Chordomas: Histopathological Study in View of Anatomical Location

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MeSH: Adult ; Age Factors ; Aged ; Aged, 80 and over ; Chordoma/mortality ; Chordoma/pathology* ; Cranial Fossa, Posterior/pathology ; Disease-Free Survival ; Female ; Humans ; Ki-67 Antigen/metabolism ; Male ; Middle Aged ; Proportional Hazards Models ; Risk Factors ; Sacrum/pathology ; Skull Base Neoplasms/mortality ; Skull Base Neoplasms/pathology* ; Survival Rate ; Young Adult

Keywords: Bone Neoplasms ; Brain Neoplasms ; Chordoma ; Notochord ; Skull Base Neoplasms ; Soft Tissue Neoplasms

Abstract: BACKGROUND: Chordomas are aggressive bone tumors that have a predilection for the axial skeleton including the skull base and spinal/sacral bones. However, the histopathological and clinical differences between skull base chordoma (SBC) and sacral/spinal chordoma (SC) are unclear as previous studies have been focused on patient prognosis and treatment outcome. This study aimed to evaluate the clinicopathologic features and prognosis of chordoma according to its location.

METHODS: Patients with chordomas were enrolled, and the histopathologic features were compared according to the tumor location.

RESULTS: A total of 52 patients were enrolled. SBCs had more abundant chondroid matrix and diffuse growth pattern, while SCs had non-chondroid, myxoid matrix and a lobulating pattern, typical of chordoma. Old age and residual tumors were risk factors for shorter overall survival in SBCs. The chondroid matrix was an independent risk factor for shorter disease-free survival in the overall population.

CONCLUSION: Chordomas have different histopathologic features depending on the anatomical location.