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http://dx.doi.org/10.3340/jkns.2020.0243

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

Choi, Ho Yong (Department of Neurosurgery, Kyung Hee University Hospital at Gangdong, Kyung Hee University School of Medicine)
Kim, Kyung Hyun (Division of Pediatric Neurosurgery, Department of Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine)
Cho, Byung-Kyu (Department of Neurosurgery, Armed Forces Capital Hospital)
Wang, Kyu-Chang (Neuro-oncology Clinic, National Cancer Center)
Phi, Ji Hoon (Division of Pediatric Neurosurgery, Department of Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine)
Lee, Ji Yeoun (Division of Pediatric Neurosurgery, Department of Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine)
Park, Sung-Hye (Department of Pathology, Seoul National University Hospital, Seoul National University College of Medicine)
Kim, Seung-Ki (Division of Pediatric Neurosurgery, Department of Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine)
Publication Information
Journal of Korean Neurosurgical Society / v.64, no.4, 2021 , pp. 592-607 More about this Journal
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.
Keywords
Spinal cord neoplasms; Spinal neoplasms; Pediatrics; Prognosis;
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1 Papagelopoulos PJ, Peterson HA, Ebersold MJ, Emmanuel PR, Choudhury SN, Quast LM : Spinal column deformity and instability after lumbar or thoracolumbar laminectomy for intraspinal tumors in children and young adults. Spine (Phila Pa 1976) 22 : 442-451, 1997   DOI
2 Spacca B, Giordano F, Donati P, Genitori L : Spinal tumors in children: long-term retrospective evaluation of a series of 134 cases treated in a single unit of pediatric neurosurgery. Spine J 15 : 1949-1955, 2015   DOI
3 Choi GH, Oh JK, Kim TY, You NK, Lee HS, Yoon DH, et al. : The clinical features and surgical outcomes of pediatric patients with primary spinal cord tumor. Childs Nerv Syst 28 : 897-904, 2012   DOI
4 Ostrom QT, Gittleman H, Truitt G, Boscia A, Kruchko C, Barnholtz-Sloan JS : CBTRUS statistical report: primary brain and other central nervous system tumors diagnosed in the United States in 2011-2015. Neuro Oncol 20(suppl_4) : iv1-iv86, 2018   DOI
5 Wetjen NM, Raffel C : Principles and practice of pediatric neurosurgery, ed 2. New York : Thieme, 2008, pp694-705
6 Birch BD, Johnson JP, Parsa A, Desai RD, Yoon JT, Lycette CA, et al. : Frequent type 2 neurofibromatosis gene transcript mutations in sporadic intramedullary spinal cord ependymomas. Neurosurgery 39 : 135-140, 1996   DOI
7 Chou SC, Kuo MF, Lai DM, Chen CM, Xiao F, Tsuang FY, et al. : Contemporary management of pediatric spinal tumors: a single institute's experience in Taiwan in the modern era. J Neurooncol 146 : 501-511, 2020   DOI
8 Crawford JR, Zaninovic A, Santi M, Rushing EJ, Olsen CH, Keating RF, et al. : Primary spinal cord tumors of childhood: effects of clinical presentation, radiographic features, and pathology on survival. J Neurooncol 95 : 259-269, 2009   DOI
9 Houten JK, Weiner HL : Pediatric intramedullary spinal cord tumors: special considerations. J Neurooncol 47 : 225-230, 2000   DOI
10 McGirt MJ, Chaichana KL, Atiba A, Attenello F, Woodworth GF, Jallo GI : Neurological outcome after resection of intramedullary spinal cord tumors in children. Childs Nerv Syst 24 : 93-97, 2008
11 Nadkarni TD, Rekate HL : Pediatric intramedullary spinal cord tumors. Critical review of the literature. Childs Nerv Syst 15 : 17-28, 1999   DOI
12 Amene C, Levy M, Crawford J : Pediatric Spinal Cord Tumors in Hayat M (ed) : Tumors of the Central Nervous System. Dordrecht : Springer, 2014, Vol 11, pp197-212
13 Ahmed R, Menezes AH, Awe OO, Mahaney KB, Torner JC, Weinstein SL : Long-term incidence and risk factors for development of spinal deformity following resection of pediatric intramedullary spinal cord tumors. J Neurosurg Pediatr 13 : 613-621, 2014   DOI
14 Azad TD, Pendharkar AV, Pan J, Huang Y, Li A, Esparza R, et al. : Surgical outcomes of pediatric spinal cord astrocytomas: systematic review and meta-analysis. J Neurosurg Pediatr 22 : 404-410, 2018   DOI
15 Baysefer A, Akay KM, Izci Y, Kayali H, Timurkaynak E : The clinical and surgical aspects of spinal tumors in children. Pediatr Neurol 31 : 261-266, 2004   DOI
16 Cho BK, Park IS, Wang KC, Choi KS : Intraspinal tumors in children: clinical analysis of 24 cases (1973~1986). J Korean Neurosurg Soc 17 : 1359-1368, 1988
17 Constantini S, Houten J, Miller DC, Freed D, Ozek MM, Rorke LB, et al. : Intramedullary spinal cord tumors in children under the age of 3 years. J Neurosurg 85 : 1036-1043, 1996   DOI
18 Constantini S, Miller DC, Allen JC, Rorke LB, Freed D, Epstein FJ : Radical excision of intramedullary spinal cord tumors: surgical morbidity and long-term follow-up evaluation in 164 children and young adults. J Neurosurg 93(2 Suppl) : 183-193, 2000   DOI
19 Elia-Pasquet S, Provost D, Jaffre A, Loiseau H, Vital A, Kantor G, et al. : Incidence of central nervous system tumors in Gironde, France. Neuroepidemiology 23 : 110-117, 2004   DOI
20 Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, Cavenee WK, et al. : The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol 131 : 803-820, 2016   DOI
21 Fortuna A, Nolletti A, Nardi P, Caruso R : Spinal neurinomas and meningiomas in children. Acta Neurochir (Wien) 55 : 329-341, 1981   DOI
22 Kutluk T, Varan A, Kafali C, Hayran M, Soylemezoglu F, Zorlu F, et al. : Pediatric intramedullary spinal cord tumors: a single center experience. Eur J Paediatr Neurol 19 : 41-47, 2015   DOI
23 Liigant A, Asser T, Kulla A, Kaasik AE : Epidemiology of primary central nervous system tumors in Estonia. Neuroepidemiology 19 : 300-311, 2000   DOI
24 Manzano G, Green BA, Vanni S, Levi AD : Contemporary management of adult intramedullary spinal tumors-pathology and neurological outcomes related to surgical resection. Spinal Cord 46 : 540-546, 2008   DOI
25 McCormick PC, Torres R, Post KD, Stein BM : Intramedullary ependymoma of the spinal cord. J Neurosurg 72 : 523-532, 1990   DOI
26 McGirt MJ, Chaichana KL, Atiba A, Attenello F, Yao KC, Jallo GI : Resection of intramedullary spinal cord tumors in children: assessment of long-term motor and sensory deficits. J Neurosurg Pediatr 1 : 63-67, 2008   DOI
27 Murovic J, Sundaresan N : Pediatric spinal axis tumors. Neurosurg Clin N Am 3 : 947-958, 1992   DOI
28 Neumann HP, Eggert HR, Weigel K, Friedburg H, Wiestler OD, Schollmeyer P : Hemangioblastomas of the central nervous system. A 10-year study with special reference to von Hippel-Lindau syndrome. J Neurosurg 70 : 24-30, 1989   DOI
29 Schellinger KA, Propp JM, Villano JL, McCarthy BJ : Descriptive epidemiology of primary spinal cord tumors. J Neurooncol 87 : 173-179, 2008   DOI
30 Schick U, Marquardt G : Pediatric spinal tumors. Pediatr Neurosurg 35 : 120-127, 2001   DOI
31 Wilson PE, Oleszek JL, Clayton GH : Pediatric spinal cord tumors and masses. J Spinal Cord Med 30 Suppl 1(Suppl 1) : S15-S20, 2007   DOI
32 Yao KC, McGirt MJ, Chaichana KL, Constantini S, Jallo GI : Risk factors for progressive spinal deformity following resection of intramedullary spinal cord tumors in children: an analysis of 161 consecutive cases. J Neurosurg 107(6 Suppl) : 463-468, 2007
33 Ahmed R, Menezes AH, Awe OO, Torner JC : Long-term disease and neurological outcomes in patients with pediatric intramedullary spinal cord tumors. J Neurosurg Pediatr 13 : 600-612, 2014   DOI