Liu Chenglei, Xi Yan, Li Mei, Jiao Qiong, Zhang Huizhen, Yang Qingcheng, Yao Weiwu
Department of Radiology, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China.
Department of Pathology, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China.
PLoS One. 2017 Mar 16;12(3):e0173665. doi: 10.1371/journal.pone.0173665. eCollection 2017.
Dedifferentiated chondrosarcoma is a rare, highly malignant tumor with a poor survival. There are many confusing issues concerning the imaging feature that can facilitate early diagnosis and the factors that might be related to outcomes.
Twenty-three patients with dedifferentiated chondrosarcoma confirmed by pathology were retrospectively reviewed from 2008 to 2015. The patients' clinical information, images from radiographs (n = 17), CT (n = 19), and MRI (n = 17), histological features, treatment and prognosis were analyzed.
There were 12 males and 11 females, and the mean age was 50.39 years old. Fourteen cases affected the axial bone (pelvis, spine), and 9 cases involved the appendicular bone. Seven (41.17%), 9 (47.36), and 12 (66.66%) lesions showed a biphasic nature on radiograph, CT and MRI, respectively. Of the lesions, 17.39% (4/23) were accompanied by pathological fractures. Histologically, the cartilage component was considered histological Grade1 in 12 patients and Grade 2 in 11 patients. The dedifferentiated component showed features of osteosarcoma in 8 cases, malignant fibrous histiocytoma in3 cases, myofibroblastic sarcoma in 1 case and spindle cell sarcoma in 11cases. Twenty-two cases were treated with surgical resection, and 17 cases achieved adequate (wide or radical) surgical margin. In 8 cases, surgery was combined with adjuvant chemotherapy. The overall median survival time was nine months; 17.4% of patients survived to five years.
Axial bone location, lung metastasis at diagnosis, inadequate surgical margin, incorrect diagnosis before surgery and pathological fractures was related to poorer outcome. Pre- or postoperative chemotherapy had no definitively effect on improved survival.
去分化软骨肉瘤是一种罕见的高恶性肿瘤,生存率低。关于有助于早期诊断的影像学特征以及可能与预后相关的因素,存在许多令人困惑的问题。
回顾性分析2008年至2015年23例经病理确诊的去分化软骨肉瘤患者。分析患者的临床信息、X线片(n = 17)、CT(n = 19)和MRI(n = 17)图像、组织学特征、治疗及预后情况。
男性12例,女性11例,平均年龄50.39岁。14例累及中轴骨(骨盆、脊柱),9例累及四肢骨。分别有7例(41.17%)、9例(47.36%)和12例(66.66%)病变在X线片、CT和MRI上表现为双相性。其中17.39%(4/23)的病变伴有病理性骨折。组织学上,12例患者的软骨成分组织学分级为1级,11例为2级。去分化成分中,8例表现为骨肉瘤特征,3例为恶性纤维组织细胞瘤,1例为肌纤维母细胞肉瘤,11例为梭形细胞肉瘤。22例接受手术切除,17例达到足够(广泛或根治性)手术切缘。8例患者手术联合辅助化疗。总体中位生存时间为9个月;17.4%的患者存活至5年。
中轴骨部位、诊断时肺转移、手术切缘不足、术前误诊及病理性骨折与较差的预后相关。术前或术后化疗对提高生存率无明确作用。