Department of Surgical Oncology, Sri Devaraj Urs Academy of Higher Education & Research, Tamaka, Kolar 563101, Karnataka, India.
Consultant Medical Oncologist, Royal Marsden Hospital NHS Trust, 203 Fulham Rd, London SW3 6JJ, UK.
Future Oncol. 2021 Dec;17(35):4983-4991. doi: 10.2217/fon-2021-0830. Epub 2021 Nov 4.
Dedifferentiated chondrosarcomas are aggressive variants of chondrosarcoma, associated with poor outcomes. Tumor biphasism is the norm. The majority of these tumors are symptomatic at presentation. Radiologically, large soft tissue masses with bony destruction predominate. Treatment protocols of these tumors are not well defined. Surgical resection forms the standard of care for localized disease. (Neo)adjuvant therapies remain controversial as the results from multiple (mainly retrospective) studies remain conflicting. Age at presentation, stage and ability to obtain negative resection margins are important prognostic factors. The overall prognosis is dismal. Newer and novel therapies targeting the complex genetic makeup of these tumors have renewed interest in the adjuvant setting that could hold promise in the near future.
去分化软骨肉瘤是软骨肉瘤的侵袭性变异型，预后不良。肿瘤的双相性是常态。这些肿瘤大多数在出现时就有症状。放射学上，以骨破坏为主的大软组织肿块占主导地位。这些肿瘤的治疗方案尚未明确。手术切除是局部疾病的标准治疗方法。（新）辅助治疗仍然存在争议，因为多项（主要是回顾性）研究的结果仍然存在冲突。发病时的年龄、分期和获得阴性切缘的能力是重要的预后因素。总体预后较差。针对这些肿瘤复杂遗传结构的新型治疗方法在辅助治疗方面重新引起了人们的兴趣，这在不久的将来可能会有希望。
