J Korean Neurosurg Soc.  2021 Jul;64(4):592-607. 10.3340/jkns.2020.0243.

Clinicopathological Features of Primary Solitary Spinal Cord Tumors in Pediatric Patients : A 32-Year Single Institution Experience

Affiliations
  • 1Department of Neurosurgery, Kyung Hee University Hospital at Gangdong, Kyung Hee University School of Medicine, Seoul, Korea
  • 2Division of Pediatric Neurosurgery, Department of Neurosurgery, Seoul National University Children’s Hospital, Seoul National University College of Medicine, Seoul, Korea
  • 3Department of Neurosurgery, Armed Forces Capital Hospital, Seongnam, Korea
  • 4Neuro-oncology Clinic, National Cancer Center, Goyang, Korea
  • 5Department of Anatomy, Seoul National University College of Medicine, Seoul, Korea
  • 6Department of Pathology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea

Abstract


Objective
: Few studies exist on primary spinal cord tumors (PSCTs) in pediatric patients. The purpose of this study was to perform descriptive analysis and detailed survival analysis for PSCTs.
Methods
: Between 1985 and 2017, 126 pediatric patients (male : female, 56 : 70) with PSCTs underwent surgery in a single institution. We retrospectively analyzed data regarding demographics, tumor characteristics, outcomes, and survival statistics. Subgroup analysis was performed for the intramedullary (IM) tumors and extradural (ED) tumors separately.
Results
: The mean age of the participants was 6.4±5.04 years, and the mean follow-up time was 69.5±46.30 months. The most common compartment was the ED compartment (n=57, 45.2%), followed by the IM (n=43, 34.1%) and intradural extramedullary (IDEM; n=16, 12.7%) compartments. Approximately half of PSCTs were malignant (n=69, 54.8%). The most common pathologies were schwannomas (n=14) and neuroblastomas (n=14). Twenty-two patients (17.5%) died from the disease, with a mean disease duration of 15.8±15.85 months. Thirty-six patients (28.6%) suffered from progression, with a mean period of 22.6±30.81 months. The 10-year overall survival (OS) rates and progression-free survival (PFS) rates were 81% and 66%, respectively. Regarding IM tumors, the 10-year OS rates and PFS rates were 79% and 57%, respectively. In ED tumors, the 10-year OS rates and PFS rates were 80% and 81%, respectively. Pathology and the extent of resection showed beneficial effects on OS for total PSCTs, IM tumors, and ED tumors. PFS was affected by both the extent of removal and pathology in total PSCTs and ED tumors; however, pathology was a main determinant of PFS rather than the extent of removal in IM tumors. The degree of improvement in the modified McCormick scale showed a trend towards improvement in patients with IM tumors who achieved gross total removal (p=0.447).
Conclusion
: Approximately half of PSCTs were malignant, and ED tumors were most common. The most common pathologies were schwannomas and neuroblastomas. Both the pathology and extent of resection had a decisive effect on OS. For IM tumors, pathology was a main determinant of PFS rather than the extent of removal. Radical excision of IM tumors could be a viable option for better survival without an increased risk of worse functional outcomes.

Keyword

Spinal cord neoplasms; Spinal neoplasms; Pediatrics; Prognosis

Figure

  • Fig. 1. Functional outcome before and after surgery. The postoperative modified McCormick scale improved after the operation in all three groups (total group, intramedullary group, and extradural group). The amount of improvement did not show a significant difference among groups.

  • Fig. 2. Kaplan-Meier survival curves of pediatric patients with primary spinal cord tumors (PSCTs). Overall survival (OS) and progression-free survival (PFS) for pediatric patients with PSCTs. In contrast to the OS curve (A), PFS statistics show a continuous decline after early rapid descent (B). OS of PSCTs according to malignancy (C). OS rates were significantly lower in patients with malignant tumors (p<0.001). PFS was also significantly lower in patients with malignant tumors (p=0.031). For benign tumors, there exists late progression following surgery (D). In the gross total resection (GTR) group, OS rates were superior to those in the non-GTR group (p<0.001) (E). PFS was significantly higher in patients with GTR than in those without GTR (p=0.003) (F).

  • Fig. 3. Kaplan-Meier survival curves of pediatric patients with intramedullary (IM) tumors. Overall survival (OS) and progression-free survival (PFS) for pediatric patients with IM spinal cord tumors (A and B). In high-grade IM tumors, OS rates were worse than in low-grade IM tumors (p<0.001) (C). PFS rates were also significantly different between groups with high-grade IM tumors and low-grade tumors (p<0.001) (D). IM tumors with gross total resection (GTR) show higher OS rates than those without GTR (0.025) (E). Regarding PFS, the results are inconclusive between groups with GTR and non-GTR (p=0.641) (F).

  • Fig. 4. Kaplan-Meier survival curves of pediatric patients with extradural (ED) tumors. Overall survival (OS) and progression-free survival (PFS) for pediatric patients with ED spinal cord tumors (A and B). For malignant ED tumors, OS rates were worse than for benign ED tumors (p=0.019) (C). PFS rates were also significantly different between groups with malignant tumors and benign tumors (p=0.018) (D). ED tumors with gross total resection (GTR) showed higher OS rates than those without GTR (p=0.007) (E). PFS was also significantly different between groups with GTR and non-GTR (p=0.007) (F).


Reference

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