髋部非典型骨膜软骨瘤1例

2018-10-23 杨勇志 刘凡 张德宝 中国骨伤

患者,男,22岁,入院前7个月无明显诱因出现右侧髋部疼痛,行走时加重,休息可缓解。无发热、盗汗及夜间痛,无外伤史。前往当地医院拍片未见异常,未予处理。近40d来症状加重,并伴右侧髋部活动受限,遂来我院就诊。查体:右侧髋部无红肿,右侧腹股沟中点及右侧坐骨内侧压痛阳性,右髋后伸、外展受限。右髋Tomas征阳性,“4”字试验阳性。

临床资料
 
患者,男,22岁,入院前7个月无明显诱因出现右侧髋部疼痛,行走时加重,休息可缓解。无发热、盗汗及夜间痛,无外伤史。前往当地医院拍片未见异常,未予处理。近40d来症状加重,并伴右侧髋部活动受限,遂来我院就诊。查体:右侧髋部无红肿,右侧腹股沟中点及右侧坐骨内侧压痛阳性,右髋后伸、外展受限。右髋Tomas征阳性,“4”字试验阳性。

入院后检查:髋关节多排CT平扫加三维重建影像显示右侧闭孔外肌见3.5CM×3.5CM高密度肿块,CT990HU,边界较清。周围肌间隙清晰,邻近坐骨骨皮质密度略增高。余髋关节骨质未见破坏。磁共振髋关节平扫检查见:右侧闭孔外肌异常信号,大小约3.5CM×3.5CM,T1W1、T2W1呈低信号,内见点状稍高信号,病灶边缘清晰,周围见少许条状长T1长T2信号。实验室检查:血常规、CRP、ESR无异常,胆碱酯酶1266IU/L。


初步诊断:右侧髋部肿物(性质待查)。完善各项术前检查检验后,无明显手术禁忌证。全麻下行右侧髋部肿物切除术。术中可见肿物大小约3CM×3CM×4CM,包膜完整,与周围骨皮质无明显连接。肿块的外层为坚硬外壳,切开后内部包含半透明的,易碎的软骨样结构。将肿物及其包膜彻底切除,术后病理回报:右髋部不典型骨膜骨软骨瘤,明确诊断。术后随访时间1年,未见肿物复发。

讨论

骨膜软骨瘤也称骨皮质旁软骨瘤,它是源于骨膜或骨膜下结缔组织的一种罕见的透明软骨良性肿瘤,最先在1952年由Lichtenstein和Hall描述。骨膜软骨瘤占所有软骨瘤的2%以下,好发于长管状骨的干骺端,在骨膜下邻近皮质骨表面发育,生长期间逐渐从干骺端移出,最大直径一般不超过6CM。

虽然各个年龄段均可发病,但主要以30岁以下男性居多。目前骨膜软骨瘤的发病机制和遗传背景仍不清楚。Buddingh等曾对良性软骨肿瘤的细胞遗传学做过相关研究,发现其发病可能与染色体片段6q,12q的异常有关。

deSantos等描述过骨膜软骨瘤的3个主要的X线影像学特征:(1)相邻的骨皮质呈扇形或重塑为其他形状。(2)软骨基质的出现,表现为小点和钙化小环。(3)周围软组织肿胀。骨膜软骨瘤生长过程中会压迫、侵蚀周围的骨质,但因生长缓慢,骨皮质有足够时间增生修复,所以会产生较为典型的伴有反应性硬化基底的半月形皮质凹陷。但并不是所有的骨膜软骨瘤都会有典型放射学表现,仅有50%的骨膜软骨瘤伴有基底部钙化。本文中提到的病例并没有典型的半月形凹陷和环状钙化,但确实存在骨皮质改变和小的钙化区域的特征。核磁共振上,Chandramohan等在文献综述中提到,大多数T1加权的MR图像显示单一的低信号或等信号,T2加权像表现为均匀的高信号,这也符合大部分良性软骨肿瘤中心部分的成像特点。Aoki等和Geirnaerdt等也详细描述了良性软骨肿瘤的Gd-DTPA成像特点。注射Gd-DTPA后大多数良性软骨肿瘤在边缘呈环形和弧形增强,这是由于包裹肿瘤的纤维组织具有丰富的毛细血管。骨膜软骨瘤的Gd-DTPA增强程度与四周透明软骨或囊性黏液样组织的血管化程度呈正相关。

骨膜软骨瘤发病率低,临床中容易被误诊,应引起足够的重视。骨膜软骨瘤的临床症状没有特异性,可表现为可触及的肿块,肿胀和疼痛,也可以没有任何症状。最常见的生长部位是长管状骨,尤其是肱骨近端,其次是手部和脚部的小管状骨,但生长于髋部的还是非常少见的。骨膜软骨瘤极易与骨膜软骨肉瘤相混淆。其鉴别诊断主要有:(1)前者为压迫性骨缺损,而后者多为侵袭性骨破坏。(2)前者单向生长,后者放射状生长。(3)放射性核素检查:明显浓集者为肉瘤。(4)病理活检:若诊断困难时,及时、准确的活检,不失为最简捷的鉴别方法。该肿瘤还要注意与纤维皮质骨缺损、骨膜骨肉瘤、骨化性肌炎及腱鞘巨细胞瘤等相鉴别。本例患者非常容易被误诊为骨化性肌炎,但患者没有右髋部外伤史,有助于鉴别诊断。

骨膜软骨瘤最主要的治疗手段是手术切除。因其具有一定的恶变和复发倾向,所以发现后应尽早手术。复发主要是由于切除不彻底,局部整体切除病灶,合理处理肿瘤边缘,能够最大程度的减少复发。对于肿瘤边缘的处理,一些学者认为刮除肿瘤下皮质骨能够降低复发概率。若肿物已经严重累及关节、影响功能者,则应考虑人工关节置换。

原始出处:

杨勇志,刘凡,张德宝,谷贵山.髋部非典型骨膜软骨瘤1例[J].中国骨伤,2018,31(9):871~873

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    2018-11-05 衣带渐宽

    学习

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    2018-10-25 liuquan
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    2018-10-23 医者仁心5538

    学习了

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