Clinical outcome for patients with dedifferentiated chondrosarcoma: a report of 9 cases at a single institute.
Clinical outcome for patients with dedifferentiated chondrosarcoma: a report of 9 cases at a single institute.
复制标题
DOI：
10.1186/1749-799x-7-38
复制
发表时间：
2012-12-10
影响因子：
2.6
通讯作者：
Iwamoto Y
中科院分区：
文献类型：
--
作者：
Yokota K;Sakamoto A;Matsumoto Y;Matsuda S;Harimaya K;Oda Y;Iwamoto Y
去分化软骨肉瘤由两种可区分的成分组成：低级软骨肉瘤成分和高级去分化成分。本文报告9例去分化软骨肉瘤，男4例，女5例。平均年龄为58.6岁（范围，37-86岁）。肿瘤位于长骨7例（股骨5例，肱骨1例，胫骨1例），骨盆2例。从出现症状到治疗的平均时间为9.4（范围，1-40）个月。在X线平片上，所有9例（100%）均可见基质矿化。9例中5例（56%）骨破坏，1例（11%）股骨病理性骨折。所有病例均观察到肺转移（最初5例，治疗过程中4例）。8例进行了手术，其中2例发生局部复发（复发时间，2个月和10个月）。4例接受化疗，但未导致明显改善。9例均死于肺转移，中位生存期10个月（3.4-18.8）。诊断时有原发转移是一个重要的不利预后因素。去分化软骨肉瘤的预后很差。由于缺乏令人信服的证据证明化疗的益处，完全手术切除是最初推荐的治疗方法。
Dedifferentiated chondrosarcomas consist of two distinguishable components: low-grade chondrosarcoma components and high-grade dedifferentiated components. Nine cases (4 males, 5 females) of dedifferentiated chondrosarcoma were treated in our institute. The average age was 58.6 (range, 37–86) years. The tumor location was the long bone in 7 cases (femur, n=5; humerus, n=1; tibia, n=1) and the pelvic bone in 2 cases. The average time from appearance of symptoms to treatment was 9.4 (range, 1–40) months. On plain radiographs, matrix mineralization was seen in all 9 cases (100%). Bone destruction was observed in 5 of 9 cases (56%), while pathological fracture was seen in one femur case (11%). Lung metastasis was observed in all cases (initially in 5 cases; during the treatment course in 4 cases). Surgery was performed in 8 cases, with local recurrence occurring in 2 of those cases (time to recurrence, 2 and 10 months). Chemotherapy was administered in 4 cases, but did not result in significant improvement. All 9 cases died of lung metastases, with a median survival time of 10 (range, 3.4-18.8) months. The presence of initial metastasis at diagnosis was a significant unfavorable prognostic factor. The prognosis of dedifferentiated chondrosarcoma is dismal. With the lack of convincing evidence of the benefit of chemotherapy, complete surgical excision is the initial recommended treatment.