Neuronal/glioneuronal tumors are uncommon neoplasms of the CNS with frequent association with refractory epilepsy. Reports documenting the entire spectrum of neuronal/glioneuronal tumors are scarce in the literature. Zulch et al. from Germany in a large series reported that neuronal/glioneuronal tumors accounted for 0.4% (38/9000 cases) of all brain tumors, with similar incidence reported from Japan (0.4%), with higher incidence from Korea (2.1%). However, data from the Indian subcontinent are lacking. We reviewed 244 cases of neuronal/glioneuronal tumors of the CNS diagnosed over the last decade at our Institute and they constituted 0.86% of all CNS tumors (244/28061) received in that period. Mean age at presentation was 25.06 years (range: 1-75 years) with male preponderance (M:F = 1.54 : 1). The majority occurred in third decade (76 cases, 31.4%), with only few cases occurring beyond fifth decade (13 cases, 5.3%). Ganglioglioma/gangliocytoma (94 cases, 38.52%) was the most frequent followed by central neurocytoma (86 cases, 35.24%), paraganglioma (32 cases, 13.52%), dysembryoplastic neuroepithelial tumors (DNET) (21 cases, 8.6%), desmoplastic infantile astrocytoma/desmoplastic infantile ganglioglioma (DIA/DIG) (6 cases, 2.45%), papillary glioneuronal tumor (PGNT) (3 cases, 1.22%) and rosette-forming glioneuronal tumor (RGNT) (1 case, 0.4%). Association with seizures was noted in 40.95% of cases. Glioneuronal tumors are an expanding group of tumors with varying spectra of morphologic patterns and biological behavior. An improved understanding has direct clinical implications for optimizing current treatments and developing novel therapeutic approaches. Although most glioneuronal tumors carry a favorable prognosis, other factors such as inaccessibility to surgical resection and rarely, malignant transformation, make it difficult to accurately predict the biological behavior based on histopathology alone. Reliable prognostic markers remain to be defined.
Cervical spine injuries occur infrequently in children but are associated with significant disability and mortality. A retrospective analysis was performed of 84 consecutive pediatric spine injuries treated at our institute from January 2002 to December 2011. The mean age was 14.7 years. There were 18 patients (21%) in group A (0-12 years) and 66 patients (79%) in group B (13-18 years). Overall, injury was more common in boys (ratio of 6:1). Trivial fall was the predominant cause in group A and fall from height in group B. There were 30 children (36%) with injuries of the upper cervical spine, 53 (63%) with injuries of the lower cervical spine and 1 patient (1%) with a combined injury of upper cervical spine and thoracic spine. Overall, 22% of the group A children and 67% of the group B patients had more severe injuries (Frankel grades A, B and C); 21% (18/84) were treated by surgical fusion. Where follow-up was available, 17 out of 22 children (77%) had good outcome (Frankel grade >C). In conclusion, mechanisms and patterns of injury in children are age related and the majority of the children had good outcome.
Figure 2. Close-up of juxtaposed glial (g) and mesenchymal (m) components. Note rich reticulin response limited to mesenchymal component. Immunohistochemistry shows GFAP confined to glial zones in contrast vimentin positivity is noted in both components. High MIB-1 labeling was evident in both components as well as p53 expression.
CVJ pathology is a predisposing factor for HCDD. HCDD presents in a poorer Nurick's grade compared to lower cervical PIVD and majority of the patients have T2 hyperintense intramedullary signal changes in MRI. Presence of T2 hyperintense intramedullary signal changes is associated with poorer pre-op clinical status and poorer post-op outcome.
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