Spinal cord tumors are very rare neoplasms in children. Unlike 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
Primary pediatric spinal cord tumors account for 0.5-10% of all pediatric CNS tumors.1
Low-grade gliomas, which include astrocytomas, gangliomas, oligodendrogliomas, mixed gliomas, and other rare glial tumors, are together the most common CNS neoplasms,2 but pediatric spinal cord gliomas are rare, accounting for only 6.3% of all
primary spinal cord tumors.3 High-grade gliomas are extremely rare, accounting for 10-15% of all pediatric spinal cord gliomas.4
The predominant histology of spinal tumors for children 0 – 19 years of age is ependymal tumors (21%), then other astrocytomas, including glioblastoma (20.5%).13
Metastatic tumors are very rare in children and may occur as drop metastases from brain tumors.
There has been an increased rate of primary malignant brain and CNS tumors for children 0 – 14 years and an increased rate for primary malignant and non-malignant brain and CNS tumors for adolescents (15 – 19 years) in the last decade, while adult incidence has decreased.13
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.1 Radiation therapy may generate malignant transformation by accelerating a tumor’s natural tendency towards dedifferentiation or by engendering a denovo 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.2
Pain of bony segment directly over the tumor is the most common presenting complaint in children.
- The onset of back pain may be over months.
- Pediatric back pain should be thoroughly investigated, as it is uncommon.
Older children may also note a history of progressive motor weakness.
- Less commonly, coordination and balance impairments, gait dysfunction, or urinary incontinence are seen.
In infants, nonspecific crying episodes followed by motor weakness or torticollis may be seen.
Long-term motor and sensory neuropathies deficits are often seen post-intramedullary tumor resection in children.
- 1/3 may experience improvements in motor, sensory and urinary dysfunction years after resection.
- 2/3 experienced long-term dysesthetic symptoms that impacted quality of life.
- Older age, pre-operative symptom duration > 12 months, and greater post-operative neurological deficit increased the risk of long-term dysesthesias.5
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. 4,6
Specific secondary or associated conditions and complications
Neurofibromatosis is associated with low-grade gliomas.
Radiation and chemotherapy are added when a complete (>90%) resection is not possible.
Orthopedic morbidities, most often severe kyphoscoliosis, occurs in 35% of children treated for low-grade spinal gliomas.2 Plus, early scoliosis is the most common sign of a thoracic astrocytoma.
Significant syringes can also be found in many children with astrocytomas.
2. ESSENTIALS OF ASSESSMENT
- Ask about neurological deficits, both motor and sensory.
- Evaluate for dysphagia, neurogenic bowel and bladder, spasticity, scoliosis, syrinx.
- Take a detailed pain history.
- Ask about 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 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, most painful directly over the tumor.
Do a complete neuromuscular exam: evaluating for motor strength, coordination, reflexes, vibration, proprioception, light and dull touch is in order.
An ASIA exam, keeping in mind the child’s developmental age and need for modesty, should be done. (See http://content.learnshare.com/courses/120/394001player./html).
The rectal exam and use of suppositories in contraindicated immunocompromised children, but there is no contraindication pre-treatment.
Check for scoliosis and ensure proper healing of post-laminectomy or laminotomy healing incisions as appropriate.
- WeeFIM or FIM system
- Pediatric Evaluation of Disability Inventory (PEDI)
- Karnofsky Performance Score (KPS): used by oncologists; standard measure of cancer patient’s ability to perform ordinary tasks (see http://medicalcriteria.blogspot.com/2012/02/karnofsky-performance-status-kps-scale.html).
- Peds Quality of Life Inventory (http://www.pedsql.org).
- Modified McCormick Scale (mMS) – used by some neurosurgical studies to determine pre- and postoperative neurological function: (see ref 29 or http://www.nature.com/sc/journal/v46/n8/fig_tab/sc200851t1.html)).
Histological and molecular verification of the tumor, with World Health Organization (WHO) grading, is paramount to pathological diagnosis. Immunohistochemistry, such as SOX-10 staining, can help differentiate ependymomas from astrocytomas 12. Information on the underlying genetic abnormalities in pediatric diffuse gliomas is allowing better separation of these tumors from similar adult versions 10. Cerebral spinal fluid (CSF) studies are generally useful to to rule out other causes of spinal cord lesions such as infection, but are not diagnostic for intramedullary tumors. See treatment for other guidelines.
MRI with and without gadolinium enhancement is the procedure of choice for preoperative evaluation of patients with spinal cord tumor. Intraoperative imaging may also decrease the need for second 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 favorable.7,8,9
Tumor grade and the extent of surgical resection predict outcome.2
- 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 indistinct borders.
- Ependymomas, schwannomas, and especially meningiomas are totally resected more often because of 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.11
Tumor reoccurrence is usually a very poor prognostic indication.
(see History section)
Social role and social support system
Ask about members of child’s family, as well as details of the home, school, and recreational environment.
3. 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.
There are no standardized treatment guidelines. Inpatient versus outpatient rehabilitation is dependent on stage and type of treatment, child’s age and ability to participate in acute rehabilitation, and family needs.
General consideration for rehab 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); 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 tumors and their families will need ongoing counseling and psychological support throughout the treatment process.
Management at different disease stages:
The goal of rehabilitation is to maximize function through individualized therapy programs, education of compensatory strategies, and provision of equipment, while improving the quality of life. Reducing caregiver and societal burdens are also secondary goals.
- 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 extremity orthoses, and mobility devices are provided to maximize mobility.
- Functional or resting upper extremity orthoses may be needed to maximize function or prevent deformity.
- Assistive devices provided to maximize independence in self-cares and higher level ADLs.
- Swallow assessments are necessary for children/adolescents with signs or symptoms of dysphagia.
- Augmentative communication systems may be needed.
- Nutritional assessment for optimal weight management and recovery is necessary.
- Outpatient therapy programs designed to condition, strengthen, and maximize return to home, recreation, and school environments.
Terminal or Palliative Phase:
- Still some attainable functional goals.
- 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 that includes pediatric physiatrists, rehabilitation nursing, physical therapists, occupational therapists, speech therapists, rehabilitation psychologists, cognitive or educational specialists, nutritionists, art and music therapists, social
workers, case managers, child life specialists, and chaplains, as well as 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 outcome.
Patient & family education
Families should be encouraged to search local hospital websites and speak with local hospital social workers.
An Individualized Education Plan or 504 Plan may be needed to facilitate return to school. Neuropsychological testing is also very helpful to assess cognitive function and assist planning for school return.
Camps may be available in certain areas.
- American Childhood Cancer Organization (national resource)
- International Confederation of Childhood Cancer Parent Organization (international resource, http://icccpo.org/index.cfm ).
- Pediatric Oncology Resource Center, founded by parents of children with cancer, at http://www.ped-onc.org/cfissues/cfissues.html
- http://www.acco.org for free webinars, face-to-face support groups
Low WeeFIM or FIM scores correlated with severe neurological deficits at diagnosis, such as paraparesis, but not with tumor histology or localization, age at diagnosis, prediagnostic symptomatic interval, or the management of spinal cord compression.
Translation into practice: practice “pearls”/performance improvement in practice (PIPs)/changes in clinical practice behaviors and skills
Communicate frequently with oncological, neurosurgical, and orthopedic specialists to maximally manage patient care.
Frequent laboratory studies will be needed during rehabilitation; discuss with the treating pediatric oncologist.
Platelets greater than 50,000 are recommended to avoid bleeding during exercise.
Different rehabilitation services may be needed at different disease stages.
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.
4. 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.5
Intra-operative muscle and epidural electrode MEPs best guide the degree of resection.
Targeted and conformal radiotherapy techniques and adjuvant chemotherapy have reduced the doses of craniospinal radiotherapy administered.
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 in addition to histology to define and diagnose many tumor entities. This system includes a simplified algorithm for classification of diffuse gliomas based on histological and genetic features. Although this new system affects brain tumors more than the very rare spinal cord tumors in children, it is hoped that this new classification system will allow better classification for clinical and experimental trials, more precise epidemiological categorization, and eventually lead to better tailoring of patient therapy.10
5. GAPS IN THE EVIDENCE-BASED KNOWLEDGE
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.
- Winograd E, Pencovich N, Yalon M, Soffer D, Beni-Adani J, Constantini S. Malignant transformation in pediatric intramedullary tumors: case-based update. Childs Nerv Syst. 2012; 28:1679-1686.
- Scheinemann K, Bartels U, Huand A, et al. Survival and functional outcome of childhood spinal cord low grade gliomas. J Neurosurg Pediatrics. 2009;4:254-261.
- Engelhard HH, Villano L, Porter KR, et al. Clinical presentation, histology, and treament in 430 patients with primary tumors of the spinal cord, spinal meninges, or cauda equina. J Neurosurg Spine. 2010;13:67-77.
- Houten JK, Cooper PR. Spinal cord astrocytomas: presentation, management, and outcome. J Neuro-onc. 2000;47:219-224.
- 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 Pediatrics. 2008;1:63-67.
- Townsend N, Handler M, Fleitz J, Foreman N. Intramedullary spinal cord astrocytomas in children. Pediatr Blood Cancer. 2004;43:629 – 632.
- Constantini S, Houten J, Miller DC, et al. Intramedullary spinal cord tumors in children under the age of 3 years. J Neurosurg. 1996;85(6):1036-1043.
- Constantini S, Miller DC, Allen JC, Roeke LB, Freed D, Epstein FJ. Radical excision of intramedullary spinal cord tumors: surgical morbidity and long-term follow-up in 164 children and adults. Neurosurg. 2000;93(2 Suppl):183-193.
- Crawford JR, Zaninovic A, Santi M, et al. Primary spinal cord tumors of childhood: effects of clinical presentation. radiographic features, and pathology on survival. J Neurooncol. 2009;95:259-269.
- Louis, D.N., Perry, A., Reifenberger, G. et al. Acta Neuropathol (2016) The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary.131: 803. doi:10.1007/s00401-016-1545-1.
- Oh MC, Sayegh ET, Safaee M, Sun MZ, Kaur G, Kim JM, Aranda D, Molinaro AM, Gupta N and Parsa AT. Prognosis by tumor location for pediatric spinal cord ependymomas. J Neurosurg Pediatrics. 2013, 11:282–288.
- B.K. Kleinschmidt-DeMasters MD, Andrew M. Donson MS, Abby M. Richmond MD, Melike Pekmezci MD, Tarik Tihan MD, Nicholas K. Foreman MD. 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. Journal of Neuropathology and Experimental Neurology. First published online: 4 March 2016. 75 (4): 295-298. http://dx.doi.org/10.1093/jnen/nlw010.
- Quinn T. Ostrom, Haley Gittleman, Jordonna Fulop, Max Liu, Rachel Blanda, Courtney Kromer, Yingli Wolinsky, Carol Kruchko, and Jill S. Barnholtz-Sloan.CBTRUS Statistical Report: Primary Brain and Central Nervous System Tumors Diagnosed in the United States in 2008-2012. Neuro Oncol (2015) 17 (suppl 4): iv1-iv62.
Original Version of the Topic
Colleen A. Wunderlich, MD. Pediatric spinal cord tumors. 09/20/2013.
Colleen A. Wunderlich, MD
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