去分化软骨肉瘤40例临床病理特征与预后分析

Clinicopathological features and prognosis of dedifferentiated chondrosarcoma: an analysis of 40 cases

目的探讨去分化软骨肉瘤(dedifferentiated chondrosarcoma,DDCS)的临床、影像、病理学特征及诊断、治疗和预后等。方法收集2005年1月~2017年8月上海交通大学附属第六人民医院经病理确诊的40例DDCS,包括临床、影像、病理和随访资料,采用HE、免疫组化对其进行分析并复习相关文献。结果 40例DDCS中男性21例,女性19例,平均年龄51岁。发病部位分别为髋部、股骨、肱骨、胸骨、胫骨、肩部、手指、胸椎。临床主要表现为疼痛、肿胀、活动受限等。典型的影像学表现为双相征,即呈现出软骨肉瘤的点状、环状钙化和具有侵袭性特征的软组织肿块两种表现。镜下可见界限清楚的高分化软骨肉瘤成分和高度恶性成分(包括骨肉瘤、恶性纤维组织细胞瘤、纤维肉瘤、不能明确分类的梭形细胞肉瘤),或去分化为低级别肿瘤(包括骨巨细胞瘤或低级别骨肉瘤),两种成分比例不定。40例中有34例术前行穿刺活检,仅9例正确诊断为DDCS,穿刺诊断正确率为26.5%。在初诊时已发生转移患者的平均生存时间明显短于未发生转移患者。结论 DDCS的诊断需临床、影像和病理三结合。其组织学形态表现复杂多样、术前穿刺活检存在局限性,其诊断比较困难。初次诊断时伴有转移的患者预后更差。

Purpose To explore the clinical, imaging, pathologic features and diagnosis, treatment and prognosis of dedifferentiated chondrosarcoma （DDCS）. Methods Forty ca- ses of DDCS confirmed by pathologists were collected from Shanghai Jiaotong University Affiliated Sixth People＇ s Hospital from January 2005 to August 2017, including clinical, imaging, pathologic and follow-up data. HE, immunohistochemistry and statistical methods were used with review of the related litera- ture. Results There were 21 males and 19 females in 40 cases of DDCS, with an average age of 51 years. The tumors were lo- cated in hip, proximal femur, humerus, sternum, tibia, shoul- der, finger, and thoracic cavity. The main clinical manifesta- tions were local pain, swelling, limited mobility and so on. Typ- ical radiographic manifestations are ＂bimorphic features＂, which showed two manifestations of dot or circular like calcification of chondrosarcoma, and invasive soft tissue masses. Histologically, clearly defined well-differentiated chondrosarcoma componentsjuxtaposed highly malignant dedifferentiation components, which can display as follows ： osteosarcoma, fibrosarcoma, malignant fibrous histiocytoma, and spindle cell sarcoma that could not be clearly classified. The dedifferentiated components might also be low-grade tumors such as giant cell tumor of bone or low grade osteosarcoma. The proportions of these two ingredients were un- certain. Preoperative biopsy was performed in 34 cases, of whom only 9 （26. 5% ） were correctly diagnosed as DDCS. The aver- age survival time of patients with metastasis at first visit was sig- nificantly shorter than the patient without metastasis. Conclu- sion Diagnosis of DDCS should be combined with clinical fea- tures, imaging and histological morphology. As their histological morphology complex, and preoperative puncture biopsy limita- tions, its diagnosis is very difficult. Patients with metastases at the time of initial diagnosis have a worse prognosis.