A clinical analysis of 19 cases of chordomas with poor differentiation treated using diverse therapeutic modalities

There is an urgent need to enhance clinicians' ability to diagnose and treat chordomas with poor differentiation, particularly poorly differentiated chordomas (PDCs), through clinical case analysis.

A retrospective review was conducted on the clinical data of 19 patients with pathologically confirmed chordomas with poor differentiation, treated at Hebei Yizhou Cancer Hospital between July 2021 and May 2024. All patients underwent surgery. 9 received proton radiation, with 4 also having conventional chemotherapy. 3 patients had both conventional chemotherapy and targeted therapy after proton radiation, while another 3 received chemotherapy, targeted therapy, and immunotherapy. The progression of the disease was primarily monitored through follow-up telephone consultations. The study used polytomous logistic regression to assess the impact of age and gender on chordoma lesion sites, and ordinal logistic regression to evaluate the effects of age, gender, and lesion sites on chordoma differentiation. The COX Proportional Hazards Model analyzed the influence of these factors and surgical resection ratio on chordoma prognosis. Kaplan-Meier survival analysis assessed the impact of surgical resection ratio on prognosis.

Postoperative diagnoses revealed 12 cases of PDC, 4 of dedifferentiated chordoma (DDC), and 3 of low differentiated chordoma (LDC). Some patients experienced tumor reduction and symptom improvement. By August 2024, 8 patients had died, while 11 were still alive, with follow-up durations ranging from 12 to 65 months and an average of 29.45 months. Based on these limited 19 cases, COX regression analysis showed that sex, differentiation degrees, chordoma locations, treatment regimens, and surgical resection ratio were not independent prognostic factors (all P > 0.05). Younger age was linked to higher differentiation (P < 0.05), and chordomas on the clivus had the worst differentiation and highest malignancy (P < 0.001).

Based on the analysis of 19 chordoma cases, it appears that for chordomas with poor differentiation, the completion of surgical resection followed by adjuvant chemotherapy, radiotherapy, and targeted therapy may enhance patient prognosis to some extent. The efficacy of immunotherapy for chordomas with poor differentiation warrants further investigation. Additionally, potential prognostic factors influencing chordoma outcomes should be validated in studies involving larger populations.