neuroplex
New York Association of Neuropathologists
1/15/2008 Case 4: Ganglioma, SC
Presented by: Dr. Weidenheim - Montefiore Medical Center
Clinical History:
Case of a 29 year old male with a past history of viral meningitis, who presented with low back pain which spread to both buttocks and legs, associated with peri-anal numbness. Symptoms progressed over a 3 month period. He developed urinary retention, constipation, and inability to walk. Imaging revealed a 5 x 2.5 cm partially cystic mass in the conus medullaris, with a heterogeneous signal, evidence of hemorrhage and a syrinx above the mass involving most of the thoracic spinal cord. Slides showed a glial tumor with a fibrillary astrocytic background and numerous large pleomorphic disorganized neuronal cells.
Diagnostic Notes:
The diagnosis was ganglioglioma. Ganglioglioma is a well differentiated, slowly growing neuroepithelial tumor consisting of neurons with glia. According to the WHO 2007 classification, most gangliogliomas are WHO Grade I. Some tumors with anaplastic features are considered Grade III. Interestingly, in the new classification, criteria for Grade II are not well established. These represent 0.4% of all CNS tumors, and 1.3% of all brain tumors, are the most frequent entity seen in patients with long standing epilepsy and occur mostly in young patients (</= 20y) with a mean age at diagnosis 8.5 yr. Most are supratentorial but this example is in the spinal cord. Pediatric Spinal Cord tumors (PSCT) occur at an incidence is 1 tumor/million children/year. From a series of 26 PSCT in 31 counties around CHOP over 14 years (population-based) these were mostly astrocytomas with a rare ependymoma or ganglioglioma; a referral based series from NYU revealed 27% gangliogliomas as well as other lesions including ependymomas, JPA, neuronal tumors, hemangioblastoma, mixed gliomas; other series have included occasional cysts, teratoma, tuberculoma, AVM in this region. Gangliogliomas tend to be more common in males (M:F = 1.5:1) with a median age at diagnosis 6 years (range, 7 mo-25 y). They are most common in the cervicothoracic region and tend to be large (4-8 vertebral segments) at diagnosis. Gross total or subtotal resection is therapy of choice and the 5 year survival is 88%. Recurrence in 30% followed a period of stable function. Radical surgery is the treatment of choice because of the minimal morbidity and indolent behavior of most tumors
References:
- Jallo GI, Freed D, Epstein FJ. Spinal cord gangliogliomas; a review of 56 patients. J Neuron Oncol 2004;68:71-77
- Hardison HH et al. Outcome of children with primary intramedullary spinal cord tumors. Childs Nerv Syst 1987;3:89-92.
- McGirt MJ et al. Neurological outcome after resection of intramedullary spinal cord tumors in children. Childs Nerv Syst2007;24:93-97.
- Tobias M et al. Surgical management of long intramedullary spinal cord tumors. Childs Nerv Syst 207;DOI10.1007/s00381-007-0405-7.
