spinal_cord_glioma

see also Spinal cord astrocytoma.

The level of discordant diagnoses in children and adolescents with institutional diagnosis of high grade glioma (HGG) of the spinal cord was 44% in a experience. However, there was no significant difference in outcome between patients with confirmed and discordant diagnosis. This group of tumor deserves a specific attention in future trials 1).


High-grade spinal cord gliomas are rare and carry a poor prognosis.

A number of treatment modalities exist for spinal cord gliomas, but no consensus exists regarding their management.

Despite their increasing clinical incidence, intramedullary spinal cord gliomas remain without an effective treatment strategy. Given their diffusely invasive nature, surgical resection is perilous, and both chemotherapeutic and radiation options demonstrate poor response.

Over the past decade, researchers have found that neural stem cells (NSCs) migrate toward inflammatory sites, including tumors. This insight has inspired research into genetic engineering of human NSCs to express enzymes such as cytosine deaminase (CD) and thymidine kinase (TK). These enzymes enable these cells to convert the nontoxic prodrugs 5-fluorocytosine (5FC) and ganciclovir (GCV) into oncolytic 5-fluorouracil and GCV-triphosphate, respectively.

Both of these agents inhibit tumor growth by impeding DNA elongation, thus triggering apoptosis. Ropper et al3 hypothesized that these dual gene-engineered human NSCs could be used to effectively treat intramedullary spinal cord gliomas by using their glioma trackability to deliver targeted chemotherapeutic agents.

Cordectomy represents a possible option for treating these lesions; however, few cases have been reported in adults, and none have been reported in the pediatric population. The authors describe the use of cordectomy for the treatment of a high-grade spinal glioma in a 9-year-old boy who remains cancer free 14 years following his initial presentation 2).

Twenty-five patients with spinal cord glioma of grade IV who underwent surgery in a single institute were selected. All grade IV spinal cord glioma histologically confirmed as glioblastoma or “diffuse midline glioma with H3 K27M-mutant” by the 2016 WHO classification of the central nervous system were included. Basic demographics, treatment modalities, and pathological tumor molecular profiles were investigated for prognosis.

RESULTS: Mean age was 39.1 yr; male to female ratio was 18 : 7. Tumor was located in thoracic cord (53.3%), cervical cord (40%), and lumbar area (6.7%). Median overall survival was 37.1 mo; median disease-free survival was 18.5 mo. Treatment modality showed no statistical difference. Only K27M profile showed significant prognostic value, 20 patients (80%) showed K27M mutation positive, K27M mutation patients showed longer overall survival (40.07 mo) than K27M negative patients (11.63 mo, P < .0001), and disease-free survival (20.85 vs 8.72 mo, P = .0241).

CONCLUSION: This study is the first and largest report of the prognosis of primary spinal cord grade IV glioma using the new WHO classification. This study reported survival analysis and prognostic factors, and revealed that H3.3 K27M mutation is not a major poor prognostic factor. Further studies to explore K27M mutations needed for risk stratification and therapy optimization 3).


1)
Bouffet E, Allen JC, Boyett JM, Yates A, Gilles F, Burger PC, Davis RL, Becker LE, Pollack IF, Finlay JL. The influence of central review on outcome in malignant gliomas of the spinal cord: the CCG-945 experience. J Neurosurg Pediatr. 2016 Apr;17(4):453-9. doi: 10.3171/2015.10.PEDS1581. Epub 2015 Dec 18. PubMed PMID: 26684767.
2)
Crowley RW, Burke RM, Lopes MB, Hamilton DK, Jane JA Sr. Long-term cure of high-grade spinal cord glioma in a pediatric patient who underwent cordectomy. J Neurosurg Spine. 2015 Aug 7:1-7. [Epub ahead of print] PubMed PMID: 26252785.
3)
Yi S, Choi S, Shin DA, Kim DS, Choi J, Ha Y, Kim KN, Suh CO, Chang JH, Kim SH, Yoon DH. Impact of H3.3 K27M Mutation on Prognosis and Survival of Grade IV Spinal Cord Glioma on the Basis of New 2016 World Health Organization Classification of the Central Nervous System. Neurosurgery. 2018 May 1. doi: 10.1093/neuros/nyy150. [Epub ahead of print] PubMed PMID: 29718432.
  • spinal_cord_glioma.txt
  • Last modified: 2018/05/04 21:16
  • by administrador