Intracranial Chordomas:Clinical Review and Therapeutic Outcome

Kim, Choong Hyun; Ha, Young Il; Song, Kwan Young; Hahn, Yun Kyung

Abstract

Chordomas are uncommon tumors arising from the embryonic remnants of the fetal notochord. The spheno-occipital region accounts for 36 percent of chordomas. The authors are presenting four cases of intracranial chordomas with clinical manifestation was analyzed. All patients were men and their mean age was 37 years old. The presenting complaints were diplopia in 2 patients and decreased visual acuity in the order two. Radiologic studies demonstrated extensive bony destruction of the skull base and soft mass with punctate calcification in all 4 cases. Treatment consisted of subtotal removal of tumor and subsequent radiotherapy. Histopathologically, three of the four patients had typical chordomas and the remaining case was confirmed as chondroid chordoma. The three typical chordomas recurred after initial treatment. 2 of these three were reoperated but died in the 10 months and 33 months, respectively after initial diagnosis. The patient with chondroid chordoma remained well for 46 months after initial treatment without significant neurological deficits. In conclusion, therapeutic outcome of intracranial chordomas are grim because of their aggressive that, frequent recurrence and inability to remove tumors completely. With the exception, chondroid chordoma had good prognosis and long term survival.