CRANIAL CHORDOMAS IN CHILDREN AND ADOLESCENTS

Chordomas are rare tumors that usually occur in adults. This report de scribes four cases of intracranial chordomas treated in patients 20 ye ars of age or younger by the senior author (O.A.M.) during a 4-year pe riod. 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 whethe r the patient is younger or older than 5 years of age. The younger pop ulation 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 instanc e of metastasis; it showed no chondroid component compared to a 17.1% instance in the older patients. The prognosis for patients with a chor doma is related directly to the histological pattern of the tumor, the atypical chordoma carries a poor prognosis. The prognoses for childre n 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 ther apy and those who had surgery alone was statistically significant (p = 0.00446). No correlation was found between radical resection or radia tion therapy and an improved prognosis for patients younger than 5 yea rs of age who had a tumor with an atypical histological pattern. This study identifies and delineates the distinction between these age grou ps and provides a review of the potential prognostic factors.