Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
Fröhlich, E.
Chordomas: A retrospective clinical and immunohistochemical study.
[ Dissertation ] Graz Medical University; 2008. pp.107.
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- Autor*innen der Med Uni Graz:
- Betreuer*innen:
- Beham Alfred
- Leithner Andreas
- Altmetrics:
- Abstract:
- Objective: Chordomas are infrequent malignant tumours that develop from notochordal remnants. Main localisation is the axial skeleton. Purpose of the clinical study part: investigation of prognostic factors (localisation, volume, histological features, metastases, margin status, radiotherapy). Purpose of the immunohstochemical study part: Investigation of the expression of survivin, DR 4 and 5, ezrin and c-Kit as potential molecular targets in future therapy strategies for chordomas. Patients/Methods: 39 patients (mean age 51 years) underwent surgery. 34 patients were observed for 53 months averaged; Tumour localisation: scull 19 (48,7%), sacrum 11 (28,2%), cervical spine 6, lumbar and thoracic spine 3. Mean tumour volume: 189,7cm3. 3 patients developed metastases. Histological features pictured: 34 classic, 4 chondroid, 1 dedifferentiated chordoma. Within the clinical study we elicited OS, RFS, tumour volume, resection status and radiotherapy. Within the immunohistochemical analyses 36 samples were investigated. Evaluation:description of percentage of stained cells; cytoplasmic, nuclear or diffuse expression, intensity of immunoreactivity. Results: Resection status:25 intralesional, 2 marginal, 2 wide resections. 5 patients: biopsy only. Of these 5 patients 2 underwent RTX. Residual tumours:13 patients. Recurrences:6 patients, all after intralesional resection. OS: 74,7% after 5,3 years and 32,5% after 9,4 years. RFS: 77% after 5 years. 12 patients died. Out of these 4 survived 78 months (m) after intralesional resection, followed by radiotherapy. In other 5 patients surgery alone was done (45m). 2 patients rejected any therapy (10m). 1 patient underwent gamma-knife-therapy (7m). Within the immunohistochemical study all investigated factors presented a staining reaction, except c-kit. Survival analyses and the evaluation of the correlation between the expression of DR4/5 and survivin did not result in significant p-values. Conclusion: Due to the localisation chordomas often present a special challenge to an interdisciplinary team. Because of rarity of chordomas and heterogeneity of therapy no significant p-values were found. Despite this fact we draw on already published analyses that marginal or wide resection status combined with radiotherapy leads to the longest RFS and OS. To the authors knowledge this is the first study examining the factors survivin, ezrin, DR4 and DR5 in chordoma samples. All aforementioned factors presented with a positive staining, except c-kit. Due to the small number of examined samples no significant results were achieved.