Primary leiomyosarcoma of the femur

Leiomyosarcoma usually arises in the uterus, gastrointestinal tract, retroperitoneum, or soft tissue. Primary leiomyosarcoma of bone is rare. We encountered two patients with primary leiomyosarcoma of the femur; one was a 24-year-old woman and the other, a 41-year-old woman. Bone destruction observed on plain radiographs was minimal in both patients. Magnetic resonance imaging (MRI) showed low signal intensity on T1-weighted images and high signal intensity on T2-weighted images, with these areas being much larger than the findings to be expected from the plain radiographs. Histological examination revealed spindle-cell sarcoma, with an interlacing pattern, acidophilic cytoplasm and blunt-ended or "cigar-shaped" nuclei, in both patients. In both patients, immunohistochemical examination showed positive staining for vimentin, desmin, and α-smooth muscle actin. Extensive examination of the gastrointestinal tract and uterus revealed no primary lesion. Therefore, the leiomyosarcoma in the femur was considered to be primary rather than metastatic. Histological examination, including immunohistochemistry, and the exclusion of an extraskeletal primary lesion, is necessary in diagnosing primary leiomyosarcoma of bone. Received: May 15, 2001 / Accepted: October 31, 2001     

Adamantinoma of the Tibia: Local Resection and Distraction Osteogenesis

We describe a case of adamantinoma of the distal tibia in a 31-year-old woman. Segmental resection of the part of the tibia containing the tumour in normal bone was done and the bone defect was bridged with bone transport and external fixation. The tibia healed uneventfully and one year after the operation the patient resumed full activity. Three years postoperatively no local recurrence or metastasis has occurred.     

恶性肿瘤致股骨近端病理性骨折的手术治疗

目的：探讨恶性肿瘤致股骨近端病理性骨折手术治疗的方法。方法：对1997．8～2003．6期间16例股骨近端病理性骨折患者的手术治疗进行回顾性分析。结果：除一例术后死于急性呼吸窘迫综合症外，余15例无手术并发症，术后疼痛明显缓解，伤口愈合良好，均存活在3个月以上，12例术后继续原发肿瘤的治疗。结论：恶性肿瘤致股骨近端的病理性骨折可积极手术治疗。采用带锁髓内钉或人工股骨头置换加骨水泥固定可取得较好的效果。     

Adamantinoma: an unusual bone tumour

Adamantinoma is a rare tumour, which most often affects the tibia and produces lytic and sometimes destructive lesions, which can cause fractures. The lesions occur principally in adults and are more common in males. A small percentage of the patients develop metastases, sometimes quite late in the course. Our institution has treated 42 patients with adamantinomas since 1972 and has evaluated them by imaging studies and histology. The majority of the patients were treated by resection of the lesion and insertion of an intercalary allograft. Only three of the patients died of disease with the time until death ranging from 10 to 17 years. Recurrence occurred in only three patients and the allograft success rate in terms of function was 71% at a mean time of 10 years.     

进行性假性类风湿性发育不良症并双侧股骨髁上骨折1例

正患者,女,12岁。5岁开始逐渐出现行走不稳,左右摇摆,未予重视。2013年出现双髋关节疼痛,逐渐加重,5个月前发展为无法行走,需要坐轮椅。曾被诊断为"脑炎"、"先天发育异常"、"幼年性类风湿"等疾病,均未确诊,症状无改善。1 d前不慎从轮椅上摔下,双膝着地,双膝关节上方处剧烈疼痛,活动不能,为进一步治疗来我院就诊。急诊X线片(图1a,1b)示股骨远端及胫骨近端骨骺及干骺端扩大,双侧股     

Neonatal osteofibrous dysplasia: a case report

We describe a case of neonatal osteofibrous dysplasia that was diagnosed 3 days after birth. Osteofibrous dysplasia is a fibro-osseous lesion rarely seen in long bones. It is also known as congenital osteitis fibrosa or ossifying fibroma of bones. Although it occurs in children under 10 years of age, it is extremely rare in newborn babies. It must be differentiated from adamantinoma due to their radiological appearance, predilection for tibia and fibula, clinical picture, and certain histological similarities.     

截骨矫形钢板固定治疗股骨近段纤维结构不良伴髋内翻畸形

目的 探讨股骨转子下截骨矫形、动力髋或髁螺钉系统固定治疗股骨近段纤维结构不良(fibrous dysplasia,FD)伴髋内翻畸形的临床疗效.方法 回顾性分析2001年4月至2010年5月收治的26例股骨FD伴髋内翻畸形患者的临床资料,男9例,女17例;年龄10～53岁,中位年龄19岁.单骨型14例,多骨型12例.病灶长度为多骨型9～36 cm(平均19 cm),单骨型7～15 cm(平均9 cm);17例合并病理性骨折;术前颈干角65°～110°(平均92°),患肢短缩1.5～4.5cm(平均2.8 cm).21例病灶累及股骨颈者采用髋螺钉固定,5例未累及股骨颈者采用髁螺钉固定.结果 手术时间80～170 min(平均120min),出血量280～1650ml(平均960ml).术后颈干角为119°～140°(平均127°);患肢平均延长2.3 cm(1.5～3.6 cm).随访9～118个月(平均39个月),截骨面均愈合.除1例颈干角从术后126°减小到术后56个月的115°,其余患者无髋内翻畸形复发,无内固定断裂或松动.1例股骨颈变短、髋螺钉沿滑槽滑动,但螺钉未切割股骨头;1例术后7年外伤后钢板远端处股骨干骨折.根据Guille的功能评价标准,24例满意、2例不满意.结论 转子下截骨矫形、动力髋或髁螺钉系统内固定能有效地纠正股骨上段FD伴髋内翻畸形,改善患肢功能.

Abstract：

Objective To investigate the clinical effect of subtrochanteric osteotomy plus dynamic hip/condyle screw (DHS/DCS)fixation to treat the fibrous dysplasia of the proximal femur with coxa vara.Methods Twenty-six clinical cases of femoral fibrous dysplasia with coxa vara were retrospectively analysed from April 2001 to May 2010.There were 9 males and 17 females,with a median age of 19(10 to 53).Forteen patients presented with monostotic disease,and 12 with polyostotic disease.The length of the lesion for polyostotic disease was from 9 cm to 36 cm,while for monostotic disease was from 7 cm to 15 cm.Seventeen cases were merged with pathologic fracture.The neck shaft angles were from 65 to 110 degree preoperation.The shortage of limbs was from 1.5 cm to 4.5 cm.Twenty-one patients involved in femoral neck were fixed with DHS and the other 5 cases with DCS.Results The duration of operation was from 80 to 170 min.The amout of bleeding was from 280 to 1650 ml with the average of 960 ml.The average postoperative neck shaft angles were 127 degree(119 to 140).The shortened limbs were extended 2.3 cm for average (1.5 to 3.6 cm).The follow-up time was from 9 to 118 months with the average of 39 months.All patients with osteotomy were healed.The neck shaft angle of 1 case decreased from post-operative 126°to 115°56months post-operatively,no coxa adducta recurrented and all internal fixations were in position.Hip screw backed out through the barrel in one case with the shortage of femoral neck.One case had femoral fracture after an injury.According to Guille function standard,24 cases were regarded as satisfied and 2 cases as unsatisfied.Conclusion Subtroehanteric osteotomy plus DHS/DCS fixation can effectively correct the fibrous dysplasia of the proximal femur with coxa vara,and significantly improve the function.     

股骨近段纤维结构不良内翻畸形的手术疗效

目的 评估和分析股骨近段纤维结构不良内翻畸形手术治疗的临床疗效.方法 2000年8月至2009年5月,采用截骨矫形、病灶刮除、打压植骨、股骨重建钉内固定治疗21例(24个)股骨纤维结构不良伴重度内翻畸形患者,男12例,女9例;年龄14～39岁,平均22.7岁.其中15例为单纯髋内翻畸形,6例同时伴髋内翻及股骨内翻畸形.颈干角55°～105°,平均75°;股骨内翻角18°～45°,平均30°;患侧股骨长度较对侧短缩2.0～4.5 cm,平均3.4cm.其中13例患者因发生病理性骨折而行手术治疗.结果 21例患者均获得随访,随访时间21个月至7年,平均3年4个月.24个股骨共30处截骨面中,28处于术后3～6个月骨性愈合,2例双段远端截骨面各有1处在术后12个月时仍不愈合,经再次植骨3个月后骨性愈合.21例内翻畸形股骨力线均完全矫正,术后颈干角矫正为95°～135°,平均118°;股骨内翻角完全纠正.股骨长度较术前延长1.8～3.6 cm,平均2.8cm.术后无一例患者发生感染、畸形进展及再骨折.17例患者疼痛视觉模拟评分(visual analogue scales,VAS)由术前7～10分降至术后0分,4例VAS评分由术前8～10分降至术后3～4分.Harris髋关节功能评分优12例,良6例,可3例.结论 外翻截骨可有效矫正内翻畸形,股骨重建钉可稳定股骨,大量打压式植骨可有效恢复骨量.

Abstract：

Objective To evaluate and analyse the effectiveness of surgical treatment for fibrous dysplasia in proximal femur with severe varus deformity.Methods A retrospective study was performed in 21 patients (24 femora)of fibrous dysplasia who were treated in our hospital between August 2000 and May 2009.All patients had severe femoral varus deformity.The four-step procedures were performed orderlv as valgus osteotomy,lesion curettage,impacting of massive bone allograft,and fixation by femoral intramedullary nail.There were 6 patients with monostotic disease,15 with polyostotic diseases,including 12 males and 9 females with a mean age of 22.7 years(range,14-39 years).The average neck-shaft angle and femoral varus angle was 75°(range 55°-105°)and 30°(range,18°-45°),respectively.The average length of thigh shortened 3.4 cm(range,2.0-4.5 cm)compared with the contralateral thigh.Results All of the 21 patients were followed up from 21 months to 7 years with an average period of 3 years and 4 months.There were 30 osteotomy sites in 24 femurs,28 osteotomy sites showed bone union after 3-6 months from surgery.Two distal location of the double-level osteotomy showed nonunion,which received bone grafting again and got bone union after 3 months finally.The femoral mechanical alignments of the 21 patients had been recriticd.The average neck-shaft angle was 118°(range,95°-135°)postoperatively,the femoral varus angle disappeared.The average extremity lengthening was 2.8 cm(range,1.8-3.6 cm)postoperatively.There were no infection,recurrent fracture and progression of deformity.The visual analogue scales(VAS)score of 17 patients decreased to zero postoperatively from 7-10 preoperatively,and that of the other 4 patients decreased to 3-4 postoperatively from 8-10 preoperatively.The result of Harris hip functional score was excellent in 12 cases,good in 6,and fair in 3.Conclusion The valgus osteotomy can rectify varus deformity effectively.The reconstract nail of the fumer can support the stability of femur.Impacting of massive bone allograft can improve the capacity of the femur.     

1例股骨下段孤立性浆细胞瘤的诊治并文献综述

[目的]报道1例股骨下段孤立性浆细胞瘤的诊治并综述相关文献.[方法]查阅有关骨内孤立性浆细胞瘤的文献,结合1例发生于股骨下段孤市性浆细胞瘤的诊治过程,从发病、诊断、治疗、预后等方面认识该罕见病.[结果]孤立性浆细胞是一种以单克隆浆细胞失控增殖为特征的少见肿瘤;活检病理及免疫细胞化学检测确诊后,局部彻底切除和放疗是当前较有效的治疗方案;该病的预后和M蛋白水平及持续时间、原发瘤大小、有无复发等因素有关.[结论]孤立性浆细胞瘤是一种易向多发性骨髓瘤恶变的罕见肿瘤,及时的确诊、手术、放疗以及长期随访对改善预后很重要.     

Malignant solitary tumor in a child: a case report and review of the literature

Solitary fibrous tumor in the mesentery is rare. We report a case of a malignant solitary fibrous tumor in the appendical mesentery of 6-year-old boy. Computed tomography and ultrasound of the abdomen demonstrated a well-defined solid mass, 4.0 cm in diameter, in the lower right abdomen. At laparotomy, an encapsulated tumor was observed in the appendical mesentery and was easily separated from the appendix. Immunohistochemistry stain showed that the spindle-shaped cells were positive for CD34 and neuron-specific enolase. DOG-1, CD117, desmin, S-100 protein, and SMA were negative. The sequence of KIT and platelet-derived growth factor receptor α was presented with wild type, no mutation. The patient received the reoperation 5 months postoperation. Despite these treatments, the relapsed tumor rapidly and markedly enlarged. The patient died of recurrent tumor with liver dissemination 7 months after reoperation.     

Adamantinoma of the tibia: a clinicoradiologic study of four cases

Adamantinoma of long bones is a malignant primitive bone tumor with epithelial origin, involving the tibia in a great majority of cases. It is a rare neoplasm with less than 200 cases reported in the world literature. The authors report four cases of tibial adamantinoma and describe the clinical, imaging and histologic features of this neoplasm with emphasis on preoperative imaging, surgical treatment and post-operative follow-up.     

骨干人工假体在股骨近端恶性肿瘤切除后骨缺损重建中的初步应用

 目的 探讨采用骨干人工假体对股骨近端骨干恶性肿瘤切除后结构性骨缺损进行重建的可行性。方法 2011年6月至2014年7月对7例股骨近端恶性骨肿瘤患者采用骨干人工假体置换,男3例,女4例;年龄28~76岁,平均61.8岁。原发恶性骨肿瘤4例,转移癌3例。术前常规行X线、CT及MR检查,通过MRI测量瘤段骨长度及软组织侵犯范围,再根据截骨长度选择骨干人工假体配件。取股骨近端外侧直切口,逐层切开皮肤、皮下组织,按肿瘤切除原则充分显露术前MRI测量的瘤段骨及软组织肿瘤并截骨,扩髓后安装骨干人工假体,依次缝合各层组织。术后切除组织送病理学检查。结果 手术时间45~120 min,平均90 min;术中出血量200~800 ml,平均400 ml。随访6~24个月,平均14个月。1例骨肉瘤患者术后1年因出现肺转移而死亡,3例原发恶性骨肿瘤患者术后未出现术区复发及远处转移。1例乳腺转移癌患者术前多发转移,术后行综合治疗,带瘤生存;2例乳腺转移癌患者随访期间未出现原位复发或转移。7例患者术后患肢髋关节功能均无受限。结论 使用骨干人工假体对股骨近端骨干恶性肿瘤切除后结构性骨缺损进行重建,保留了邻近的髋关节及肌腱附着点,操作简单,住院时间较短,可早期负重,是一种理想的治疗股骨近端骨肿瘤的保肢手术方法。     

股骨近端内侧支撑固定的有限元分析

目的通过有限元分析股骨转子间骨折六部分分型各型骨折经股骨近端内侧钢板固定后的稳定性,探讨股骨近端内侧钢板对股骨近端内侧壁的支撑作用。方法运用有限元法建立股骨转子间骨折六部分分型各型骨折以股骨近端防旋髓内钉(PFNA)、内侧钢板(双皮质)、PFNA+内侧钢板(单皮质)固定后的模型。按参考文献提供参数,对模型的边界条件及材料属性进行设定,按体重70 kg的成人正常行走时髋部受力峰值进行加载。按上述条件得到21组内固定模型的应力与形变位移图,分析各组模型重要部位的应力峰值及各骨折块的位移数据。结果PFNA固定各型骨折时,股骨各重要位置承受应力变化及骨折块位移幅度均较小,大转子部位的骨折块位移增加幅度稍大;内侧钢板固定时,随着骨折块的增加,尤其是外侧骨折块的增多,股骨重要位置处及内固定自身所承受的应力均明显的增加,骨折块位移大幅增加,但内侧骨折块位移幅度始终较小;PFNA+内侧钢板固定时,股骨各重要位置承受应力变化及骨折块位移幅度是3种内固定方式中最小的。结论股骨近端内侧钢板能有效减小股骨近端应力的集中程度,对股骨近端内侧壁起到较好的支撑作用;对于不稳定型骨折应用PFNA辅助内侧钢板固定时较单独应用PFNA能有效降低股骨所受应力,各骨折块位移均未见明显增加,且内固定本身应力未见明显异常,能起到坚强固定的作用。     

股骨大转子滑囊结核并发大转子骨质破坏1例

患者,男,41岁,右侧髋关节外侧间歇性疼痛3年,加重1年入院。3年前无明显诱因出现右侧髋关节外侧不适、疼痛,疼痛性质为酸痛感,常可耐受,在行走、下蹲、起立、上台阶或肢体被动内收时疼痛感加重,平时不能侧卧,休息时疼痛可缓解。1年前上述症状加重,患者未对上述症状采取任何治疗措施。2011年12月14日就诊于我院,门诊以“右侧大转子骨质破坏,右大转子区软组织肿物”收入院。患者否认结核病病史,     

巨大股骨近端软骨肉瘤1例

1 病例资料 患者,女,44岁,因左大腿疼痛半个月于2011年6月入院.8年前无明显诱因出现左大腿肿块,偶感酸胀,后肿块进一步长大,经外院影像学检查显示左股骨肿瘤,行病灶清除术.术后复发,先后2次做病灶清除术.近1年来肿块日趋增大,致行走不便,无疼痛,半个月前无明显诱因出现左大腿酸痛,不能行走,自行口服曲马多缓释片止痛,疗效欠佳.     

Solitary lymphangioma of the femur. A case report

 Lymphangioma of the bone is a benign lesion, characterized by newly formed lymph vessels. It usually occurs in a diffuse manner or as multiple lesions. Solitary lymphangioma of the bone is an extremely rare condition. We herein describe a case of solitary lymphangioma of the femur in a 55-year-old woman who presented with knee pain. Radiographs showed an eccentric osteolytic lesion in the distal femur. This is the first reported case of solitary lymphangioma of the bone occurring in the femur. Received: December 7, 2001 / Accepted: February 18, 2002