Artikel
Chordoma of the skull base – single-centre experience of a rare disease
Chordome der Schädelbasis: Eine monozentrische Studie zu einem seltenen Krankheitsbild
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Autoren
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Hanah Gull
- Universitätsklinikum Essen, Klinik für Neurochirurgie und Wirbelsäulenchirurgie, Essen, Deutschland
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Marvin Darkwah Oppong
- Universitätsklinikum Essen, Klinik für Neurochirurgie und Wirbelsäulenchirurgie, Essen, Deutschland
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Oliver Gembruch
- Universitätsklinikum Essen, Klinik für Neurochirurgie und Wirbelsäulenchirurgie, Essen, Deutschland
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Alejandro Santos Saint Romain
- Universitätsklinikum Essen, Klinik für Neurochirurgie und Wirbelsäulenchirurgie, Essen, Deutschland
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Mehdi Chihi
- Universitätsklinikum Essen, Klinik für Neurochirurgie und Wirbelsäulenchirurgie, Essen, Deutschland
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Ramazan Jabbarli
- Universitätsklinikum Essen, Klinik für Neurochirurgie und Wirbelsäulenchirurgie, Essen, Deutschland
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Neriman Oezkan
- Universitätsklinikum Essen, Klinik für Neurochirurgie und Wirbelsäulenchirurgie, Essen, Deutschland
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Karsten H. Wrede
- Universitätsklinikum Essen, Klinik für Neurochirurgie und Wirbelsäulenchirurgie, Essen, Deutschland
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Ulrich Sure
- Universitätsklinikum Essen, Klinik für Neurochirurgie und Wirbelsäulenchirurgie, Essen, Deutschland
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Yahya Ahmadipour
- Universitätsklinikum Essen, Klinik für Neurochirurgie und Wirbelsäulenchirurgie, Essen, Deutschland
| Veröffentlicht: | 25. Mai 2022 |
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Gliederung
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Objective: Chordoma is a rare disease that belongs to the group of malignant bone and soft tissue tumors of sarcomas. It is a locally aggressive, osteodestructive, metastasizing tumor with preferred localization in the skull base and sacrum. Due to the anatomic localization and infiltration, complete resection is difficult. An optimal treatment concept has not been established so far, as chordomas are known to be chemo- and radioresistant if applied in regular doses. The aim of this study was to analyze the course of surgically treated chordoma patients.
Methods: Our department's medical records of 70 chordoma patients treated from January 1998 to December 2020 were analyzed retrospectively regarding demographic, clinical outcome, and molecular characteristics. Univariate and multivariate analyses were used. P-value ≤ 0,05 was considered statistically significant.
Results: Thirty-eight patients with histologically confirmed chordoma located in the clivus from 9 to 81 years with a mean of 53.17 (± 18.55 standard deviation) were included into the final analysis. The male to female ratio was 1.17: 1. Gross total resection was achieved in 4 cases. All patients underwent postoperative radiotherapy, consisting of protons or carbon-12 irradiation. 11 patients underwent additional chemotherapy and/ or immunotherapy. Recurrent local diseases after therapy was observed in 58% (n=22) of the patients, while 39% (n=15) developed osseus, pulmonary, intraspinal and intradural metastases. In histopathological examination, a higher Ki67/ MIB-1 proliferation index was not significantly correlated with an increased rate of tumor recurrence, metastasis, or a shorter patients' overall survival (Log Rank p= 0.328, p= 0.224, p= 0,577). In contrast, S-100 level negatively correlated with a higher incidence of metastasis (Log Rank p= 0.005).
Conclusion: Current guideline-based therapy seems ineffective regarding the management and outcome of patients suffering from chordoma. S-100 index reflects malignancy in clival chordomas. Further, multicenter studies are urgently needed to unravel this tumor entity to develop new adjuvant therapy modalities.
