We report a case of a 32 year-old male, admitted for a lytic lesion of the distal femur. One month after the first X-ray, clinical and imaging deterioration was evident. Open biopsy revealed fibrous dysplasia. Three m...We report a case of a 32 year-old male, admitted for a lytic lesion of the distal femur. One month after the first X-ray, clinical and imaging deterioration was evident. Open biopsy revealed fibrous dysplasia. Three months later, the lytic lesion had spread to the whole distal third of the femur reaching the articular cartilage. The malignant clinical and imaging features necessitated excision of the lesion and reconstruction with a custom-made total knee arthroplasty. Intraoperatively, no obvious soft tissue infiltration was evident. Nevertheless, an excision of the distal 15.5 cm of the femur including 3.0 cm of the surrounding muscles was finally performed. The histological examination of the excised specimen revealed central low-grade osteosarcoma. Based on the morphological features of the excised tumor, allied to the clinical findings, the diagnosis of low-grade central osteosarcoma was finally made although characters of a fibrous dysplasia were apparent. Central low-grade osteosarcoma is a rare, well-differentiated sub-type of osteosarcoma, with clinical, imaging, and histological features similar to benign tumours. Thus, initial misdiagnosis is usual with the condition commonly mistaken for fibrous dysplasia. Central low-grade osteosarcoma is usually treated with surgery alone, with rare cases of distal metastases. However, regional recurrence is quite frequent after close margin excision.展开更多
目的分析骨骺牵开保骺术在儿童股骨骨肉瘤保肢术中的疗效及应用价值。方法 2007年1月-2011年1月,收治6例股骨远端骨肉瘤患儿。男4例,女2例;年龄9～14岁,平均11.4岁。病程1～9个月,平均4.8个月。术前经穿刺或切开活检证实为骨肉瘤,其中...目的分析骨骺牵开保骺术在儿童股骨骨肉瘤保肢术中的疗效及应用价值。方法 2007年1月-2011年1月,收治6例股骨远端骨肉瘤患儿。男4例,女2例;年龄9～14岁,平均11.4岁。病程1～9个月,平均4.8个月。术前经穿刺或切开活检证实为骨肉瘤,其中成骨型骨肉瘤1例,软骨母细胞型骨肉瘤1例,骨母细胞型骨肉瘤1例,未明确细胞分类的骨肉瘤3例。根据Enneking提出的外科分期系统,其中ⅡA期1例,ⅡB期5例。术前采用新辅助化疗2个周期;首先行Ca adell骨骺牵开保骺术,牵开时间4～7 d,平均5.7 d;骨骺牵开保骺术完成1～2 d后行骨肿瘤切除,采用大段同种异体骨移植重建;术后进一步化疗并定期随访了解骨折愈合、下肢发育及局部和全身并发症等情况,并根据美国肌肉骨骼肿瘤学会(MSTS)评分系统评分及双膝关节活动度(range of motion,ROM)进行功能评定。结果术后发生切口浅表感染1例,经换药治疗后愈合;其余患儿切口均Ⅰ期愈合。6例均获随访,随访时间1～5年,平均2.5年;无明显肢体肿胀及疼痛等症状,未发生同种异体骨排斥反应及内固定物松动、断裂等。术后随访无肿瘤转移及局部复发等并发症发生。术后6～9个月干骺端骨性愈合5例,14个月1例;骨干部位均发生延迟愈合,术后12～48个月骨折端均有不同程度骨痂形成,但骨折线仍清晰可见。末次随访时患肢短缩1～3 cm 4例,3～5 cm 2例;发生代偿性脊柱侧弯3例,行走明显跛行2例;MSTS评分为(27.20±1.92)分,与术前(19.60±2.74)分比较差异有统计学意义(t=—4.12,P=0.00);患侧膝关节ROM为(127.00±17.89)°,与术前(109.00±12.45)°比较差异无统计学意义(t=—1.84,P=0.10),与健侧膝关节ROM(126.00±9.62)°比较差异无统计学意义(t=—0.11,P=0.92)。结论骨骺牵开保骺术可应用于骨骺尚未闭合的儿童股骨骨肉瘤保肢术中,具有操作简便、临床疗效好、术后并发症少等优点。展开更多
文摘We report a case of a 32 year-old male, admitted for a lytic lesion of the distal femur. One month after the first X-ray, clinical and imaging deterioration was evident. Open biopsy revealed fibrous dysplasia. Three months later, the lytic lesion had spread to the whole distal third of the femur reaching the articular cartilage. The malignant clinical and imaging features necessitated excision of the lesion and reconstruction with a custom-made total knee arthroplasty. Intraoperatively, no obvious soft tissue infiltration was evident. Nevertheless, an excision of the distal 15.5 cm of the femur including 3.0 cm of the surrounding muscles was finally performed. The histological examination of the excised specimen revealed central low-grade osteosarcoma. Based on the morphological features of the excised tumor, allied to the clinical findings, the diagnosis of low-grade central osteosarcoma was finally made although characters of a fibrous dysplasia were apparent. Central low-grade osteosarcoma is a rare, well-differentiated sub-type of osteosarcoma, with clinical, imaging, and histological features similar to benign tumours. Thus, initial misdiagnosis is usual with the condition commonly mistaken for fibrous dysplasia. Central low-grade osteosarcoma is usually treated with surgery alone, with rare cases of distal metastases. However, regional recurrence is quite frequent after close margin excision.
文摘目的分析骨骺牵开保骺术在儿童股骨骨肉瘤保肢术中的疗效及应用价值。方法 2007年1月-2011年1月,收治6例股骨远端骨肉瘤患儿。男4例,女2例;年龄9～14岁,平均11.4岁。病程1～9个月,平均4.8个月。术前经穿刺或切开活检证实为骨肉瘤,其中成骨型骨肉瘤1例,软骨母细胞型骨肉瘤1例,骨母细胞型骨肉瘤1例,未明确细胞分类的骨肉瘤3例。根据Enneking提出的外科分期系统,其中ⅡA期1例,ⅡB期5例。术前采用新辅助化疗2个周期;首先行Ca adell骨骺牵开保骺术,牵开时间4～7 d,平均5.7 d;骨骺牵开保骺术完成1～2 d后行骨肿瘤切除,采用大段同种异体骨移植重建;术后进一步化疗并定期随访了解骨折愈合、下肢发育及局部和全身并发症等情况,并根据美国肌肉骨骼肿瘤学会(MSTS)评分系统评分及双膝关节活动度(range of motion,ROM)进行功能评定。结果术后发生切口浅表感染1例,经换药治疗后愈合;其余患儿切口均Ⅰ期愈合。6例均获随访,随访时间1～5年,平均2.5年;无明显肢体肿胀及疼痛等症状,未发生同种异体骨排斥反应及内固定物松动、断裂等。术后随访无肿瘤转移及局部复发等并发症发生。术后6～9个月干骺端骨性愈合5例,14个月1例;骨干部位均发生延迟愈合,术后12～48个月骨折端均有不同程度骨痂形成,但骨折线仍清晰可见。末次随访时患肢短缩1～3 cm 4例,3～5 cm 2例;发生代偿性脊柱侧弯3例,行走明显跛行2例;MSTS评分为(27.20±1.92)分,与术前(19.60±2.74)分比较差异有统计学意义(t=—4.12,P=0.00);患侧膝关节ROM为(127.00±17.89)°,与术前(109.00±12.45)°比较差异无统计学意义(t=—1.84,P=0.10),与健侧膝关节ROM(126.00±9.62)°比较差异无统计学意义(t=—0.11,P=0.92)。结论骨骺牵开保骺术可应用于骨骺尚未闭合的儿童股骨骨肉瘤保肢术中,具有操作简便、临床疗效好、术后并发症少等优点。
