• Spinal cord glioneuronal tumor with rosetted neuropil-like islands in pediatric age group
  • Sevda Ghader,1 Fatemeh Nasiri,2 Ali Razmara,3 Elaheh Oliyaei,4,*
    1. Department of pediatrics,faculty of medicine,Urmia university of medical sciences,Urmia,Iran
    4. Faculty of Paramedicine, Department of medicine, Bushehr University of Medical Science, Bushehr, Iran


  • Introduction: Glioneuronal neoplasms are unusual tumors categorized through manner of approach of world health organization (WHO) into three sorts consisting of papillary glioneuronal tumor (PGT), rosetted glioneuronal tumor with neuropil-like islands (GTNI), and rosette-forming glioneuronal tumor (RGNT) of fourth ventricle. Glioneuronal tumors commonly have an impact on children and more youthful adults with a predilection for the temporal lobe but recently, GTNIs happening in extraventricular sites especially spinal cord and those with malignant behaviors were reported. The literature in this novel neoplasm is sparse and confined, very probably for their rarity and variable clinical aggressiveness. Reviews on charactristics of this tumor are fewer and far between with the current as a great deal as a decade old.
  • Methods: In the forthcoming systematic study, the required data were collected using keywords and citing valid databases such as: Scopus PubMed, Google Scholar and ProQuest. The statistical population of the study includes all studies conducted up to 2022 in the field of Spinal cord glioneuronal tumor with rosetted neuropil-like islands in pediatric age group. After reviewing the relevant findings and evaluating the data quality, a total of 16 articles were analyzed.
  • Results: These tumors were mainly described withinside the pediatric population, with a median age of five years and a slow growing route with a slight female dominance. The imaging characteristic of spinal GTNI is that of a big intramedullary mass, with MRI findings showing a solid mass without or with cystic components. GNTNI appears with a biphasic histology, including neurocytic cells that surround precise oval neuropil-rich islands and protoplasmic, fibrillary, or gemistocytic astrocytes as part of the glial part. This glial component shows anaplastic features consisting of more frequent mitosis, increased cellularity, and nuclear pleomorphic and high proliferation index. Vascular proliferation and necrosis are unusual. Considering the location of GTNI tumors, most of them were positioned withinside the supratentorial and few were in spinal regions. However, authors describe disseminated or spinal disease at primary stage of tumor frequently. The most frequent clinical symptoms of spinal GTNI are weakness, numbness, and limb paresthesia. Other symptoms include lumbar, chest, and lower back pain. Scoliosis and urinary incontinence, superior neck pain and dizziness with no dominant neurological change were moreover noticed in a few cases. According to literature reviews, spinal GTNIs are poor in their prognosis. Clinical treatment includes tumor resection combined with chemotherapy and radiotherapy.
  • Conclusion: In summary, as glioneuronal neoplasms are low grade and nicely manageable, the knowledge of their clinical presentation and histological analysis is essential for treatment. GTNI withinside the spinal cord is unusual and appears to be more aggressive; therefore, it is all-important to distinguish GTNI from exclusive benign glioneuronal tumors. Moreover, the pathophysiologies and biological features of spinal GTNI are not definitely known yet. Further researches with long-time period follow-up based mostly on treatment plans are required to further improve the management of GTNIs.
  • Keywords: spinal cord tumors, Neuropil-riched islands، Neurologic symptoms، tumors in Pediatric Age Group