesis at the last available evaluation. Discussion: Magnetic resonance imaging delineated the extent of the lesion and its impact on the spinal cord but did not allow definitive differentiation of ganglioneuroma from neurofibroma or schwannoma. Radiopathological correlation prevented diagnostic anchoring associated with NF1. Conclusion: Ganglioneuroma should be included in the differential diagnosis of cervical intradural extramedullary masses in patients with NF1. Complete resection is the treatment of choice when it can be performed without compromising neurological function or cervical stability.