1588. Highly Unusual Presentation of Spinal Extraventricular Neurocytoma

Authors: Carlos Goulart, MD; Shawn Rai; Stephanie Zyck; Michael Galgano (Syracuse, NY)

Introduction:

Central Neurocytomas (CN) are benign tumors that commonly arise from the lateral ventricle. Extraventricular Neurocytoma (EVN) refers to similar tumors that arise from extra ventricular tissue. These lesions generally affect children and young adults and are extremely rare in the spine. There are limited reports of spinal cord EVNs where they usually present with paresthesias, numbness, and weakness. They are mostly misdiagnosed as oligodendrogliomas or ependymomas. To our knowledge there is a single report in the literature of a thoracic spinal cord neurocytoma-like lesion presenting with diffuse leptomeningeal spread and hydrocephalus in a 15-month-old patient.

Methods:

We present a rare case of spinal cord EVN with a highly unusual presentation of visual decline and communicating hydrocephalus from diffuse leptomeningeal spread.

Results:

The patient is a previously healthy 19-year-old male with progressive blurry vision and headaches.  His symptoms were initially treated as a migraine at an outside institution. His vision significantly worsened.  An MRI showed communicating hydrocephalus with diffuse leptomeningeal enhancement. Due to the progression of his symptoms, an external ventricular drain was placed with opening pressure higher than 30cm of H2O.  A ventriculo-peritoneal shunt was eventually placed and dural biopsy at that time was non-diagnostic.  MRI of the spine revealed an intradural extramedullary mass at T11-12. He then underwent T11-12 laminectomy and gross total resection of this lesion. Histology showed a grade II neurocytoma without atypical features. Although the initial dural biopsy was negative, we feel that the continued leptomeningeal enhancement is in fact indicative of a diffuse neoplastic process associated with the neurocytoma.

  

Conclusion:

Spinal cord EVNs are extremely rare lesions that usually presents with symptoms due to mass effect. We describe a case of thoracic spine EVN presenting with visual loss and communicating hydrocephalus with evidence of leptomeningeal enhancement.