Tata Memorial Hospital, Mumbai, India.
J Pediatr Orthop B. 2020 Sep;29(5):505-509. doi: 10.1097/BPB.0000000000000641.
The objective of this study was to evaluate the characteristics and oncologic outcomes of a single institution series of chondrosarcoma of pelvis and extremities in patients who were 21 years or younger. Between January 2006 and December 2016, we operated on 249 chondrosarcomas of the pelvis and extremities. Eleven (4%) of these were 21 years or younger. The mean age was 18 years (range 14-21 years). There were eight males and three females. Four occurred in the pelvis. The other sites were humerus (three) and one each in the scapula, clavicle, metatarsal and proximal femur. There were five primary chondrosarcomas and 6 (55%) secondary chondrosarcomas. After appropriate surgical excision, we had nine free margins and two margins were microscopically involved. None of the 11 patients received adjuvant chemotherapy or radiotherapy. One patient never followed up after surgery and another was lost to follow-up after 32 months. Eight were alive and one had died. All survivors had minimum follow-up of 2 years (range 26-106 months). There was one local recurrence. The overall and disease-free survival at 5 years for all patients was 89 %. There was no statistically significant difference in survival for primary chondrosarcomas compared to secondary chondrosarcomas (P = 0.061), or pelvic chondrosarcomas compared to other sites (P = 0.264). Chondrosarcomas in children and adolescents constitute <5% of all chondrosarcomas. Secondary chondrosarcomas constituted more than half the cases. Overall, outcomes in the young were no different from those in adults.
本研究旨在评估 21 岁及以下患者骨盆和四肢软骨肉瘤的特征和肿瘤学结果。2006 年 1 月至 2016 年 12 月，我们对 249 例骨盆和四肢软骨肉瘤患者进行了手术。其中 11 例（4%）为 21 岁及以下患者。平均年龄为 18 岁（范围 14-21 岁）。其中男性 8 例，女性 3 例。4 例发生在骨盆。其他部位分别为肱骨（3 例）、肩胛骨、锁骨、跖骨和股骨近端各 1 例。其中 5 例为原发性软骨肉瘤，6 例（55%）为继发性软骨肉瘤。在适当的手术切除后，我们有 9 例为无瘤切缘，2 例为镜下切缘受累。11 例患者中均未接受辅助化疗或放疗。1 例患者术后未随访，另 1 例患者随访 32 个月后失访。8 例患者存活，1 例死亡。所有幸存者的随访时间均至少为 2 年（范围 26-106 个月）。其中 1 例出现局部复发。所有患者的总生存率和无病生存率为 5 年 89%。原发性软骨肉瘤与继发性软骨肉瘤（P=0.061）或骨盆软骨肉瘤与其他部位（P=0.264）的生存率无统计学差异。儿童和青少年的软骨肉瘤占所有软骨肉瘤的<5%。继发性软骨肉瘤占半数以上病例。总体而言，年轻患者的预后与成年患者无差异。
