This chapter attempts to bring together what is known about an emerging group of mixed tumors in the pediatric spinal cord that display both astrocytic and neuronal elements, and are difficult to classify. Only a handful of cases have been adequately documented in the literature (Poliani et al., Neuropathology 29:574–578, 2009; Psarros et al., J Neurosurg 103(Suppl):184–190, 2005; Rickert et al., Acta Neuropathol 112:231–233, 2006; Scholz et al., Acta Neuropathol 117:591–593, 2009; Syed et al., Acta Neuropathol 111:53–55, 2006), and are generally known as mixed glioneuronal tumors (GNT). The family members of this group of neoplasms are discussed under three broad groups – spinal GNTs, spinal gangliogliomas, and unclassified entities. Emphasis is given to the unique ‘spinal GNTs’ in this chapter, and these are tentatively classified into two types, a more easily identifiable variety with distinct neuronal rosettes (‘Type 1’), and a less structured variant designated as ‘Type 2’. Bulk of the documented lesions fall into the Type 1 category, based on their initial presentation and histology. The ‘spinal gangliogliomas’ are discussed only briefly, being similar in pathology and behavior to their counterparts in other parts of the brain. The ‘unclassifiable’ group is an enigma, and awaits further multicenter collaborative studies for accurate categorization. The glioneuronal tumors in the spinal cord behave like low-grade neoplasms in most instances, with rare exceptions where prognosis can be unpredictable, and can lead to metastases, further morbidity, and grave outcomes.
Glioneuronal tumor Pediatric Spinal
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I am grateful to Professor Arie Perry, neuropathologist, Washington University Medical Center, St LouIs, MO, and Tarik Tihan, neuropathologist, University of San Francisco Medical Center, San Francisco, CA, for their valuable suggestions and help with this write-up.
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