Chordomas and chondrosarcomas of the skull base: comparative analysis of clinical results in 30 patients

Abstract

Chordomas and chondrosarcomas occur rarely in the skull base and have been often considered commonly with respect to the management. The aim of this study was to investigate the clinical results in each series of these tumors and analyze comparative outcome. Between 1991 and 2005, 30 consecutive patients with pathological diagnosis of chordoma (n=19) or chondrosarcoma (n=11) of the skull base were managed by multimodal treatment combining surgical resection with conventional photon radiotherapy and/or gamma knife radiosurgery. A retrospective analysis was conducted on these patients (aged 3 to 69 years; mean age of 37.3 years; 17 females and 13 males; mean follow-up of 56.1 months). Four deaths occurred among the patients with chordoma. The progression-free survival rate at 3 and 5 years was 61.5 and 40.0% in chordoma. In contrast, no mortalities were observed, and there was only one recurrence among the patients with chondrosarcoma. The progression-free survival rate at 3 and 5 years was 88.9 and 80.0% in chondrosarcoma. Among 15 survivors with chordoma, four patients were suffering from severe disability with progressive disease. On the other hand, most patients with chondrosarcoma harbored stable disease and less disabling symptoms except one instance of recurrence. As the biological behavior of chordoma is much more aggressive than that of chondrosarcoma in the skull base, one should make a distinction between these entities to plan an optimal treatment strategy.

Determinants of quality of life in patients with skull base chordoma

“…Despite these limitations, the study cohort showed characteristics that resemble those of skull base chordoma patients in other clinical studies including mean age at diagnosis, neurological symptoms and signs at presentation, and treatment received. 7,14,43 The physical functioning of the patients in this study was reflected by a mean KPS score of 82, which is similar to that reported in the literature for other patients with skull base chordoma (mean KPS score range 69-84). 7,14,43,60 The congruence of the study cohort clinical characteristics with published data supports the external validity of our questionnaire.…”

“…7,14,43 The physical functioning of the patients in this study was reflected by a mean KPS score of 82, which is similar to that reported in the literature for other patients with skull base chordoma (mean KPS score range 69-84). 7,14,43,60 The congruence of the study cohort clinical characteristics with published data supports the external validity of our questionnaire. Internal validity was supported using previously validated functional and quality of life outcome instruments.…”

Skull base chordomas: clinical outcome in a consecutive series of 45 patients with long-term follow-up and evaluation of clinical and biological prognostic factors

“…8,18 In our series the mean best postoperative KPS score recorded at follow-up was 92.84 (range 60-100), higher than the score recorded before surgery. These data suggest that the overall impact of surgery on patient quality of life seems to be positive or at least not detrimental.…”

“…patient population, tumor location, and extension In our series epidemiological data are comparable to those reported in the literature (Table 4): we found a male predilection with a male/female ratio of 2:1 8,30,38 and an onset peak age of 46.7 years. 1,8,10,11,18,19,49,53 Whether sex and age can affect prognosis is still a matter of debate. We did not find any significant correlation between patient sex and survival.…”

Sphenoid Masses in Children: Radiologic Differential Diagnosis with Pathologic Correlation

“…24 Treatment involves maximum safe resection along with high-dose radiation therapy via proton beam radiation or radiosurgery. 43 Craniopharyngioma Craniopharyngioma (Fig 9) is a slow-growing benign tumor arising from retained elements of the Rathke pouch, the embryologic forerunner of the anterior pituitary gland. 44 There is a bimodal age distribution: school-age children and adults 50 -60 years of age.…”