Adamantinoma associated with fibrous dysplasia

Summary Three cases of adamantinoma of the tibia, seen in one hospital over a period of 7 years, are described. They illustrate the difficulty in differentiating this lesion from fibrous dysplasia on radiographic and histological grounds. Thermography may help in the differential diagnosis.

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Résumé Trois cas d'adamantinome du tibia ont été observés en 7 ans dans un même hôpital. Ils illustrent la difficulté qu'il y a à distinguer cette lésion de la dysplasie fibreuse en se fondant sur des arguments radiologiques et histologiques. La thermographie peut aider au diagnostic différentiel.

     

Aggressive aneurysmal bone cyst in association with polyostotic fibrous dysplasia: A case report

IntroductionAneurysmal bone cyst occurring in the setting of previously diagnosed fibrous dysplasia is rare. While both are benign processes, pain, compression of nearby structures and risk of fracture can require treatment.Presentation of caseIn this report, we describe a 56 year old male who developed an aggressive aneurysmal bone cyst secondary to fibrous dysplasia in the proximal tibia over a period of 8 months. He required an above knee amputation for disease and symptom control due to the aggressive nature of disease and medical comorbidities.DiscussionThe diagnosis of a secondary lesion can prove difficult. It is important to exclude a malignant disease process, particularly when imaging demonstrates an aggressive appearance. In this case, repeat imaging, CT guided biopsies and an open biopsy were performed to exclude malignancy prior to definitive surgical management.ConclusionIn order to exclude secondary lesions, we suggest further investigation for new onset pain in the setting of a benign lesion.     

病灶清除、植骨联合内固定治疗下肢长管状骨纤维结构不良的疗效和并发症观察

背景：骨纤维结构不良临床上可表现为单发或多发的骨骼畸形、疼痛和病理性骨折。目前该病的治疗方法以手术清除病灶、植骨内固定为主。目的：探讨病灶清除、植骨联合髓外固定治疗下肢长管状骨纤维结构不良的疗效和可行性。方法方法：选取2010年2月至2012年11月我院收治的15例骨纤维结构不良患者的病例资料进行回顾性分析,男8例,女7例;年龄26-48岁,平均（32.1±1.2）岁;股骨9例,胫骨6例。均采用病灶清除,取髂骨植骨,动力髋螺钉或锁定接骨板固定。围手术期予抗炎、补液对症处理,术后石膏固定,指导患者功能锻炼。髋关节功能按Harris评分系统评定。膝关节功能按Merchan评分系统评定。术后定期摄X线片,观察肿瘤复发情况,植骨融合情况和不良反应发生情况。结果：所有伤口均甲级愈合,按期拆线。所有患者均完成18个月随访,无1例复发,植骨均融合,术后部分负重下地时间（4.3±0.6）个月,X线片未见骨溶解,术后（4.0±0.8）个月可见新生骨长入,2例患者残留手术区疼痛,对症治疗后缓解。术后髋关节功能Harris评分为97.2分,膝关节功能Merchan评分为95.8分,均较术前显著提高（P〈0.05）。结论：病灶清除、植骨联合髓外固定在下肢长管状骨纤维结构不良患者中固定可靠,预后佳,是一种可行的治疗方法。     

带血运骨膜内组合双段腓骨移植治疗下肢长骨骨纤维结构不良致大段骨缺损

目的 探讨下肢长骨大段骨纤维结构不良切除后骨缺损的修复方法.方法 1995年5月至2003年8月,6例骨纤维结构不良患者.男5例,女1例.年龄14～40岁(平均25.5岁).胫骨干2例,股骨干4例.肿瘤切除后骨缺损的长度为15～22 cm.移植双腓骨段长度17～24 cm.均从双侧切取带腓血管的腓骨,在一端将腓血管吻合,使成一条腓血管相连的双段腓骨.将双段腓骨的前内侧骨膜从中央纵行切开并向两侧剥离至骨嵴.双段腓骨折叠,其前内侧面相对,两断端同定,将相对缘游离的骨膜纵行缝合,使其组合为一个骨膜包裹的粗骨.将与双段腓骨相连的腓血管襻在移植体一端制成"U"形,勿形成锐角.能保证血管供血不受影响.解剖出受区血管.将组合的腓骨插入骨折两断端间,如不稳定,加用钢丝或螺钉固定.精确无误地将腓血管与受区血管进行吻合,通过移植体骨膜出血情况判断血管吻合口情况.结果 随访6～10年,平均7.4年.1年后骨性愈合;5年后5例移植骨髓腔再通.移植的双腓骨未发生肿瘤,X线片显示为正常骨骼;1例股骨干于正常骨干远段复发,但移植骨部分未复发.骨移植体愈合好,无发生骨不连、骨感染等并发症.膝关节伸屈功能恢复正常,逐渐恢复日常活动与工作.结论 骨膜内组合双腓骨移植足修复下肢长骨大段缺损的良好方法 .     

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.     

全身多发性骨纤维异样增殖症合并动脉瘤样骨囊肿1例

1 病例资料 患者,女,24岁。主诉：双上肢左大腿反复疼痛9年。病史：9年前外伤后摄X线片见左股骨和胫骨病变,外院诊为骨纤维异样增殖症,行股骨和胫骨肿瘤刮除自体并异体骨植骨术。此后又发现多处病灶、外院于2001-2002年先后两次对左胫骨无端和左肱骨行肿瘤刮除异体骨植骨术。     

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.     

脊柱骨纤维结构不良1例

1 病例资料 患者,女,64岁。2006年6月23日就诊。5年前无明显诱因出现双下肢无力,自觉腿软,抬腿无力。     

颅骨多发骨纤维异常增殖症1例

患者，女，48岁，13岁时开始出现无诱因的头疼，初期呈间歇性后逐渐加重，严重时头疼剧烈不能睡眠。在当地医院就诊未发现明显异常，只进行了对症治疗，15岁发现下颌骨异样改变，呈不对称性右恻外突。到积水潭医院就诊，诊断为右侧下颌骨纤维异常增殖症，因年龄小术后易复发而未治疗。20岁左右上颌骨出现与下颌骨相同之异样改变。直至30岁后病情出现稳定，但每年春季仍有轻度头疼症状。     

骨纤维结构不良中癌基因蛋白的表达及意义

了解骨纤维结构不良中癌基因蛋白的表达情况。方法应用免疫组化法对３７例骨纤维结构不良行多种癌基因蛋白检测。结果３７例实验组中ｃ－ｆｏｓ、ｃ－ｍｙｃ蛋白均有高表达（Ｐ＜０．０１）。ｒａｓｐ２１蛋白在该病恶变组中有高表达（Ｐ＜０．０５）。结论ｃ－ｆｏｓ、ｃ－ｍｙｃ蛋白过度表达在骨纤维结构不良的发生发展中起重要作用，ｒａｓｐ２１蛋白过度表达在该病的恶性变过程中起作用。本研究结果对骨纤维结构不良的诊断和治疗具有广阔的应用前景。     

Scoliosis and spine involvement in fibrous dysplasia of bone

Few studies focused on the prevalence of scoliosis and involvement of the spine in patients with fibrous dysplasia (FD) of bone. We examined for FD involvement of the spine and scoliosis in 56 patients affected by FD of bone. Fifty patients were part of a cohort reported in a multicentric study on FD promoted by European Pediatric Orthopedic Society (EPOS) in 1999, and six were new patients. There were 30 females and 26 males (mean age 12.5 years; range 1–42 years). Twenty-three had monostotic FD, 9 polyostotic FD, and 24 McCune-Albright Syndrome (MAS). Scoliosis was observed in 11 cases of polyostotic FD and MAS (33.3%). In seven of the patients with scoliosis (63.3%) spine was involved by FD lesional tissue. FD lesions involved the thoracic or lumbar spine in all patients but one, where cervical spine was also affected. A correlation between scoliosis and either spinal (p < 0.01) or pelvic lesions (p < 0.05) and pelvic obliquity (p < 0.01) was observed. Three of the 11 patients showed familiarity for scoliosis but in 2 of them spine was involved by FD. Scoliosis and spine involvement were never detected in monostotic FD. This study indicates that in FD patients with polyostotic disease (1) the prevalences of FD involvement of the spine and scoliosis are high enough to include spine in the clinico-radiographic survey of these patients, and (2) the involvement of the spine and pelvis by FD lesions and pelvic obliquity are important determinants in the occurrence of scoliosis.     

经眶外侧颅内外联合径路治疗颅眶骨纤维异常增殖症

目的 探讨颅眶骨纤维异常增殖症手术治疗的径路. 方法 对累及额骨、眼眶、蝶骨、颞骨的复杂颅面骨纤维异常增殖症患者,应用经眶外侧颅内外联合径路进行手术治疗,包括眶外侧及颞部开窗,病灶的部分或大部切除,视神经减压,颅眶骨外形重建等. 结果 自2005年以来,于临床治疗8例患者,术后随访9个月至3年,无明显并发症发生,复杂颅眶部畸形得以矫正,视力障碍有所改善.头颅CT示骨瓣愈合良好,未见病变复发. 结论 经眶外侧颅内外联合径路可作为治疗颅眶部骨纤维异常增殖症的首选方法.     

骨纤维结构不良的显微外科修复

为了解骨纤维结构不良的显微外科修复的效果，回顾性研究了1981年以来我院收治的25例患者。病变部位：单发局限型肱骨3例．股骨8例；单发广泛型肱骨1例，股骨7例．胫骨6例。采用7种带不同血管蒂的骨（膜）瓣移位、移植术修复，随访时间半年至17年，仅1例复发，无恶变病例，平均骨愈合时间8～10周．原有骨畸形得到不同程度矫正，关节功能恢复良好。显微骨移植对单发型骨纤维结构不良具有良好的效果。     

肋骨骨纤维异常增殖症2例

病例1．患者男．33岁。发现左侧前胸壁肿块,运动后自觉胸闷、不适10余年。触诊肿块不活动．有轻压痛。实验室检查未见异常。CT：左侧第3肋骨前端呈囊状膨胀性改变,约4．12cm×8．30cm,其内可见纤细的骨嵴（图1）,向胸内生长,相邻肺组织受压．周围皮肤隆起;骨皮质变薄周边硬化,未见骨膜反应及骨质破坏改变。行左侧第3前肋骨切除术。病理：镜下可见大量增生的纤维组织、骨样组织和新生骨小梁。诊断：肋骨骨纤维异常增殖症。     

脊柱纤维结构不良的影像学特点

目的 :探讨脊柱纤维结构不良的影像学特征。方法 :回顾性分析2004年4月~2014年1月收治的10例经手术病理确诊的脊柱纤维结构不良患者的影像学资料,其中颈椎3例,胸椎4例,腰椎3例。男6例,女4例。所有病例均行X线平片、CT和MRI检查。根据影像学检查结果系统评估病灶部位、范围、基质密度、边界、附件有无受累、受累椎体骨皮质有无破坏,有无软组织肿块。结果:脊柱纤维结构不良X线平片表现为圆形或类圆形囊状低密度影,4例位于胸椎者显示不佳,余6例中5例基质密度均匀,6例边界清晰,所有患者均未见皮质破坏及软组织肿块。CT表现为类圆形低密度影,边界清晰,骨皮质略微变薄但连续性未破坏。基质密度均匀者7例,9例位于椎体内,1例累及附件骨,均未见皮质破坏及软组织肿块。MRI均表现为TIWI中均匀长T1信号,T2WI呈混杂偏低信号,在TIWI、T2WI及T2抑脂像中病灶周围均有较完整的低或极低信号环状带;2例增强扫描时病灶信号轻度强化。结论:脊柱纤维结构不良具有一定的良性瘤样病变的影像学特征,包括基质均匀、边界清晰、明显的硬化缘、无骨皮质破坏和软组织肿块,这些特征有助于诊断和鉴别诊断。     

Low-grade central osteosarcoma of distal femur,resembling fibrous dysplasia

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. Intra-operatively, 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.     

跟骨外踝骨纤维结构不良1例

1病例资料患者,女,30岁。于5年前无明显诱因出现左踝关节处疼痛,肿胀不适,劳累、受凉、行走后加重,休息后缓解。在当地医院口服药物治疗效果不佳。症状加重半个月入我院。查体:左踝关节轻度肿胀,肤色不红,皮温不高,外踝局部稍压痛,踝关节活动正常。X线和CT检查示:左跟骨、外踝囊性变,跟骨病灶位于跟距关节处(图1～3)。实验室检查血常规、血沉均正常,碱性磷酸酶正常。术前检查无手术禁忌,硬膜外麻醉下行左跟骨、外踝病灶清除术和跟距关节植骨融合术,术中病灶呈磨砂玻璃样改变。术后踝关节石膏制动6周。1年后复查无疼痛,足踝活动良好。病理…     

颅颌面骨纤维异常增殖症的手术治疗

目的 探索颅颌面骨纤维异常增殖症及其造成的颅颌面骨骼畸形的有效安全的手术矫正方法.方法 根据患者的具体情况设计不同手术切除和重建的方案,经头皮冠状切口或口内切口及下睑缘切口入路,对病灶进行完全或部分切除,采用自体颅骨外板或内板、下颌骨外板、Med-por、钛网、个性化钛修复体及人工骨重建颅颌面骨骼支架.结果 19例中,完全切除4例,不完全切除15例.自体颅骨内板修复1例,下颌骨外板修复2例,Medpor修复3例,钛网2例,个性化钛修复体修复2例,人工骨修复1例.随访9个月～5年,效果满意.结论 在充分做好术前设计和严防并发症的情况下,加以现代麻醉和颅颌面外科技术的保证,利用现代计算机模拟手术,可安全进行病变骨广泛切除、重建颅颌面骨骼支架,远期疗效尚需进一步观察.