Cranial chordomas in children and adolescents

Chordomas are rare tumors that usually occur in adults. This report describes four cases of intracranial chordomas treated in patients 20 years of age or younger by the senior author (O.A.M.) during a 4-year period. The authors also reviewed the literature on pediatric patients, which revealed that the clinical presentations, histological patterns, and behaviors of these tumors differ considerably depending on whether the patient is younger or older than 5 years of age. The younger population had a wider range of presenting symptoms, a greater prevalence of atypical histological findings with aggressive behavior, a greater range of cellularity than the classic chordomas, and a higher instance of metastasis; it showed no chondroid component compared to a 17.1% instance in the older patients. The prognosis for patients with a chordoma is related directly to the histological pattern of the tumor; the atypical chordoma carries a poor prognosis. The prognoses for children older than 5 years of age with a classic or chondroid tumor were not significantly different (p=0.788). At follow up, the difference in survival rates between patients undergoing surgery plus radiation therapy and those who had surgery alone was statistically significant (p=0.00446). No correlation was found between radical resection or radiation therapy and an improved prognosis for patients younger than 5 years of age who had a tumor with an atypical histological pattern. This study identifies and delineates the distinction between these age groups and provides a review of the potential prognostic factors.