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. 2019 Apr 8;34(13):e107.
doi: 10.3346/jkms.2019.34.e107.

Chordomas: Histopathological Study in View of Anatomical Location

Yoon Jin Cha  1 , Yeon Lim Suh  2
Affiliations

Chordomas: Histopathological Study in View of Anatomical Location

Yoon Jin Cha et al. J Korean Med Sci. .

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.

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

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Conflict of interest statement

The authors have no potential conflicts of interest to disclose.

Figures

Fig. 1
Fig. 1. Characteristic histologic features of chordomas. (A) Chondroid matrix is frequently found in clival/intradural chordomas. (B) Fibrous septae with lobulating pattern is often observed in spinal/sacral chordomas. (C) BNCT-like area in clival chordoma. The cells resemble mature adipocytes and have eccentrically located small nuclei. Hyaline globules, one of the features of BNCT, are occasionally observed in BNCT-like areas of chordomas (inlet).
BNCT = benign notochordal cell tumor.
Fig. 2
Fig. 2. Overall survival and disease-free survival based on tumor location. Overall survival (A) tends to be lower in patients with clival/intradural chordomas than in those with spinal/sacral chordomas, although the difference is not statistically significant. However, disease-free survival (B) of patients with clival/intradural chordomas is significantly lower than that of those with spinal/sacral chordomas.
See this image and copyright information in PMC

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