Pamir M N, Kiliç T, Türe U, Ozek M M
Department of Neurosurgery Istanbul, Marmara University Institute of Neurosciences, Istanbul, Turkey.
Acta Neurochir (Wien). 2004 Apr;146(4):343-54; discusion 354. doi: 10.1007/s00701-004-0218-3. Epub 2004 Feb 16.
To analyze a series of patients with pathologically confirmed skull-base chordoma, and to develop an algorithm for the management of this challenging disease based on the data, our experience, and the current literature.
Between the years 1986 and 2001, 26 chordoma patients received multimodality treatment with various combinations of conventional surgery, skull-base surgical techniques, and gamma-knife surgery at the Marmara University Faculty of Medicine. A total of 57 procedures (43 tumor excision surgeries, 7 gamma-knife procedures, and 7 other operations to treat complications) were performed. The mean follow-up period was 4 years (48.5 months). Karnofsky scoring was used to follow the patients' clinical conditions, and magnetic resonance image analysis was used to measure tumor volume over time.
Seven patients died during follow-up. Two of the deaths were due to surgical complications, four resulted from clinical deterioration related to tumor recurrence, and one was unrelated to neoplasia. The rate of tumor recurrence after the first surgical treatment was 58%. Residual tumor volume was lower in the cases in whom skull-base approaches were used as first-line management. The 19 survivors showed little change in clinical status from initial diagnosis to the most recent follow-up check. The mean follow-up time after gamma-knife treatment was 23.3 months. During this period, mean tumor volume increased 28% above the mean volume at the time of gamma-knife surgery. The mean Karnofsky score decreased by 6% during the same time frame.
The most effective first-line treatment for chordoma patients is surgery. The findings for residual tumor volume indicated that skull-base approaches are the best surgical option, and the complication rates for these techniques are acceptable. However, it is rare that surgery ever biologically eradicates this disease, and the data showed that these chordomas almost always progress if the tumor volume at the time of diagnosis exceeds 20 cm(3). Based on our experience and the biological character of the disease, we now advocate radiosurgical treatment (gamma-knife in our case) immediately after the first-line skull-base surgery when the tumor residual volume is <30 cm(3).
分析一系列经病理确诊的颅底脊索瘤患者，并根据所获数据、我们的经验以及当前文献，制定针对这种具有挑战性疾病的治疗方案。
1986年至2001年间，26例脊索瘤患者在马尔马拉大学医学院接受了多模式治疗，包括传统手术、颅底手术技术和伽玛刀手术的各种组合。共进行了57次手术（43次肿瘤切除手术、7次伽玛刀手术以及7次治疗并发症的其他手术）。平均随访期为4年（48.5个月）。采用卡诺夫斯基评分来跟踪患者的临床状况，并通过磁共振图像分析来测量肿瘤体积随时间的变化。
7例患者在随访期间死亡。其中2例死于手术并发症，4例因肿瘤复发导致临床状况恶化，1例与肿瘤形成无关。首次手术治疗后的肿瘤复发率为58%。在将颅底入路作为一线治疗方法的病例中，残余肿瘤体积较小。19例存活患者从初始诊断到最近一次随访检查，临床状况变化不大。伽玛刀治疗后的平均随访时间为23.3个月。在此期间，平均肿瘤体积比伽玛刀手术时的平均体积增加了28%。同一时间段内，卡诺夫斯基评分平均下降了6%。
脊索瘤患者最有效的一线治疗方法是手术。残余肿瘤体积的研究结果表明，颅底入路是最佳的手术选择，且这些技术的并发症发生率是可以接受的。然而，手术几乎不可能从生物学角度根除这种疾病，数据显示，如果诊断时肿瘤体积超过20 cm³，这些脊索瘤几乎总会进展。基于我们的经验和该疾病的生物学特性，我们现在主张在一线颅底手术后，当肿瘤残余体积<30 cm³时，立即进行放射外科治疗（我们采用的是伽玛刀）。
