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

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.

Epidemiology and Comparative Analysis of Outcomes of Intramedullary Spinal Cord Tumor Between Pediatric and Adult Patients

“…Astrocytoma was the most common histopathological type in this study (41%), which is similar to previous reports (32.2%–74%) 2,6–8,10–12 . Similarly, low-grade astrocytoma was the most common pathology in pediatric patients 1,2,6,7,12,13 .…”

“…Presumably, because patients who experienced symptom progression to paresis or gait disturbance were taken to the hospital by their parents. Aligned with our results, the motor deficit was the most common presenting symptom in prior work on this topic 6–11 . Only two previous studies reported that either pain or sensory deficit was the most common symptom (Supplemental Table 1, Supplemental Digital Content 1, http://links.lww.com/SLA/E744).…”

“…However, there was no significant difference when compared across each pathological type, except PFS in ependymoma. Previous studies have also reported poor 5-year OS rates (76%–82%) and PFS rates (71%–73%) in PISCTs 11,15 . Our adjusted Cox model did not identify pediatric age as a statistically significant prognostic factor of OS.…”

“…Hence, poor prognosis in PISCTs could be attributed to other specific characteristics of pediatric PISCTs. Some previous studies have shown that histopathological grade is a prognostic factor in PISCTs 1,6,7,9,11,12,18 . However, they did not consider that it is a prognostic factor in adult ISCTs also.…”

“…Aligned with our results, the motor deficit was the most common presenting symptom in prior work on this topic. [6][7][8][9][10][11] Only two previous studies reported that either pain or sensory deficit was the most common symptom (Supplemental Table 1, Supplemental Digital Content 1, http://links.lww.com/SLA/E744). 1,12 However, no adjusted analyses were performed and ours is among the first to characterize differences in preoperative symptoms between pediatric and adult patients with ISCTs.…”