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Chondrosarcoma arising in the head and neck and craniofacial region is an uncommon lesion. The nasal septum is a particularly rare site of origin, with approximately 30 cases previously reported in the English literature. We present six new cases of chondrosarcoma arising in the nasal septum. Each of these tumors required cranial base surgical approaches for removal. Current imaging techniques allow a very accurate diagnosis to be made before biopsy. The characteristic ring-forming calcifications seen on computed tomography scans can be correlated with the histologic pattern of calcification. Magnetic resonance imaging techniques allow precise definition of tumor extent, which is particularly important because the disease is best treated with primary surgery. Advances in imaging and surgical techniques allow a much more complete tumor removal. It is hoped that this will increase the likelihood of cure in these patients. Surgical management and indications for adjuvant therapy are discussed.

Craniopharyngiomas (CP), Rathke's cleft cysts (RCC), and sellar xanthogranulomas (XG) are closely related lesions. As expression of cytokeratins 8 (CK8) and 20 (CK20) was reported in RCC but not in CP, the present study investigates the reproducibility of immunohistochemical distinction between CP and RCC, attempting to identify the relationship of XG to these lesions. A comparative study of 55 patient specimens (25 CP, 28 RCC, and 2 XG) was analyzed for the histological features of xanthomatous changes and squamous metaplasia, and expression of CK8 and CK20. In the 25 CP cases, xanthomatous changes were seen in 5 (20%), with CK8 reactivity demonstrated in all 25 cases. A prominent xanthomatous component was identified in 13 of 28 RCC (46%), and squamous metaplasia was seen in 11 (39%), 9 of which also contained xanthomatous features. CK8 reactivity was demonstrated in all 28 RCC cases, whereas CK20 was seen only in 9 cases (32%). Of the two cases diagnosed as XG, none contained epithelium, and immunohistochemistry for cytokeratins was not observed. Overall, differential expression of cytokeratins cannot reliably distinguish CP from RCC. Furthermore, expression of CK20 in RCC is generally seen within a background of prominent squamous metaplasia and reactive xanthomatous changes.