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Acıbadem Üniversitesi, Tıp Fakültesi, Nöroşirürji Anabilim Dalı, İstanbul, Türkiye Chordomas are slow-growing, locally aggressive tumors thought to originate from the embryonal notochord. They can be seen along the entire spine from the cranial base to the sacrum and have high recurrence rates and can also rarely metastasize despite having a benign character. They can be evaluated in two groups regarding clinical behavior as slow growth and rapid/aggressive growth. Chordomas of the cranial base can lead to cranial nerve findings. The most common symptoms are diplopia and headache. The most effective treatment approach is surgical excision but the infiltrative-destructive nature of the tumor, proximity to neurovascular structures, and the large size at the time of diagnosis make oncological resection impossible for some cases. Adjuvant treatment is needed in the majority of the patients. Heavy particle radiation and radiosurgical methods are reported to be effective for the treatment of residual/recurrent tumors. Anahtar Kelimeler : Chordoma, Chondrosarcoma, Skull base surgery