title: Chordoma: clinical characteristics, management and prognosis of a case series of 25 patients.
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) beta 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 multi-disciplinary therapeutic team is, however, essential to succeed in improving patients' outcome.
authors.affiliation: Department of Medical Oncology A, Regina Elena National Cancer Institute, Rome, Italy. ferraresi@ifo.it
authors.forename: Virginia
authors.initials: V
authors.lastname: Ferraresi
authors.forename: Carmen
authors.initials: C
authors.lastname: Nuzzo
authors.forename: Carmine
authors.initials: C
authors.lastname: Zoccali
authors.forename: Ferdinando
authors.initials: F
authors.lastname: Marandino
authors.forename: Antonello
authors.initials: A
authors.lastname: Vidiri
authors.forename: Nicola
authors.initials: N
authors.lastname: Salducca
authors.forename: Massimo
authors.initials: M
authors.lastname: Zeuli
authors.forename: Diana
authors.initials: D
authors.lastname: Giannarelli
authors.forename: Francesco
authors.initials: F
authors.lastname: Cognetti
authors.forename: Roberto
authors.initials: R
authors.lastname: Biagini
mesh_terms: D000328:Adult; D000368:Aged; D001859:Bone Neoplasms / Q000175:diagnosis / Q000473:pathology; D002817:Chordoma / Q000175:diagnosis* / Q000473:pathology*; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009362:Neoplasm Metastasis; D049268:Positron-Emission Tomography; D011379:Prognosis; D017479:Receptors, Platelet-Derived Growth Factor / Q000378:metabolism; D013131:Spine / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D017479:Receptors, Platelet-Derived Growth Factor