Skull base chondroid chordoma: atypical case manifesting as intratumoral hemorrhage and literature review.

Abstract

Chondroid chordoma (CC) is a rare but commonest subtype of chordoma with little reported clinical information. The present study summarizes and updates present knowledge of CC. Literature search for demographic data and clinical appearance of cranial CCs except for those entirely confined to the sinonasal region. A total of 48 English language papers published from 1968-2013 were retrieved describing 132 CCs as skull base tumors. The male-to-female ratio was 1:1. The mean age at diagnosis was 43 years, predisposing to the third to fifth decades of life. The clival (34%) and spheno-occipital (29%) regions were the most frequent sites of origin followed by the sellar (12%) and sphenoid (5%) regions. Intratumoral calcification and bony erosion were identified as the characteristic neuroimaging findings. Surgical resection by the transcranial, transsphenoidal, transnasal, transpharyngeal, or transpalatal route with or without adjuvant radiotherapy was the main treatment option. The initial treatment outcome was satisfactory in 82% of cases with considerably better prognosis compared with typical chordomas. CC is a distinct entity to be discriminated from the typical type of chordoma. There are no distinguishing features on magnetic resonance imaging between CC and typical chordoma. Intratumoral calcification and concurrent bony erosion on neuroimaging should suggest the possibility of CC. Extensive surgical resection and adjuvant radiotherapy can achieve satisfactory outcome.