Paediatric Chordomas

Kévin Beccaria, Christian Sainte-Rose, Michel Zerah, Stéphanie Puget

Paediatric chordomas are rare malignant tumours arising from primitive notochordal remnants with a high rate of recurrence. Only 5 % of them occur in the first two decades such less than 300 paediatric cases have been reported so far in the literature. In children, the average age at diagnosis is 10 years with a male-to-female ratio closed to 1. On the opposite to adults, the majority of paediatric chordomas are intracranial, characteristically centered on the sphenooccipital synchondrosis. Metastatic spread seems to be the prerogative of the under 5-year-old children with more frequent sacro-coccygeal locations and undifferentiated histology. The clinical presentation depends entirely on the tumour location. The most common presenting symptoms are diplopia and signs of raised intracranial pressure. Sacrococcygeal forms may present with an ulcerated subcutaneous mass, radicular pain, bladder and bowel dysfunctions. Diagnosis is suspected on computerised tomography showing the bone destruction and with typically lobulated appearance, hyperintense on T2-weighted magnetic resonance imaging. Today, treatment relies on as complete surgical resection as possible (rarely achieved because of frequent invasiveness of functional structures) followed by adjuvant radiotherapy by proton therapy. The role of chemotherapy has not been proven. Prognosis is better than in adults and depends on the extent of surgical resection, age and histology subgroup. Biological markers are still lacking to improve prognosis by developing targeted therapy.