Department of Medical Oncology A, Regina Elena National Cancer Institute, Rome, Italy.
BMC Cancer. 2010 Jan 28;10:22. doi: 10.1186/1471-2407-10-22.
Adequate surgery still remains the only curative treatment of chordoma. Interesting clinical data on advanced disease with molecularly targeted therapies were reported.
We described the clinical outcome of a series of chordoma patients followed at Regina Elena National Cancer Centre of Rome from 2004 to 2008.
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.
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.
充分的手术仍然是脊索瘤唯一的治愈性治疗方法。有趣的是，有报道称，晚期疾病采用分子靶向治疗具有良好的临床数据。
我们描述了 2004 年至 2008 年在罗马 Regina Elena 国家癌症中心接受治疗的一系列脊索瘤患者的临床结果。
25 例连续的骶骨（11 例）、脊柱（13 例）和颅底（1 例）脊索瘤患者进入我们的观察。6 例（24%）为原发性疾病，14 例（56%）为复发性疾病，5 例（20%）为转移性疾病。25 例患者中有 22 例选择手术作为主要治疗方法。22 例患者中，5 例（23%）达到广泛切除，17 例（77%）为次广泛切除；4 例在院治疗的患者中有 3 例达到广泛切除。初次手术后，3 例患者接受质子或高能光子放疗。4 例获得广泛切除的患者中，1 例在手术后 20 个月仍无疾病证据；2 例在手术后 3 个月和 36 个月无疾病证据后死亡。17 例次广泛切除的患者中有 16 例（94%）在中位时间 18 个月时发生局部进展，2 年局部无进展生存率为 47%。5 年无转移生存率为 78.3%。17 例局部晚期和/或有转移且表达血小板衍生生长因子受体（PDGFR）β的患者接受甲磺酸伊马替尼治疗。在所有治疗病例中，观察到的最佳反应是疾病的 RECIST 稳定。11 例有症状的患者中，有 6 例（54%）疼痛减轻，止痛药用量减少。整个患者系列的 5 年和 10 年生存率分别为 76.7%和 59.7%。
尽管手术技术取得了进展，靶向治疗也取得了成果，但仍需要付出更多的努力来更好地控制疾病。然而，多学科治疗团队的具体经验对于提高患者的预后至关重要。
