Intramedullary Spinal Cord Tumors in Children

What are intramedullary spinal cord tumors in children?

Tumors that develop within the spinal cord itself, with the potential to infiltrate, compress, and injure motor and sensory pathways.

Intramedullary Spinal Cord Tumors (IMSCTs) are neoplasms that arise within the substance of the spinal cord, in contrast to extramedullary tumors (such as meningiomas or schwannomas) that compress the cord from the outside.

In children, they are rare—approximately 4–6% of all pediatric CNS tumors—but they account for roughly 55% of intradural tumors in this age group. They most often occur in the cervical and thoracic spine and less commonly in the lumbar/sacral region.

Because space within the spinal cord is limited, even relatively small tumors can cause significant neurological symptoms. For this reason, early diagnosis and treatment are critical.

How common are they, and which tumor types occur in children?

Rare entities with a characteristic pediatric profile—most commonly astrocytoma and ependymoma.

Intramedullary tumors in children have an estimated incidence of about 1 per 100,000 and occur at similar rates in boys and girls.

The two most common types are:

• Astrocytomas (~60%): often located in the cervicothoracic region. Low-grade tumors (e.g., pilocytic astrocytoma) are common in children and generally have a much better prognosis.
• Ependymomas (~12–16%): typically central within the cord, more sharply demarcated, also with a preference for the cervical spine.

Less commonly, children may develop gangliogliomas, oligodendrogliomas, hemangioblastomas, or rarer entities such as PNET, neurocytoma, teratoma, and others.

Which symptoms should raise concern?

Progressive spine pain, limb weakness, gait changes, or disturbances in bladder/bowel control.

The clinical picture depends on the tumor’s location, size, and growth rate. Symptoms often evolve slowly and subtly over months.

Common symptoms and signs include:

• Pain in the neck, thoracic spine, or lower back—often worse at night or with movement.
• Weakness in the arms and/or legs, difficulty running, or trouble walking on uneven ground.
• Gait changes (instability, dragging a foot, frequent tripping).
• Sensory disturbances (numbness, tingling, or dysesthesias along the spine or in the limbs).
• New or worsening scoliosis, particularly with thoracic tumors.
• With tumors near the conus: bladder or bowel dysfunction (rare as an early symptom, more often later).

Any child with persistent spinal pain, unexplained weakness, progressive scoliosis, or a change in gait should be evaluated by a pediatric neurologist or pediatric neurosurgeon, and an MRI of the spine should be obtained when indicated.

Why do they occur—are they hereditary?

Most cases are sporadic, but a small subset is associated with genetic syndromes.

In the vast majority of children, intramedullary tumors are sporadic and are not related to diet, trauma, stress, or anything a family did or did not do.

In a small percentage, tumors may be linked to hereditary syndromes, such as:

• Neurofibromatosis type 2 (NF2) – particularly associated with ependymomas.
• Li-Fraumeni syndrome, Turcot syndrome, Tuberous sclerosis – associated with an increased risk of gliomas.

When a syndrome is suspected (multiple tumors, suggestive family history), genetic testing and specialized counseling may be recommended.

How is the diagnosis made, and what is the role of MRI?

MRI with contrast is the key test; definitive diagnosis is established through histopathology after surgery.

Spinal MRI with contrast is the imaging study of choice to confirm the diagnosis:

• Tumors often appear as spinal cord expansion with one or more enhancing lesions.
• On T2 sequences, a syrinx/hydromyelia or cysts at tumor margins are often present.
• Certain features (e.g., hemosiderin deposition) may be more consistent with ependymoma.

In specialized centers, Diffusion Tensor Imaging (DTI) and tractography can help demonstrate whether functional pathways are displaced or infiltrated, which can improve surgical planning and safety.

Definitive diagnosis (tumor type and grade) is made after histopathological and immunohistochemical analysis of resected tumor tissue.

How are they classified—what do “astrocytoma” and “ependymoma” mean?

Histology and WHO grade strongly influence prognosis and the need for additional therapies.

Broadly, we differentiate:

• Astrocytomas – often more “diffuse” and may span multiple levels. Pilocytic (grade I) and other low-grade (grade II) tumors generally have a much better outlook than anaplastic astrocytomas or glioblastomas.
• Ependymomas – more commonly central, well-demarcated, with a clearer plane from normal cord. This often makes them more suitable for near-total or gross-total resection.

The pathologist assigns a WHO grade (I–IV). In general, a lower grade and a more complete resection are associated with a better prognosis and a lower likelihood of needing radiotherapy.

What are the main treatment options?

Surgery is the cornerstone; radiotherapy and chemotherapy play supportive, case-specific roles.

Treatment is individualized, but typically includes:

• Microsurgical resection as the primary treatment, performed in a specialized pediatric center.
• Radiotherapy as adjuvant treatment in:
  • residual disease after resection,
  • higher-grade astrocytomas or malignant ependymomas,
  • tumor recurrences.
• Chemotherapy mainly as a “bridge” in very young children (< 3 years) to delay radiotherapy, or in particularly aggressive/rare tumors within specialized protocols.

Decisions regarding radiotherapy in children are made with great care, in collaboration between pediatric neurosurgery, pediatric oncology, and pediatric radiation oncology, to minimize long-term effects on spinal growth and development.

What does surgery involve—and how do we protect neurological function?

The goal is maximal safe resection with minimal neurological impact, supported by advanced technology and neuromonitoring.

Surgery is performed under general anesthesia with the child in the prone position. At Neuroknife, we emphasize:

• Osteoplastic laminotomy/laminoplasty rather than wide laminectomy, to preserve spinal stability whenever possible.
• Intraoperative ultrasound to localize the tumor precisely and optimize dural opening and myelotomy planning.
• Intraoperative neuromonitoring (MEPs, SSEPs, EMG and, when feasible, D-waves) to detect early changes and improve safety.
• Dorsal surface mapping to ensure the myelotomy is performed in the true midline, minimizing risk to posterior columns.
• Careful microsurgical technique with high-definition operating microscopes.

In selected cases—especially very extensive or multi-level operations—additional stabilization/fusion may be discussed, particularly in children with pre-existing scoliosis or higher deformity risk.

Possible complications and the postoperative course

Temporary worsening is common; permanent paralysis is fortunately rare. Rehabilitation and targeted physiotherapy are central to recovery.

Potential complications include:

• Temporary worsening of weakness or new sensory changes—common early and often improving over weeks to months.
• Permanent neurological injury (severe paresis/paralysis)—fortunately rare, but the most important risk balanced before and during surgery.
• Persistent neuropathic pain or dysesthesias.
• Bladder/bowel dysfunction, especially with lesions near the conus.
• Long term: spinal deformity/scoliosis, especially in young children, after multilevel laminotomies, or following radiotherapy.

The postoperative course often includes:

• ICU monitoring for 24–48 hours for close neurological observation,
• gradual mobilization with physiotherapy and occupational therapy,
• pediatric neurology and/or pediatric urology input when needed.

Prognosis and long-term quality of life

Outcomes depend on tumor type and grade, the completeness of resection, and the child’s neurological status before surgery.

In general, children with low-grade astrocytomas or benign ependymomas treated with extensive resection have strong chances of long-term disease control and preservation of good neurological function.

For ependymomas, gross-total resection is the most important favorable prognostic factor, with high rates of recurrence-free survival. In contrast, high-grade astrocytomas have a more aggressive course and more frequent recurrences.

In daily life, many children after successful treatment can:

• attend school normally,
• participate in supervised athletic activities,
• maintain independence in self-care, sometimes with minor adaptations.

Our goal is not only tumor control, but the best possible functional recovery and quality of life for the child and family.

Follow-up and surveillance for recurrence

Structured imaging, clinical examinations, and monitoring of growth and posture.

After initial treatment, follow-up typically includes:

• Regular spinal MRI (and brain MRI when indicated) to assess for residual disease or recurrence.
• Neurological exams (strength, sensation, reflexes, gait).
• Orthopedic surveillance and spine radiographs when needed for early detection of scoliosis or kyphosis.
• Coordination with physiotherapy and occupational therapy to support strength and mobility.

The frequency of visits and imaging is individualized based on tumor type, extent of resection, and time since treatment.