Dedifferentiated central chondrosarcoma
Dedifferentiated central chondrosarcoma
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DOI：
10.1002/cncr.21936
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发表时间：
2006-06-15
期刊：
影响因子：
6.2
通讯作者：
Bertoni, Franco
中科院分区：
文献类型：
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作者：
Staals, Eric L.;Bacchini, Patrizia;Bertoni, Franco
背景。传统上，中心性软骨肉瘤去分化患者的预后很差。由于关于这种罕见病变的报道不多，因此预后因素仍存在许多不确定性。方法。在这项回顾性研究中，对来自 Rizzoli 研究所的 123 名患者的临床、放射学和组织学特征以及治疗进行了回顾，试图确定哪些因素可能与去分化中央软骨肉瘤患者的预后相关。结果。在本研究纳入的 123 名患者中，109 名患者在 Rizzoli 研究所接受治疗，14 名患者接受会诊。其中男性66例，女性57例，平均年龄59岁。股骨（62 名患者）、骨盆（28 名患者）和肱骨（20 名患者）是最常见的部位。放射学检查显示，87% 的患者存在软组织肿块，53% 的患者存在双态模式。组织学上，63% 的患者软骨成分被认为是 1 级，37% 的患者软骨成分被认为是 2 级。在大多数患者中，去分化成分表现出骨肉瘤（92名患者）的特征，其次是纤维肉瘤（19名患者）和恶性纤维组织细胞瘤（9名患者）。对于 101 名患者来说，手术是最终治疗的一部分。 25 名患者接受了手术与化疗相结合的治疗。 2年和5年生存率分别为34%和24%。中位生存期为 13 个月（95% 置信区间，9-17 个月）。结论。诊断时的转移性疾病、恶性纤维组织细胞瘤去分化以及高比例的去分化成分与较差的预后相关。没有统计证据表明辅助化疗有任何有益效果。
BACKGROUND. The prognosis for patients who develop dedifferentiation of central chondrosarcoma traditionally has been poor. Because not much has been reported about this rare lesion, many uncertainties remain about prognostic factors.METHODS. in this retrospective study, the clinical, radiographic, and histologic features and the treatments in 123 patients from the Rizzoli Institute were reviewed in an attempt to define which factors may be related to outcome in patients with dedifferentiated central chondrosarcoma.RESULTS. Among 123 patients who were included in this study, 109 patients were treated at the Rizzoli Institute, and 14 patients were seen in consultation. There were 66 males and 57 females, and their average age was 59 years. The femur (62 patients), pelvis (28 patients), and humerus (20 patients) were the most common locations. Radiographically, a soft tissue mass was present in 87% of patients, and a bimorphic pattern was appreciated in 53% of patients. Histologically, the cartilaginous component was considered Grade 1 in 63% of patients and Grade 2 in 37% of patients. In most patients, the dedifferentiated component showed the features of an osteosarcoma (92 patients), followed by fibrosarcoma (19 patients), and malignant fibrous histiocytoma (9 patients). For 101 patients, surgery was a component of their definitive management. In 25 patients, surgery was combined with chemotherapy. The 2-year and 5-year survival rates were 34% and 24%, respectively. The median survival was 13 months (95% confidence interval, 9-17 months).CONCLUSIONS. Metastatic disease at diagnosis, malignant fibrous histiocytoma dedifferentiation, and a high percentage of dedifferentiated component were related to poorer outcomes. There was no statistical evidence of any beneficial effect from adjuvant chemotherapy.