Cranial Chordoma ; Clinical Presentation and Multimodality Treatment.

Oh, Seok Kwan; Kim, Jung Hoon; Ra, Young Shin; Kim, Chang Jin; Kwon, Yang; Rhim, Seung Chul; Lee, Jung Kyo; Kwon, Byung Duk; Whang, C Jin

Original Article

Journal of Korean Neurosurgical Society 1996;25(5): 962-969.

Seok Kwan Oh, Jung Hoon Kim, Young Shin Ra, Chang Jin Kim, Yang Kwon, Seung Chul Rhim, Jung Kyo Lee, Byung Duk Kwon, C Jin Whang

Department of Neurological Surgery, Asan Medical Center, College of Medicine, University of Ulsan, Seoul, Korea.

ABSTRACT

Cranial chordomas are rare and generally slow-growing malignant neoplasms of presumed notochordal origin. They seldom metastasize, but are difficult to manage because of their locally invasive nature and their proximity to critical structures. The clinical presentation and results of operative treatment, radiation therapy, and radiosurgery in a series of 10 patients with cranial chordomas seen at our hospital, between June 1989 and December 1994, are analysed. There were 4 men and 6 women with a mean age of 37.5 years. The most common presenting symptoms were visual loss, motor weakness and diplopia, and the most common presenting sign was visual field defect. The mean interval between symptom onset and initial treatment was 4.8(1-17) months. Three tumors classified as the chondroid type showed better clinical course. Extent of tumor resection included biopsy in 1 patient and subtotal or greater in 9. Four patients received postoperative radiation therapy and 4 patients stereotactic radiosurgery using Leksell gamma unit. Among them 1 patient showed tumor progression after radiation therapy but none after radiosurgery. One patient died due to tumor recurrence and two patients due to therapy but none after radiosurgery. One patient died due to tumor recurrence and two patients due to postoperative complications. At the time of analysis 7 patients were alive. The average length of follow-up for the alive patients was 34 months. The results of this study suggest that multimodality treatment using surgical debulking, radiation therapy and stereotactic radiosurgery is necessary for the optimal management of chordoma.

Key Words: Chordomas; Cranial; Chondroid; Surgery; Radiation therapy; Radiosurgery; Multimodality treatment