Peculiar pathological, radiological and clinical features of skull-base de-differentiated chordomas. Results from a referral centre case-series and literature review.

Skull-base de-differentiated chordomas are extremely rare and aggressive neoplasms with characteristic magnetic resonance imaging, surgical and histological features. Therefore, an early and accurate histological diagnosis is of paramount relevance. Molecular analysis appears promising to define mechanisms involved in tumour de-differentiation.