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Aggressive Chordomas: Clinical Outcome of 13 Patients

机译:侵略性的Chordomas:13名患者的临床结果

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The authors reviewed the files of all patients with chordomas who were admitted and treated at their institutions from 1975 to 2012. Patients were categorized by early local recurrence and metastasis. Aggressive clinical behavior was defined as local recurrence and metastasis within 24 months of diagnosis and adequate treatment (wide en bloc resection with microscopically negative tumor margins). According to these criteria, 13 patients (14.3%) had aggressive chordomas, including 7 men and 6 women, with mean age of 54 years (range, 37-65 years) at diagnosis and treatment. All patients had preoperative tumor biopsy, followed by resection with partial (7 patients) or total sacrectomy (6 patients). In all cases, biopsy and histologic analysis of resected tumor specimens showed conventional chordomas. Resection margins were wide (grossly negative) in 6 patients and wide contaminated in 7 patients. Mean maximum tumor diameter was 11.8 cm (range, 5-21 cm). Mean follow-up was 43 months (range, 8-131 months). Rates of local recurrence, metastasis, and death were evaluated. At the last follow-up, all patients had local recurrence at a mean of 13 months (range, 5-22 months). Histologic examination of recurrent tumors showed a dedifferentiated chordoma with a fibrosarcoma component in 2 patients and no histologic change in the remaining patients. In addition, 8 patients had metastases at a mean of 13 months (range, 4-24 months) and died of their disease. All histologic findings of metastatic lesions were similar to those of primary tumors. Early diagnosis of aggressive tumors requires close follow-up of patients with chordomas. Metastasis is common, with resultant poor survival.
机译:作者审查了1975年至2012年在其机构入院和治疗的Chordomas患者的档案。患者被早期局部复发和转移分类。侵略性的临床行为被定义为诊断和足够治疗的24个月内的局部复发和转移(具有显微镜阴性肿瘤边缘的宽Zhoc切除)。根据这些标准,13名患者(14.3%)具有侵略性的Chordomas,其中包括7名男性和6名女性,平均年龄为54岁(范围37-65岁),在诊断和治疗中。所有患者均有术前肿瘤活检,随后用部分(7名患者)或总死亡(6名患者)切除。在所有情况下,切除的肿瘤标本的活检和组织学分析显示出常规的Chordomas。在7名患者中,切除的边距宽(严重阴性)宽(严重阴性)和宽污染。平均最大肿瘤直径为11.8cm(范围,5-21厘米)。平均随访时间为43个月(范围,8-131个月)。评估局部复发,转移和死亡率。在最后一次随访中,所有患者均以13个月(范围5-22个月)的局部复发。复发性肿瘤的组织学检查表明,2名患者中具有纤维肉瘤成分的消化不异性脊髓瘤,其余患者没有组织学变化。此外,8名患者的平均转移为13个月(范围,4-24个月)并死于疾病。转移性病变的所有组织学结果与原发性肿瘤的组织学结果相似。侵袭性肿瘤的早期诊断需要密切关注Chordomas的患者。转移是常见的,结果存活差。

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