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XXI Congreso Nacional de la Sociedad Española de Neurocirugía
Barcelona, 17-19 May 2017
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C. Barrena López, I. Arrese Regañon and R. Sarabia Herrero

Hospital Universitario Río Hortega, Valladolid, Spain.

Objectives: Ganglioneuroma (GN) is an uncommon, slow-growing tumor originating from the neural crest-derived cells which form the sympathetic nervous system during embryonic development. GN are most commonly found in the mediastinum and retroperitoneum, being intraspinal GNs extremely rare. The authors report a case of GN arising from S1 nerve root.

Methods: A 40-years-old man presented right sciatica episode, which worsened with Valsalva maneuvers. The pain was accompanied by right global muscle weakness and tingling pain. Neurological examination displayed positive Lasegue's test and sensory deficit over right L5-S1 dermatomes. Lumbar MRI without contrast described a well-circumscribed longitudinal lesion arising from L5-S1 disc space. On T1WI, the lesion appeared homogeneously hypo-intense and slightly hyper-intense on T2WI. The patient underwent surgery through paramedial lumbar L5S1 approach. We observed a mass well-encapsulated, solid and oval shaped inside S1 right root. Subtotal resection was performed. Histological examination showed clusters of ganglion mature cells with neuromatous stroma. Inmunohistochemical analysis was positive for S-100, synaptophysin and neurofilament. The diagnosis was ganglioneuroma.

Results: The incidence of GN is estimated to be over 0.1-0.5% of total SNC tumors GN occurs prevalently in childhood and highly related with patients with neurofibromatosis type I. GNs are often asymptomatic, and the majority of them are found incidentally in healthy subjects. Due to its slow growth, symptoms may result from the compressive effect of the tumor. A minority of these tumors are functional and may secrete hormones.

Conclusions: The International Neuroblastoma Pathology Committee criteria define GN as the most mature form of neuroblastic tumors. GB is considered a benign tumor, however recurrence and malignant transformation into neuroblastoma has been published. Surgical removal is the therapy of choice. The long-term prognosis is favorable even after incomplete tumor resection.

Neurocirugía (English edition)

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