gms | German Medical Science

Objective: Chordomas of the skull base are rare, locally invasive and have a poor prognosis. Aim of this retrospective multicentric study was to evaluate patterns of care, clinical course and prognostic factors of patients initially treated with open tumor resection. The prognostic influence of transmembrane cell adhesion proteines (N-cadherin and E-cadherin) was additionally analyzed.

Method: 47 patients (21 women, mean age 48.0 years; 26 men, mean age 48.9 years) treated in five centers were included. Histology was centrally evaluated, as well as N- and E-cadherin-expression by immuno-histochemistry. Prognostic factors were obtained from multivariate regression models. For survival analysis the Kaplan-Meier method was used.

Results: The median follow-up period was 5.2 years. Gross total resection, subtotal resection, and extended biopsy were done in 14.9%, 80.9%, and 4.2%, respectively. Side effects of surgery were not associated with extent of resection. Surgery was combined with early and delayed radiotherapy in 48.9% and 14.9% of the patients and the time point of its initiation was not associated with surgical radicality. Radiotherapeutic treatment included conventional fractionated radiotherapy (10.0%), proton beam irradiation (73.3%) and radiosurgery (16.7%). The median probability to achieve additional radiotherapy after surgery was 63.8%. Median progression free survival (overall survival) was 4.3 years and 12.4 years, respectively. Multivariate analysis indicated male gender as a risk factor for tumor progression (p=0.04) and death (p=0.02), although males underwent more often radical resection. E-cadherin and N-cadherin expression did not gain prognostic influence. Neither of the other patient- tumor-, and treatment-related prognostic factors (age, duration of symptoms, Karnofsky score, surgical radicality, adjuvant radiotherapy) gained statistically significant prognostic relevance.

Conclusions: Male patients bear a higher risk of recurrency and death. E-cadherin and N-cadherin expression did not gain prognostic significance. This finding might be added to selection criteria for aggressive treatment and/or closer follow-up examination.