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Open Access Highly Accessed Research article

Chordoma: clinical characteristics, management and prognosis of a case series of 25 patients

Virginia Ferraresi1*, Carmen Nuzzo1, Carmine Zoccali2, Ferdinando Marandino3, Antonello Vidiri4, Nicola Salducca2, Massimo Zeuli1, Diana Giannarelli1, Francesco Cognetti1 and Roberto Biagini2

Author Affiliations

1 Department of Medical Oncology A, Regina Elena National Cancer Institute, Rome, Italy

2 Department of Oncological Orthopedic Surgery, Regina Elena National Cancer Institute, Rome, Italy

3 Department of Pathology, Regina Elena National Cancer Institute, Rome, Italy

4 Department of Radiology, Regina Elena National Cancer Institute, Rome, Italy

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BMC Cancer 2010, 10:22  doi:10.1186/1471-2407-10-22

Published: 28 January 2010

Abstract

Background

Adequate surgery still remains the only curative treatment of chordoma. Interesting clinical data on advanced disease with molecularly targeted therapies were reported.

Methods

We described the clinical outcome of a series of chordoma patients followed at Regina Elena National Cancer Centre of Rome from 2004 to 2008.

Results

Twenty-five consecutive patients with sacral (11 patients), spine (13 patients), and skull base (1 patient) chordoma went to our observation. Six patients (24%) had primary disease, 14(56%) a recurrent disease, and 5(20%) a metastatic spreading. Surgery was the primary option for treatment in 22 out of 25 patients. Surgical margins were wide in 5 (23%) and intralesional in 17(77%) patients; 3 out of 4 in-house treated patients obtained wide margins. After first surgery, radiotherapy (protons or high-energy photons) were delivered to 3 patients. One out of the 5 patients with wide margins is still without evidence of disease at 20 months from surgery; 2 patients died without evidence of disease after 3 and 36 months from surgery. Sixteen out of 17 (94%) patients with intralesional margins underwent local progression at a median time of 18 months with a 2-year local progression-free survival of 47%. The 5-year metastasis-free survival rate was 78.3%. Seventeen patients with locally advanced and/or metastatic disease expressing platelet-derived growth factor receptor (PDGFR) β were treated with imatinib mesylate. A RECIST stabilization of the disease was the best response observed in all treated cases. Pain relief with reduction in analgesics use was obtained in 6 out of 11 (54%) symptomatic patients. The 5- and 10-year survival rates of the entire series of patients were 76.7 and 59.7%, respectively.

Conclusions

Despite progress of surgical techniques and the results obtained with targeted therapy, more effort is needed for better disease control. Specific experience of the multidisciplinar therapeutic team is, however, essential to succeed in improving patients' outcome.