Comparative analysis of histopathological parameters, genome-wide copy number alterations, and variants in genes involved in cell cycle regulation in chordomas of the skull base and sacrum.

Chordomas are rare tumors of the axial skeleton that are refractory to conventional therapy. Few studies have compared the morphological and molecular characteristics of chordomas according to the skull base and sacral locations. Histopathological data and changes revealed by array comparative genomic hybridization (CGH) and next-generation sequencing (NGS) of cell cycle regulation genes were analyzed for 28 skull base (SBCs) and 15 sacral (SC) chordomas. All cases were conventional chordomas. SBCs were significantly more frequent in patients aged <40 years and SCs predominated in patients aged >60 years. Mitotic indices ≥2 mitoses/10 high-power fields were correlated with high degrees of nuclear atypia and Ki67 labeling indices ≥6%. We identified 321 genomic positions, and copy number variation losses were more frequent than gain. Moreover, we report a panel of 85 genetic variants of cell cycle genes and the presence of molecular clusters for chordoma as well in CGH as in NGS. These new data strengthen the view that the chordoma should not be considered as a single molecular entity.

Extended endoscopic transsphenoidal approach for suprasellar craniopharyngiomas.

BACKGROUND: Craniopharyngiomas are benign sellar lesions. Surgical excision of craniopharyngiomas is difficult because of the surrounding important neurovascular structures. The choice of surgery depends on the histological type, location, hormonal status, and size of the craniopharyngioma, surrounding neurovascular structures, and invasion of the brain parenchyma. METHODS: We describe the resection of an adamantinomatous craniopharyngioma using an extended endoscopic endonasal approach and discuss the relevant surgical anatomy, indications, limitations, and possible complications. CONCLUSIONS: The extended endoscopic endonasal approach allows successful removal of the craniopharyngioma and poses little risk to surrounding neurovascular structures.