What Is Childhood Chordoma ?

Childhood chordoma is a rare malignant bone tumor that develops from remnants of the notochord — an embryonic structure that forms the foundation of the spine during fetal development. While chordoma is more commonly diagnosed in adults between 40 and 70 years of age, it can occur in children and adolescents. Pediatric chordoma behaves differently from adult forms in certain cases and requires specialized multidisciplinary cancer care.

Chordomas are slow-growing yet locally invasive neoplasms. Most chordomas are located within the axial skeleton and commonly occur in the clivus region of the skull base and the sacrum of the lower spine. As the lesion tends to be deeply seated around sensitive neural and vascular structures, total excision may prove difficult.

What are the Main Types of Pediatric Chordoma?

Chordomas are cancers that arise from notochordal cells that remain in the spinal cord after its formation. They tend to form along the midline of the body and commonly affect:

• Skull base (clival chordoma)
• Cervical spine
• Thoracic spine (rare in children)
• Sacrum

In children, skull base chordomas are more common than sacral chordomas. Pediatric cases represent a very small percentage of all bone cancers.

Chordomas are classified as:

• Conventional chordoma
• Chondroid chordoma
• Poorly differentiated chordoma (more aggressive and more common in younger children)

Poorly differentiated chordoma tends to be more aggressive and may require more intensive treatment.

What are the Symptoms & Causes of Childhood Chordoma?

Causes

It is not entirely known what causes chordoma. The condition arises from the remnants of the notochordal cells that do not disappear after birth. Occasionally, a genetic mutation in the SMARCB1 gene causes chordoma.

Unlike many adult cancers, environmental or lifestyle factors are not linked to childhood chordoma.

Symptoms

Symptoms depend on tumor location and size.

Skull Base Chordoma Symptoms

• Persistent headache
• Double vision
• Difficulty swallowing
• Facial numbness
• Hearing changes
• Balance problems
• Weakness in limbs

Spinal or Sacral Chordoma Symptoms

• Back pain
• Lower limb weakness
• Bowel or bladder dysfunction
• Numbness in the legs
• Difficulty walking

Because these tumors grow slowly, symptoms may progress gradually over months.

How Is Childhood Chordoma Diagnosed and Evaluated?

Early diagnosis requires imaging and tissue confirmation.

Physical and Neurological Examination

A detailed neurological assessment helps determine cranial nerve or spinal involvement.

Imaging Tests

MRI

MRI is the most important imaging tool. It provides a detailed visualization of tumor extent and involvement of nearby structures.

CT Scan

CT scans are performed to assess bone damage and for surgical planning.

PET Scan

It can be performed to assess metastasis; however, this is less likely to occur initially.

Biopsy

A biopsy is essential for establishing the diagnosis since it involves an examination by cancer specialists to determine:

• Classic physaliphorous cells (in conventional chordoma)
• Immunohistochemical markers
• Genetic alterations

Because of the tumor location, the biopsy must be carefully planned to avoid spreading tumor cells.

What are the Stages of Childhood Chordoma?

Chordomas lack a conventional staging system, unlike other malignancies. Treatment options will depend upon:

• Tumor size
• Invasive features
• Ability to surgically remove the tumor
• Metastasis (which is rare but may occur)

Metastases may develop in the lungs, lymph nodes, or bones in children with a more aggressive tumor.

How Is Childhood Chordoma Treated ?

Management of chordoma in children needs a multidisciplinary approach involving pediatric oncologists, neurosurgeons, orthopedic oncologists, radiation oncologists, and physiatrists.

The main goal is to complete tumor removal while preserving neurological function.

Surgery

Surgery is the primary treatment.

Goals of Surgery

• Achieve a total tumor resection.
• Attain negative margins.
• Preserve neurological elements.

In the case of skull base tumors, surgery can be carried out using:

• Endoscopic endonasal surgery.
• Open cranial surgery.
• Combined neurosurgical procedures.

In the case of spinal or sacral tumors, surgery can be carried out using:

• En bloc resection.
• Spinal stabilization surgery.
• Reconstruction, if needed.

Complete removal leads to a substantial improvement in survival rates.

Radiation Therapy

Owing to the difficulty in complete removal, radiation therapy is a very important aspect.

Proton Beam Therapy

Used in children because of its accuracy and minimal damage to surrounding tissues.

Intensity-Modulated Radiation Therapy (IMRT)

It can also be used when proton therapy is unavailable.

Radiation therapy is usually given after surgery to lower the chances of recurrence.

Chemotherapy

Conventional chemotherapy is not very effective in the case of chordoma.

However, in poorly differentiated chordoma, chemotherapy can be an option, especially in young children.

Targeted Therapy

The studies underway are seeking substances that disrupt signaling pathways involved in chordoma development, following which the medical professionals employ a targeted approach to treat cases of chordoma recurrence and metastasis.

What Supportive Services Are Required Post-Treatment?

Post-treatment monitoring is critical because patients have a high chance of recurrence.

Follow-up care:

• MRI scans every 3-6 months initially
• Neurological examination
• Endocrine examination (if the pituitary gland is affected due to involvement of the skull base)

The reason follow-up care is significant is that recurrence may happen several years after primary treatment.

Recovery

Factors affecting recovery include:

• Tumor location
• Surgical extent
• Neurological involvement
• Effects of radiation

Children may require:

• Physical therapy
• Speech therapy
• Occupational therapy
• Endocrine management

Emotional and psychological support is also important.

What Complications Can Arise from Chordoma Treatment?

• Neurological dysfunction
• Hormonal dysfunction
• Tumor regrowth
• Side effects from radiation
• Spinal instability
• Infection
• CSF leakage (skull base surgery)

Prompt treatment can help overcome these complications.

What Is Prognosis of Childhood Chordoma?

Prognosis depends on:

• Completeness of surgical removal
• Tumor subtype
• Response to radiation
• Presence of metastasis

Children with completely resected tumors followed by radiation have better outcomes. Poorly differentiated chordoma may have a more aggressive course.

Survival rates are inconsistent, and the risk of recurrence remains a concern.

Why Chordoma needs Long-Term Follow-Up ?

Because chordoma may recur decades after the initial treatment, a lifetime of follow-up care is advised.

Follow-up includes the following:

• Routine MRI
• Neurological assessment
• Radiation changes assessment
• Developmental assessments

When Should You Seek Emergency Medical Attention for a Child with Chordoma?

The following indicates that immediate medical attention is required in children when:

• Sudden weakness
• Severe headache
• Loss of bladder or bowel control
• Seizures
• Vision changes
• Persistent vomiting

These symptoms may indicate tumor progression or complications.

Why choose Tender Palm Super Specialty Hospital for Childhood Chordoma Treatment in Lucknow, India?

Tender Palm Super-Speciality Hospital offers advanced Childhood Chordoma treatment in Lucknow, India, at an affordable cost. We have a team of experienced pediatric neurosurgeons and neuro-oncology specialists who provide accurate diagnosis and both non-surgical and surgical treatment options including en bloc surgical resection, proton beam therapy, and comprehensive oncological management procedures. Our Pediatric Neurosurgery and Neuro-Oncology team has decades of experience in successfully treating Childhood Chordoma in Lucknow, India.

To seek an Expert consultation for Childhood Chordoma Treatment in Lucknow, India:

Call us at +91-9076972161
Email at care@tenderpalm.com

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