Conventional external radiotherapy in the management of clivus chordomas with overt residual disease

Cranial chordomas are uncommon tumors accounting for less than 1% of all in tracranial neoplasms. Although they are slowly growing, rarely metastasizin g tumors, cranial chordomas are challenging to treat due to their critical location, invasive nature and aggressive recurrence. The aim of this retros pective study was to evaluate the role of conventional irradiation in the t reatment of clival chordomas with overt residual disease after incomplete s urgery. Between January 1979 and December 1997, 18 patients with histologically con firmed clival chordoma were treated with radiotherapy. Median age at the ti me of diagnosis was 32 years. The mean duration of the symptoms before diag nosis was 33.9 months. Median tumor diameter at initial presentation was 5 cm (range, 3-7 cm). The type of surgical procedure was subtotal excision in 11 patients and biopsy in 7. Radiation treatment was delivered with megavo ltage units, and total doses between 50 Gy and 64 Gy (median, 60 Gy) were a dministered with conventional daily fractions. One patient received additio nal 12.50 Gy with linear accelerator-based stereotactic radiosurgery after subtotal excision and external irradiation. The mean follow-up time was 43.2 months. Overall sur vival at 5 years was 3 5%. Eleven patients showed progression after radiotherapy. The median time to progression after radiotherapy was 40.8 months (38.4-43.2) with a 5-year progression-free survival of 23%. Five patients (29.4%) showed symptomatic relief after radiotherapy while persistent symptoms were recorded for 6 pa tients. incomplete surgery and conventional external radiotherapy with a do se of around 60 Gy seem to be inadequate in the treatment of clival chordom as.