Chondrosarcomas

Cranial chondrosarcomas are rare tumors of adult life and are occasionally difficult to differentiate from chordoma. Their preferred site of origin is the sphenoid bone, particularly in or near the sella turcica. Additional favored sites include the petrous temporal bone and clivus. Most chondrosarcomas of the skull base are primary lesions, although a minority may arise from preexisting chon-dromas or enchondromas of Ollier's disease and the related Maffucci's syndrome. Because these tumors are slow growing, local pain of long duration is the most common and frequently the only presenting complaint. With time, additional symptoms of mass effects, including cranial nerve palsies and occasion ally pituitary dysfunction, emerge. Metastases, typically to the lungs, occur primarily in high-grade tumors. Bony destruction is a uniform radiologic feature; fine stippled calcification is often characteristic. CT and MRI define the extent of the soft tissue mass. Radical surgical excision is the treatment of choice, whose curability diminishes as tumor size and grade increase. Periodic recurrence is frequent and often necessitates subsequent surgical procedures. Although most often used for incompletely resected, recurrent, and high-grade tumors, the effectiveness of radiation therapy has yet to be established. The prognosis for these tumors is variable, with 5-yr survival rates approaching 60% (114).

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