Disseminated glioneuronal tumor with neuropil-like islands of the spinal cord: a distinctive entity.

BACKGROUND: Glioneuronal tumor with neuropil-like islands (GTNI) was recently added as a novel lesion in the most recent update of the World Health Organization classification of tumors of the central nervous system in 2007. Since this tumor's initial description, approximately 28 cases of GTNI have been published. In this report, we describe the ninth case of a spinal GTNI in the world literature.

METHODS: We report a case arising in a 2-year-old female patient who presented with headaches associated with intermittent vomiting due to a tetraventricular hydrocephalus.

RESULTS: After ventriculoperitoneal shunt placement, the patient presented with lower extremity motor weakness and sensory disturbance. A dorsolumbar spine magnetic resonance imaging scan revealed an intramedullary spinal neoplasm involving T12 through L2 in association with the thick linear enhancement of the spinal cord surfaces. A brain magnetic resonance imaging scan demonstrated focal leptomeningeal enhancement in the Sylvian fissures, the basal cistern, tentorium, and multiple small cystic-like lesions extending on the cerebellar surface, brainstem, and temporal lobes. The patient underwent a T11-L2 laminectomy for a gross total tumor resection. Histology revealed a World Health Organization grade II GTNI.

CONCLUSIONS: GTNI is a rare type of glioneuronal tumor that has recently been described in the literature. The outcome of this case seems to have an unfavorable clinical course despite their low-grade morphology. However, the combination of gross total resection and adjuvant chemo-radiotherapy can enhance chances for longer survival among children with spinal GTNI associated with meningeal dissemination, and a clinical follow-up of a large series will be necessary to evaluate the long-term prognosis.