Childhood Chordoma

Childhood chordoma is a rare bone cancer that affects the spine and skull base. Although more prevalent in adults, chordomas can also be found in children and prove challenging to treat because of their location and slow growth. This blog discusses the causes, symptoms, diagnosis, treatment, and research being conducted on childhood chordoma.

Childhood chordoma is an unusual and serious disease that demands specialized treatment and ongoing care. Although improvements in medical science and therapy are enhancing survival, early diagnosis and a complete care plan are essential to the management of the condition. Families dealing with this issue should approach support and remain knowledgeable about current advancements in chordoma science.

The reason for childhood chordoma is not known. But scientists think that it could be due to genetic mutations in the cells that are derived from the notochord. In some instances, it has been linked to inherited conditions, including tuberous sclerosis, but these are uncommon. Chordoma does not seem to be caused by environmental or lifestyle factors, unlike most other cancers.

Symptoms of childhood chordoma vary depending on the tumor’s location and size. Common signs include:

• Persistent headaches
• Neck or back pain
• Difficulty swallowing
• Vision problems (blurred or double vision)
• Hearing loss
• Numbness or weakness in the arms or legs
• Changes in bowel or bladder function
• A lump or mass near the spine

Since these symptoms can mimic other conditions, diagnosing chordoma can be challenging.

Diagnosing childhood chordoma requires a combination of imaging tests and biopsy procedures. Key diagnostic steps include:

• Magnetic Resonance Imaging (MRI): Provides detailed images of soft tissues and tumors.
• Computed Tomography (CT) Scan: Helps visualize the extent of bone involvement.
• Biopsy: A small tissue sample is taken from the tumor for pathological examination to confirm the diagnosis.
• Genetic Testing: In some cases, genetic analysis of the tumor cells may be performed to identify mutations that could influence treatment.

Treatment for childhood chordoma typically involves a combination of surgery, radiation therapy, and targeted therapies.

1. Surgery: The primary treatment option is surgical removal of the tumor. However, due to its location near vital structures, complete removal can be challenging.
2. Radiation Therapy: Proton beam therapy or intensity-modulated radiation therapy (IMRT) is used to target residual tumor cells after surgery.
3. Targeted Therapy: Drugs that target specific genetic mutations in chordoma cells are being explored as potential treatments.
4. Chemotherapy: While not commonly used for chordoma, certain chemotherapy drugs may be considered in cases of aggressive or metastatic disease.
5. Supportive Care: Physical therapy, pain management, and psychological support help improve the quality of life for affected children.

Currently, there are no known methods for preventing childhood chordoma, as the exact cause remains unclear. Early detection and prompt treatment can help manage the disease and prevent complications.

The prognosis for childhood chordoma varies depending on several factors, such as the location, size, and degree of surgical resection. Although chordomas are indolent, they have a high rate of recurrence. Improved survival rates are seen with current surgical methods and radiation therapy, but long-term follow-up is necessary.

Living with chordoma requires a multidisciplinary approach, including:

• Regular follow-ups with oncologists and neurosurgeons
• Physical rehabilitation to regain strength and mobility
• Psychological support for both the child and family
• Nutritional support to maintain overall health

Ongoing research efforts are focused on:

• Genetic studies to understand mutations driving chordoma development
• Immunotherapy trials exploring immune-based treatments
• New targeted therapies designed to attack specific proteins involved in tumor growth
• Advances in surgical techniques to improve tumor removal with minimal complications

Families affected by childhood chordoma can find support through various organizations, including:

• Chordoma Foundation (www.chordomafoundation.org)
• National Cancer Institute (www.cancer.gov)
• Pediatric Brain Tumor Foundation (www.curethekids.org)
• Local support groups for parents and caregivers