Spinal cord tumors are rare central nervous system (CNS) tumors that involve the primary spinal cord, spinal meninges, or cauda equina. Unlike in adults, spinal cord tumors in children are usually primary tumors, not metastases.
Multifactorial changes at the cellular level are just beginning to be described. In different anatomical locations, the same histopathological phenotype may have different tumor biology. Malignant transformation at the molecular level is much less understood in children.
Epidemiology including risk factors and primary prevention
Compared to adults, spinal cord tumors in pediatrics have lower incidences and have different histologic features, sites of origin, and responses to treatment. Although CNS tumors are the most common solid tumor in the United States, spinal cord tumors account for 1-2% of all childhood CNS tumors.1 Due to their rarity, comprehensive epidemiologic studies have been challenging in children.
In children and adolescents (age 0-19), the overall incidence of primary tumors of the CNS is 6.14 per 100,000.2 Primary pediatric spinal cord tumors account for 1-10% of all CNS tumors in this age group, with an estimated incidence of 0.27 per 100,000 persons.3,4
Primary spinal cord tumors can be classified by anatomical sublocation5,6:
- Intradural intramedullary: Primarily glimoas, such as astrocytomas and ependymomas
- Intradural extramedullary: Primarily meningiomas and peripheral nerve sheath tumors
- Extramedullary: Primarily metastatic in origin
Common pediatric spinal tumor histologic subtypes include2:
- Ependymal Tumors (19.6%)
- Tumors of the Meninges (17.8%)
- Nerve Sheath Tumors (17.2%)
- Other Neuroepithelial Tumors (16.6%)
- Pilocytic Astrocytoma (11.4%)
- Other Astrocytoma/Glioblastoma (8.3%)
Metastatic tumors are very rare in children and may occur as drop metastases from brain tumors, unlike in adults, where most common spinal cord tumors are metastatic. In terms of location, extradural tumors account for approximately 30% of all pediatric spinal tumors.6
In the pediatric population, the 5- and 10-year survival rate after being diagnosed with a tumor of the spinal cord proper or cauda equina is 93.6% and 92.1%, respectively.2 The median age of diagnosis is 11 years and has higher incidence in caucasians.7
Pediatric spinal cord tumors are classified by the type of tissue involved, anatomical location, and growth/invasiveness. Tumor grade and histology directly affect prognosis, as some tumors are better resected than others. Malignant transformation of pediatric low-grade gliomas is very unusual compared to those in adults.8 Radiation therapy may generate malignant transformation by accelerating a tumor’s natural tendency towards dedifferentiation or by engendering a de novo high-grade tumor.
Disease progression including natural history, disease phases or stages, disease trajectory (clinical features and presentation over time)
Children with low-grade spinal cord gliomas have a peak age of diagnosis between one and two years of age, with 41% diagnosed before age four.9
Pain of the bony segment directly over the tumor is the most common presenting complaint in children.10,11 The onset of back pain may be over months. Notably, back pain is generally uncommon in pediatrics, and therefore should always be thoroughly investigated.
Other common presenting symptoms include abnormal gait or coordination difficulties, spinal deformity, focal motor weakness, and sphincter dysfunction.10 Older children may also note a history of progressive motor weakness. In infants, nonspecific crying episodes followed by motor weakness or torticollis may be seen.
Long-term motor and sensory neuropathy deficits are often seen in children following intramedullary spinal cord tumor (IMSCT) resection. In a long-term assessment of 164 children who underwent IMSCT resection12:
- 1/3 experienced improvements in motor, sensory and urinary dysfunction years after resection.
- 2/3 experienced long-term dysesthetic symptoms that impacted their quality of life.
- Independent risk factors for long-term dysesthesia include increasing age, preoperative symptom duration >12 months, and worsening postoperative neurological symptoms.
Survival depends on the histology and grade of the spinal cord tumor. High-grade intramedullary astrocytomas have poor prognoses; survival can be three to twelve months despite aggressive treatment.13,14
Specific secondary or associated conditions and complications
Neurofibromatosis is associated with low-grade gliomas. Orthopedic complications, most often severe kyphoscoliosis, occurs in 35% of children treated for low-grade spinal gliomas.9 Early scoliosis is the most common sign of a thoracic astrocytoma. Significant syrinxes can also be found in many children with astrocytomas. Treatment related complications may also occur including radiation myelopathy from radiation over the spinal cord or the vertebral column.1
Essentials of Assessment
- Ask about neurological deficits, both motor and sensory.
- Evaluate for dysphagia, neurogenic bowel and bladder, spasticity, scoliosis, and syrinx.
- Take a detailed pain history.
- Ask about the child’s or adolescent’s current schooling, academic progress, and family’s expectations on return to school post-rehabilitation.
- Take a detailed social history to determine family support, home setting, and need for ramps, doorway modifications, or other structural modifications. If environmental modifications or computer-aided environmental control systems are indicated, ask if the family has financial resources, as these items are generally not covered by insurance or Medicaid.
- Detail the child’s social role and social support system. Ask about members of child’s family, as well as details of the home, school, and recreational environment.
Check for palpable tenderness over the involved bony segment. Often it is most painful directly over the tumor.
Perform a complete neuromuscular exam: evaluate for motor strength, coordination, reflexes, vibration, proprioception, light and sharp touch.
Complete an American Spinal Injury Association (ASIA) assessment, keeping in mind the child’s developmental age and need for modesty (see the ISNCSCI Exam Pediatric Brochure at https://asia-spinalinjury.org/information/download/).
Check for presence of scoliosis/kyphosis and their flexibility.
- Functional Independence Measure (FIM) and Functional Independence Measure for Children (WeeFIM) (available at https://www.udsmr.org/)
- Pediatric Evaluation of Disability Inventory (PEDI) (available at https://www.pearsonassessments.com/)
- Pediatric Balance Scale (PBS) (see ref. 15)
- Karnofsky Performance Scale (KPS): used by oncologists; standard measure of cancer patient’s ability to perform ordinary tasks (see http://www.npcrc.org/files/news/karnofsky_performance_scale.pdf).
- Pediatric Quality of Life Inventory (PedsQL) (available at http://www.pedsql.org).
- Modified McCormick Scale (mMS) – used by some neurosurgical studies to determine pre- and postoperative neurological function: (see https://www.nature.com/articles/sc200851/tables/1).
In addition to World Health Organization (WHO) grading, histological and molecular verification of the tumor is paramount to pathological diagnosis. Immunohistochemistry, such as SOX-10 staining, can help differentiate ependymomas from astrocytomas.16 Information on the underlying genetic abnormalities in pediatric diffuse gliomas allows better separation of these tumors from similar adult versions.11 Cerebral spinal fluid (CSF) studies are generally helpful to rule out other causes of spinal cord lesions, such as infection, but are not diagnostic for intramedullary tumors. See the section on treatment for further guidelines.
MRI of the spinal cord and/or brain with and without gadolinium enhancement is the imaging modality of choice for the preoperative evaluation of spinal cord tumors. Intraoperative imaging may also decrease the need for further surgery. Imaging also plays a role in detecting tumor caused hydrocephalus, syringomyelia, scoliosis, and other complications.
Early predictions of outcomes
Early diagnosis of spinal cord tumors is most favorable.17,18,19
Tumor grade and the extent of surgical resection predict outcome.9
- Most astrocytomas are low grade and surgically curable if they can be completely resected.3
- Patients with astrocytomas and hemangioblastomas are more likely to undergo subtotal resection because of their indistinct borders.
- Ependymomas, schwannomas, and especially meningiomas are typically totally resected because of their well-defined borders.
- Ependymomas occurring in the lower half of the spinal cord have a worse prognosis; ependymomas occurring in the upper half of the spinal cord recur later and less frequently, with little or no mortality.20
Tumor recurrence usually indicates a very poor prognosis.
Low FIM or WeeFIM scores correlate with severe neurological deficits at diagnosis, such as paraparesis, but not with tumor histology or localization, age at diagnosis, pre-diagnostic symptomatic interval, or the management of spinal cord compression.
(see History section)
Social role and social support system
Ask about members of the child’s family and details of the home, school, and recreational environment. Peer mentorship, including a formalized parent-to-parent mentoring program, can be especially beneficial in supporting families by decreasing anxiety and social isolation.21
Rehabilitation Management and Treatments
Available or current treatment guidelines
The acute management of pediatric spinal cord tumors usually involves surgical resection plus chemotherapy and/or radiation therapy. Radiation and chemotherapy are generally added when a complete (>90%) resection is not possible. There are no standardized treatment guidelines. Inpatient versus outpatient rehabilitation may depend on the prognosis of the tumor (stage, types of treatment recommended), functional status change, goals of care and function and family needs.
General considerations for inpatient rehabilitation include:
- Stable vital signs, with the absence of fever.
- Platelet levels greater than 50,000 to avoid bleeding with exercise.
- The need for isolation can be a barrier in children with low absolute neutrophil counts (ANC). As such, these children and adolescents may be better served with private therapies until they are more stable and ready for intensive inpatient rehabilitation.
Children and adolescents with spinal cord tumors and their families will need ongoing counseling and psychological support throughout the treatment process.
Management at different disease stages:
The goals of rehabilitation are to maximize function through individualized therapy programs, education of compensatory strategies, and provision of equipment, while improving the quality of life and reducing caregiver burdens.
- Rarely encountered.
- Goals are to maintain or improve strength and aerobic capacity.
- Individualized interdisciplinary therapy programs are designed.
- Physiatrists manage spasticity, neuropathic and musculoskeletal pain.
- Rehabilitation nursing educates patients and families on neurogenic bowel and bladder programs.
- Gait aids, lower limb orthoses, and mobility devices are provided to maximize mobility.
- Functional or resting upper limb orthoses may be needed to maximize function or prevent deformity.
- Assistive devices are provided to maximize independence in self-care and higher-level ADLs.
- Swallow assessments are necessary for children/adolescents with signs or symptoms of dysphagia.
- Augmentative alternative communication systems may be needed.
- Nutritional assessment for optimal weight management and recovery is essential.
- Early rehabilitation post-surgical resection has been shown to improve functional outcomes and should be considered.22
- Outpatient therapy programs are designed to condition, strengthen, and maximize return to home, recreation, and school environments.
Terminal or Palliative Phase
- Still some attainable functional goals.
- The focus is mainly on symptom control, family education, and additional assistive devices and medical equipment.
- Coordination with the Palliative Care Team is essential.
Coordination of care
Interdisciplinary care is crucial and often involves pediatric physiatrists, rehabilitation nursing, physical therapists, occupational therapists, speech therapists, rehabilitation psychologists, cognitive and/or educational specialists, nutritionists, art and music therapists, social workers, case managers, child life specialists, and chaplains. Support from consulted or treating neurosurgeons, pediatric oncologists, radiation oncologists, pediatric neuroradiologists, pediatric pharmacologists, and other pediatric tumor experts is essential.
Choosing a center with pediatric-accredited neurosurgeons well-versed in pediatric spinal cord tumors has a positive effect on outcomes.
Patient & family education
Clinicians should educate patients and their families about postsurgical outcomes and the role of rehabilitation in helping to improve neurological function. After spinal cord tumor resection, most patients function independently. However, functional impairments are not uncommon, especially in those presenting with advanced tumor progression.18 Most often children may have lower limb weakness and abnormal tone. Involving rehabilitation team to address equipment and orthotic needs, therapy needs and possible medication for managing hypertonia and/or pain would be essential to maximize function and quality of life. Engaging school by educating them on the new diagnosis, discussing potential establishment of 504 plan or an IEP plan would be essential to facilitate return to school.
- American Childhood Cancer Organization (national resource for free webinars, face-to-face support groups; https://www.acco.org/)
- Childhood Cancer International (international resource, https://www.childhoodcancerinternational.org)
- Pediatric Oncology Resource Center, founded by parents of children with cancer, at http://www.ped-onc.org/cfissues/cfissues.html
The molecular histopathology of spinal cord tumors is being explored to tailor chemotherapy.
Translation into practice: practice “pearls”/performance improvement in practice (PIPs)/changes in clinical practice behaviors and skills
Communicate regularly with oncological, neurosurgical, and orthopedic specialists to maximally manage patient care. Unique rehabilitation services may be needed at different disease stages, and early rehabilitation should be considered post-surgery. Additionally, counseling, especially family counseling, should be offered to help children, siblings, and parents adjust and cope with possible changing disabilities and end-of-life cares.
Cutting Edge/ Emerging and Unique Concepts and Practice
Cutting edge concepts and practice
Modern advances in microsurgical techniques have allowed greater and more aggressive resection of spinal cord tumors.12 Intra-operative muscle and epidural electrode motor evoked potentials (MEPs) best guide the degree of resection. Targeted and conformal radiotherapy techniques and adjuvant chemotherapy have reduced the doses of craniospinal radiotherapy administered. Immunotherapy trials for pediatric brain and spinal cord tumors are also being investigated.23 Databases of currently ongoing clinical trials can be viewed at www.clinicaltrials.gov and www.curesearch.org.
In 2016, the WHO revised its classification of Tumors of the Central Nervous System (last 2007) and added molecular parameters and histology to define and diagnose many tumor entities. This system includes a simplified algorithm for the classification of diffuse gliomas based on histological and genetic features. Notably, this new system affects brain tumors more than the very rare spinal cord tumors in children. However, the goal of this new classification system is to improve classification in clinical and experimental trials, allow for more precise epidemiological categorization, and eventually lead to better tailoring of patient therapy.24
Gaps in the Evidence-Based Knowledge
Further study in the molecular biomarkers in children with spinal cord tumors could explain tumor predilection and malignant transformation in a still-developing nervous system, and enable targeted treatments to these children, allowing improved outcomes and survival.
- Kaplan N, Kwok C, Alejandro RE, Berlin H. Rehabilitation of the Child with Brain and Spinal Cord Cancer. In: Cristian A, editor. Central Nervous System Cancer Rehabilitation, Elsevier; 2019, p. 107–20.
- Ostrom QT, Patil N, Cioffi G, Waite K, Kruchko C, Barnholtz-Sloan JS. CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2013-2017. Neuro Oncol 2020;22:iv1–96. https://doi.org/10.1093/neuonc/noaa200.
- Wilson PE, Oleszek JL, Clayton GH. Pediatric spinal cord tumors and masses. J Spinal Cord Med 2007;30 Suppl 1:S15–20. https://doi.org/10.1080/10790268.2007.11753963.
- Duong LM, McCarthy BJ, McLendon RE, Dolecek TA, Kruchko C, Douglas LL, et al. Descriptive epidemiology of malignant and nonmalignant primary spinal cord, spinal meninges, and cauda equina tumors, United States, 2004-2007. Cancer 2012;118:4220–7. https://doi.org/10.1002/cncr.27390.
- Grimm S, Chamberlain MC. Adult primary spinal cord tumors. Expert Rev Neurother 2009;9:1487–95. https://doi.org/10.1586/ern.09.101.
- Binning M, Klimo P, Gluf W, Goumnerova L. Spinal Tumors in Children. Neurosurg Clin N Am 2007;18:631–58. https://doi.org/10.1016/j.nec.2007.07.001.
- Shweikeh F, Quinsey C, Murayi R, Randle R, Nuño M, Krieger MD, et al. Treatment patterns of children with spine and spinal cord tumors: national outcomes and review of the literature. Childs Nerv Syst 2017;33:1357–65. https://doi.org/10.1007/s00381-017-3433-y.
- Winograd E, Pencovich N, Yalon M, Soffer D, Beni-Adani L, Constantini S. Malignant transformation in pediatric spinal intramedullary tumors: case-based update. Childs Nerv Syst 2012;28:1679–86. https://doi.org/10.1007/s00381-012-1851-4.
- Scheinemann K, Bartels U, Huang A, Hawkins C, Kulkarni AV, Bouffet E, et al. Survival and functional outcome of childhood spinal cord low-grade gliomas. Clinical article. J Neurosurg Pediatr 2009;4:254–61. https://doi.org/10.3171/2009.4.PEDS08411.
- Wilne S, Collier J, Kennedy C, Koller K, Grundy R, Walker D. Presentation of childhood CNS tumours: a systematic review and meta-analysis. Lancet Oncol 2007;8:685–95. https://doi.org/10.1016/S1470-2045(07)70207-3.
- Huisman TAGM. Pediatric tumors of the spine. Cancer Imaging 2009;9 Spec No A:S45–8. https://doi.org/10.1102/1470-7330.2009.9012.
- 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 2008;1:63–7. https://doi.org/10.3171/PED-08/01/063.
- Houten JK, Cooper PR. Spinal cord astrocytomas: presentation, management and outcome. J Neurooncol 2000;47:219–24. https://doi.org/10.1023/a:1006466422143.
- Townsend N, Handler M, Fleitz J, Foreman N. Intramedullary spinal cord astrocytomas in children. Pediatr Blood Cancer 2004;43:629–32. https://doi.org/10.1002/pbc.20082.
- Franjoine MR, Gunther JS, Taylor MJ. Pediatric balance scale: a modified version of the berg balance scale for the school-age child with mild to moderate motor impairment. Pediatr Phys Ther 2003;15:114–28. https://doi.org/10.1097/01.PEP.0000068117.48023.18.
- Kleinschmidt-DeMasters BK, Donson AM, Richmond AM, Pekmezci M, Tihan T, Foreman NK. SOX10 Distinguishes Pilocytic and Pilomyxoid Astrocytomas From Ependymomas but Shows No Differences in Expression Level in Ependymomas From Infants Versus Older Children or Among Molecular Subgroups. J Neuropathol Exp Neurol 2016;75:295–8.
- 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 1996;85:1036–43. https://doi.org/10.3171/jns.19126.96.36.1996.
- 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 2000;93:183–93. https://doi.org/10.3171/spi.2000.93.2.0183.
- 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 2009;95:259–69. https://doi.org/10.1007/s11060-009-9925-1.
- Oh MC, Sayegh ET, Safaee M, Sun MZ, Kaur G, Kim JM, et al. Prognosis by tumor location for pediatric spinal cord ependymomas: Clinical article. J Neurosurg Pediatr 2013;11:282–8. https://doi.org/10.3171/2012.11.PEDS12292.
- Berry-Carter K, Barnett B, Canavera K, Baker JN, Mandrell BN. Development of a Structured Peer Mentoring Program for Support of Parents and Caregivers of Children with Cancer. J Pediatr Nurs 2021;59:131–6. https://doi.org/10.1016/j.pedn.2021.03.031.
- Kose N, Muezzinoglu K, Bilgin S, Karahan S, Isikay I, Bilginer B. Early rehabilitation improves neurofunctional outcome after surgery in children with spinal tumors. Neural Regeneration Res 2014;9:129. https://doi.org/10.4103/1673-5374.125340.
- Vitanza NA, Johnson AJ, Wilson AL, Brown C, Yokoyama JK, Künkele A, et al. Locoregional infusion of HER2-specific CAR T cells in children and young adults with recurrent or refractory CNS tumors: an interim analysis. Nat Med 2021:1–9. https://doi.org/10.1038/s41591-021-01404-8.
- 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 2016;131:803–20. https://doi.org/10.1007/s00401-016-1545-1.
Original Version of the Topic
Colleen A. Wunderlich, MD. Pediatric spinal cord tumors. 9/20/2013.
Previous Revision(s) of the Topic
Colleen A. Wunderlich, MD. Pediatric spinal cord tumors. 3/29/2017.
Mi Ran Shin, MD
Nothing to Disclose
Spencer Brodsky, MD
Nothing to Disclose
Olga Morozova, MD
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