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软骨肉瘤。

Chondrosarcoma.

机构信息

From the Department of Orthopaedic Oncology (Weinschenk, Lewis), and the Department of Pathology (Wang), The University of Texas MD Anderson Cancer Center, Houston, TX.

出版信息

J Am Acad Orthop Surg. 2021 Jul 1;29(13):553-562. doi: 10.5435/JAAOS-D-20-01188.

Abstract

Chondrosarcoma is the second most common primary bone tumor, with >90% of cases representing the primary conventional subtype. In addition to arising de novo, conventional chondrosarcoma can arise secondary to a benign underlying lesion, such as enchondroma or osteochondroma. Symptoms are often characterized by focal, dull, aching pain to the affected region. Grade is a well-recognized prognostic factor in these tumors. Grade I lesions/atypical cartilaginous tumors rarely metastasize, rarely recur, and have a 10-year survival rate of >80%. By contrast, grade III lesions are associated with a poor prognosis with the highest local recurrence rates, a lung metastasis rate of >50%, and a 10-year survival rate of <30%. The standard treatment of high-grade conventional chondrosarcoma is complete surgical resection with wide margin. However, low-grade lesions may be amenable to curettage plus or minus adjuvant local treatment. Conventional chondrosarcoma does not respond to chemotherapy or standard radiation doses. Adjuvant treatment can be beneficial for some subtypes such as chemotherapy for dedifferentiated and mesenchymal chondrosarcoma and radiation additionally for mesenchymal chondrosarcoma. Emerging radiation technologies may also play a useful role in treating tumors in anatomically complex areas such as the spine or pelvis.

摘要

软骨肉瘤是第二常见的原发性骨肿瘤,超过 90%的病例为原发性常规亚型。除了从头发生长外,常规软骨肉瘤也可能继发于良性基础病变,如软骨瘤或骨软骨瘤。症状通常表现为受影响区域的局部、钝痛、隐痛。分级是这些肿瘤中公认的预后因素。I 级病变/非典型软骨肿瘤很少转移,很少复发,10 年生存率>80%。相比之下,III 级病变预后不良,局部复发率最高,肺转移率>50%,10 年生存率<30%。高级别常规软骨肉瘤的标准治疗方法是广泛切除加边缘。然而,低级别病变可能适合刮除加或不加辅助局部治疗。常规软骨肉瘤对化疗或标准剂量放疗无反应。辅助治疗对某些亚型可能有益,例如去分化和间充质软骨肉瘤的化疗以及间充质软骨肉瘤的放疗。新兴的放射治疗技术也可能在治疗脊柱或骨盆等解剖复杂区域的肿瘤方面发挥有用作用。

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