Puri Ajay
Department of Surgery, Tata Memorial Hospital, HBNI, Mumbai.
EFORT Open Rev. 2020 Feb 26;5(2):90-95. doi: 10.1302/2058-5241.5.190052. eCollection 2020 Feb.
Chondrosarcomas in children and adolescents are uncommon and constitute < 5% of all chondrosarcomas. There are very few studies discussing extremity chondrosarcomas in young patients.The pelvis is the most common site, followed by the proximal femur.As cartilaginous tumours can be quite challenging to diagnose, it is best for these lesions to be discussed in a multidisciplinary meeting which includes a radiologist and a pathologist specializing in bone tumours.Treatment principles are similar to those in adults, with adequate surgical excision respecting oncologic principles being the mainstay of treatment. Select extremity Grade I chondrosarcomas may be managed with extended intralesional curettage without increasing the risk for local recurrence or metastatic disease, but case selection is critical and should be based on clinical, imaging and histological characteristics.Chondrosarcomas are resistant to chemotherapy and relatively radioresistant. For mesenchymal chondrosarcomas, there may be a role for chemotherapy, though data on this is limited.Prognosis and rate of recurrence correlate directly to the adequacy of the surgical resection.Chondrosarcomas in younger patients behave in a similar fashion to those in adults, and outcomes in the young are no different from those in adults. Cite this article: 2020;5:90-95. DOI: 10.1302/2058-5241.5.190052.
儿童和青少年的软骨肉瘤并不常见,占所有软骨肉瘤的比例不到5%。很少有研究讨论年轻患者的肢体软骨肉瘤。骨盆是最常见的部位,其次是股骨近端。由于软骨肿瘤的诊断颇具挑战性,最好在多学科会议上讨论这些病变,会议成员包括放射科医生和专门研究骨肿瘤的病理科医生。治疗原则与成人相似,遵循肿瘤学原则进行充分的手术切除是主要治疗方法。部分肢体I级软骨肉瘤可采用扩大的病灶内刮除术治疗,而不增加局部复发或转移疾病的风险,但病例选择至关重要,应基于临床、影像学和组织学特征。软骨肉瘤对化疗耐药且相对抗拒放疗。对于间叶性软骨肉瘤,化疗可能有一定作用,不过相关数据有限。预后和复发率与手术切除的充分程度直接相关。年轻患者的软骨肉瘤表现与成人相似,年轻患者的治疗结果与成人无异。引用本文:2020;5:90 - 95。DOI: 10.1302/2058 - 5241.5.190052。