Locally advanced unresectable disease, systemic treatment associated with longer OS in chondrosarcoma

Survival in patients with unresectable conventional central chondrosarcoma is poor, yet systemic treatment and radiotherapy improved OS in select patients, according to results of a retrospective study.

“In the last decades, there has been no significant improvement in the survival of patients with chondrosarcoma,” Annemiek M. van Maldegem, MD, of the department of clinical oncology at Leiden University Medical Center in the Netherlands, and colleagues wrote. “Currently, it is not believed that the available systemic therapy options improve outcome, although randomized studies and large series have not been published. Therefore, we conducted this study to gain insight into the outcome and the effectiveness of systemic treatment for patients with advanced, unresectable conventional central chondrosarcoma.”

Van Maldegem and colleagues evaluated data from 171 patients who were diagnosed with unresectable chondrosarcoma between 1980 and 2011 at one of two European bone sarcoma centers. A majority of patients were male (n=107), and the mean age of the population at diagnosis was 53 years (range, 17-90).

Forty-two percent of patients had unresectable metastatic disease in the lungs only, 26% of patients had local unresectable disease, 23% had local and unresectable lung disease, and 9% had multiorgan involvement.

Median OS was 11 months (range, 1-106).

Forty-eight percent of patients achieved 1-year OS, 24% achieved 2-year OS and 12% achieved 3-year OS. The OS rate was 6% at 4 years and 2% at 5 years.

Patients with only locally advanced unresectable disease demonstrated significantly improved OS compared with patients with metastases (P=.0014). Twenty-six percent of patients with local unresectable disease achieved 3-year OS, compared with 7% of patients with lung involvement, and 8% of patients with lung and local disease involvement.

Mean OS among patients with multiorgan involvement was 7 months, and none of these patients survived 2 years.

Systemic therapy with doxorubicin-based chemotherapy, imatinib (Gleevec, Novartis) or sirolimus (Rapamune, Pfizer) was associated with significantly improved 3-year survival compared with no treatment (26% vs. 8%; P=.0487).

Radiation therapy was associated with improved survival among patients who only had locally advanced disease (P=.0032).

Among patients with metastatic disease, those who received systemic or radiation therapy demonstrated improved OS (P=.0082).

“Our study adds significantly to the limited data available on OS for both locally and metastatic, unresectable conventional central chondrosarcoma,” the researchers concluded. “Our data demonstrate that chemotherapy in patients with unresectable chondrosarcoma may increase survival, but further studies are warranted. Radiotherapy provides a survival advantage and is common practice for locally advanced conventional central chondrosarcoma in both reference centers.”

These data may help researchers design phase 2 clinical trials to assess the efficacy of targeted therapies in this setting, Antoine Italiano, MD, PhD, of the Institut Bergonié in Bordeaux, France, wrote in an invited commentary.

“Altogether, the data presented here … suggest that, if a randomized design is chosen, then investigators should consider an anthracycline-based regimen instead of placebo for the standard arm,” Italiano wrote. “This would help to prospectively confirm the activity of anthracyclines in patients with chondrosarcoma and would facilities patients’ consent compared with a trial that offered placebo as a control arm.”

For more information:

Disclosure: The researchers report no relevant financial disclosures.