Paediatric Chordomas.

Area of Science:

• Pediatric Oncology
• Neurosurgery
• Radiotherapy

Background:

• Paediatric chordomas are rare malignant tumors originating from notochordal remnants.
• They have a high recurrence rate and fewer than 300 cases have been reported in children.
• Unlike adults, most paediatric chordomas occur intracranially, typically at the sphenooccipital synchondrosis.

Purpose of the Study:

• To summarize the characteristics, diagnosis, and treatment of paediatric chordomas.
• To highlight the differences in presentation and location between paediatric and adult chordomas.
• To discuss current treatment strategies and the need for improved prognostic markers.

Main Methods:

• Review of existing literature on paediatric chordoma cases.
• Analysis of clinical presentation based on tumour location.
• Description of diagnostic imaging techniques (CT, MRI).
• Summary of current treatment modalities including surgery and proton therapy.

Main Results:

• Paediatric chordomas predominantly affect children around age 10, with a 1:1 male-to-female ratio.
• Intracranial locations (sphenooccipital) are most common in children, while sacro-coccygeal are more frequent in younger children (<5 years) with metastatic potential.
• Common symptoms include diplopia and signs of increased intracranial pressure; sacrococcygeal forms present with masses, pain, and bowel/bladder dysfunction.
• Diagnosis relies on CT and MRI findings showing bone destruction and characteristic imaging features.
• Treatment involves maximal surgical resection followed by adjuvant proton therapy; chemotherapy has not proven effective.

Conclusions:

• Paediatric chordomas require specialized management due to their rarity and location.
• Prognosis is generally better than in adults but depends on surgical extent, age, and histology.
• Further research into biological markers is crucial for developing targeted therapies and improving long-term outcomes.