好发于青少年的高度恶性肿瘤

2017-11-20 牛晓辉 郝林 骨肿瘤

14岁男性患者确诊骨肉瘤,保肢手术的应用需考虑哪些问题?人工全股骨假体置换适应证是什么,又有何优缺点?

14岁男性患者确诊骨肉瘤,保肢手术的应用需考虑哪些问题?人工全股骨假体置换适应证是什么,又有何优缺点?

(1)病例介绍

:患者,男,14岁,主因左大腿肿胀疼痛2个月收入院。患者于2个月前运动后出现左大腿疼痛,活动后加重,近日逐渐肿胀,疼痛减轻。就诊当地医院予以对症治疗效果欠佳,近两周来,大腿肿胀疼痛加重,遂转诊入我院。

入院查体:一般情况可,左大腿弥漫肿胀,皮温较高,可触及包块广泛,边界不清,压痛,左膝关节、髋关节活动无受限。髌上10cm 周径测量显示左侧为42cm,右侧为40cm。

影像学检查:X 线片示左股骨下段正侧位,病变位于左股骨干溶骨破坏,髓腔内基质不均,边界不清,骨皮质破坏,可见层状骨膜反应,少量软组织包块形成;全身骨扫描示病变位于左股骨干,范围广泛,其他骨骼未见核素异常浓集(图37‐1)。

常规化验结果基本正常。AKP 359IU/L。

入院后行穿刺活检,病理回报为成骨肉瘤。新辅助化疗,行异环磷酰胺15g/m 2化疗、氨甲蝶呤8g/m 2化疗各两个疗程,期间患者局部疼痛及肿胀均有所减轻,周径差由化疗前的2cm减小为1cm。复查X 线,增强CT,MRI 及全身骨扫描,显示病变的肿胀减轻,软组织包块边缘出现骨化,边缘较化疗前清楚。化验显示AKP下降为89IU/L。


图37‐1 男性,14岁,X 线片显示左股骨下段正侧位,病变位于左股骨干溶骨破坏,髓腔内基质不均,边界不清,皮质骨破坏,可见层状骨膜反应,少量软组织包块形成,ECT 示 病变位于左股骨干,范围广泛,其他骨骼未见核素异常浓集

完善术前准备后,在气管插管全身麻醉下行左股骨肿瘤切除人工全股骨关节置换术。

1)患者右侧卧位,经患肢大腿外侧入路,屈髋90°,自大粗隆上10cm,经髋关节,沿股骨外侧下至胫骨结节髌韧带止点外侧。

2)切开皮肤、皮下组织及深筋膜,原穿刺活检通道梭形切除至深部组织,以缝合线将深部组织和切除的活检通道皮缘缝合,避免分离,髋关节部分切开阔筋膜张肌,向下切开阔筋膜至远端。

3)沿股外侧肌后缘分离,在保证恰当的外科边界的情况下,尽力保留股外侧肌和臀中肌的连续性或术后重建软组织的连续性,股中间肌随肿瘤切除,股骨大粗隆切断附着的外旋肌群,至关节囊,髂腰肌距止点2cm切断,股内侧肌群距肿瘤边缘1~2cm切断,股内侧肌和股直肌在肿瘤外1cm,锐性分离,保留髌骨和髌韧带的连续性。

4)膝关节侧,于胫骨上端切开关节囊,在胫骨侧切断半月板、交叉韧带止点,屈膝切断后关节囊,保护分离股动、静脉及坐骨神经,距腓肠肌内外侧头止点1~2cm切断,切开关节囊,股骨头脱位,肿瘤骨完整切除。采用国产人工全股骨假体置换,髋关节为双动半髋关节,膝关节为限制性铰链式。

5)反复冲洗、止血后,关节近端复位,远端关节装配同股骨膝关节置换。髋关节残留关节囊紧缩缝合,臀中肌与股外侧肌缝合于人工关节大粗隆的部位,股外侧肌前缘与股直肌缝合覆盖关节假体,臀大肌止点缝合于股骨假体上段。

6)留置3~4 根引流管于髋关节、膝关节和股骨假体两侧。

7)依次缝合阔筋膜、皮下组织和皮肤。

术毕常规应用抗生素10~14天,24小时引流小于25ml 后拔出引流管。

术后两周伤口一期愈合后拆线。疼痛消失后练习股四头肌等长收缩,3周后练习髋关节屈曲和膝关节屈伸活动,至6~8周后可拄拐杖,部分负重下地活动,并逐渐增加负重(图37‐2)。

术后继续使用氨甲蝶呤、异环磷酰胺、多柔比星及顺铂进行定期化疗,间期为2~3周。

术后功能锻炼:术后疼痛消失练习大腿肌肉的等长收缩,3周后练习屈髋、屈膝的关节活动,拄双拐部分负重练习行走,6~8周后拄单拐负重行走,练习恢复正常步态,视步态正常与否,弃拐行走。

术后复查随访:术后复查随访均由本院高年资医师负责,系统规范记录于科室资料库,术后化疗期间,每月摄患肢及双肺X 线片,半年行肺CT 和全身骨扫描检查;化疗结束后每3个月摄X 线片检查,半年行肺CT 和全身骨扫描检查,直至术后5年,每年复查1次。复查同时,按照MSTS系统检查肢体功能恢复情况,给予评分记录。

(2)专家分析

1)人工全股骨假体置换适应证:人工全股骨假体置换术最早于1965年Joseph Buch man 报道用于股骨佩吉特病的治疗,并且1990年Present 和Kuschner 报道了该患者术后35年的功能恢复良好,以后相继有多位学者报道。人工全股骨置换术对于股骨广泛受侵、或股骨病灶的另一端存在跳跃病灶的骨肉瘤患者,避免了髋关节离断或半骨盆截肢。随着外科技术的进步,对骨肿瘤外科分期的认识深入,肿瘤切除的外科边界达到广泛切除,可以有效降低复发率。积水潭医院曾报道17例骨肉瘤患者,复发3例,占17.6%,与同期其他部位化疗保肢组的复发率16.7%,差异不明显(P >0.05)。12例Ⅱb 期患者,4例肺转移(占33%)死亡,8例无瘤存活(占67%),低于同期其他部位化疗组的无瘤存活率77.6%,但差异不明显(P >0.05),17例患者瘤骨病灶范围明显大于其他部位的病灶范围,可能是影响患者预后的一个因素。患肢术后功能恢复方面,15例术后恢复行走,占88%,其中Ⅱb 期11例,Ⅲ期4例。依Enneking骨骼肌肉系统肿瘤术后功能评估系统,肢体功能恢复率40%~93%,平均74%,低于同期膝关节部位保肢组功能恢复率86%,与患者接受髋、膝两个关节的切除与重建有关,并随瘤骨切除较多软组织肌群,造成了关节稳定性差和关节运动所需动力不足。


图37‐2 X 线正位片显示左股骨瘤骨截除,人工全股骨置换术后

资料显示对于Ⅲb 期患者,人工全股骨假体置换术可以有效保障患者的生存质量的提高,在有限的生存期内,相对于异体骨干移植、自体瘤骨灭活再植术后等待较长时间的骨愈合,人工全股骨假体置换术可快速恢复患肢负重功能。但手术创面广泛,术中出血较多,术前需反复检查患者心肺功能,视其耐受程度决定是否手术。

2)术中、术后并发症:全股骨切除涉及多组肌群的切除,造成创面广泛渗血,术中需要采用各种有效的止血技术,减少渗血。

骨肉瘤好发于青少年,保肢手术的应用需考虑患肢与健肢不等长的问题,而且术前准确测定切除股骨的长度困难,2例患者的假体长度设计长于切除的股骨,并且为定制的不可调节长度的假体致使术中复位困难,术后腓总神经麻痹,为减轻神经张力,术后患肢放于屈膝位,3个月后开始恢复,但1例患者仍尚存屈膝25°畸形。作者认为术前采用CT 测量准确长度对于假体设计至关重要,或者改进定制假体为可调试组配假体。

术后髋关节脱位,与患者过早患肢负重有关,由于随瘤骨切除大量附着其上的肌肉组织,髋关节周围肌肉张力明显减弱,致使稳定性差,需卧床3~6周后,软组织愈合后,练习屈髋、屈膝的关节活动,视肌力恢复状况,拄双拐练习,部分负重行走,8~12周后拄单拐负重行走。

人工全股骨置换术后,假体松动相关的临床症状少有报道,可能与患者置换髋、膝两个关节后负重行走减少有关,需长期随访观察。

3)人工假体设计有待发展:目前临床需定制假体设计个体化,多样化。本组1例患者采用了保留股骨上端与假体复合的人工全股骨置换,术后髋关节的稳定性及髋关节功能的恢复优于其他患者。

(3)诊治要点

:该患者的临床表现、影像学表现及病理均支持典型骨肉瘤的诊断。患者为青少年男性,病程1个月。以小腿近端疼痛起病,影像学检查为胫骨近端干骺端的成骨性病变,伴有骨膜反应和软组织包块,病变范围长,这些表现均支持一个好发于长骨干骺端的恶性肿瘤的诊断,与典型骨肉瘤的表现相符,结合活检病理支持骨肉瘤,诊断明确。


图37‐3 术后十年患肢功能状况

(4)治疗原则

:典型骨肉瘤为高度恶性肿瘤,结合其影像学表现:存在骨外软组织包块,未见远处转移灶。Enneking分期为Ⅱb。治疗过程为术前新辅助化疗,外科手术,边界应至少达广泛性切除,术后继续进行化疗,并定期随访局部X 线,胸部CT,及全身骨扫描。因病灶范围广泛,在恰当的外科边界要求下截骨,目前的重建手段有限,人工全股骨置换是一种有效保肢的手段,并能获得良好的功能恢复。

(5)随诊结果

:患者术后未见明显并发症。目前患者术后10年,未发现局部复发及远隔转移。患者行走步态基本正常。双下肢长度相差2cm,左膝关节主动活动度(伸直至屈曲):15~90°,髋关节主动活动度(伸直至屈曲):0~90°(图37‐3)。X 线显示假体髋关节磨损髋臼(图37‐4),已置换髋臼(图37‐5);胫骨侧未见明显松动。患者为无瘤生存状态。


图37‐4 术后十年复查,患肢较对侧健肢短2cm,脊柱侧弯,患侧髋臼内壁变薄、穿透。未见局部复发


图37‐5 患侧髋臼置换术后X 线正位片表现

(6)经验教训

1)全股骨人工关节置换术适于股骨广泛受侵的恶性骨肿瘤患者,能有效恢复肢体功能。

2)对于Ⅲ期患者,是一种姑息性保肢,在有限的生存期内,能有效的改善生活质量。

3)人工假体的设计有待改进,需尽可能保留一个关节及肌肉附着点,从而提高肢体功能恢复率,避免并发症。德国某公司定制假体,股骨上段自大转子尖至小转子下5cm 保留,与假体复合,从而可保留髂腰肌、臀中肌、股外侧肌和髋关节内外旋肌群在大小转子的原始骨性附着,髋关节为双动半髋关节,膝关节为可旋转式关节。

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    2017-12-25 七九九八

    继续努力.加油加油

    0

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    2017-12-24 七九九八

    继续努力.加油加油!

    0

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    2017-12-18 七九九八

    继续努力

    0

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    2017-11-20 三生有幸9135

    学习一下谢谢分享

    0

  5. [GetPortalCommentsPageByObjectIdResponse(id=272469, encodeId=4ff42e246959, content=继续努力.加油加油, beContent=null, objectType=article, channel=null, level=null, likeNumber=72, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/ajNVdqHZLLCcmyvDOwDu2ZpuYyovmsD12IBHmUCwr5bZqNic2p50ogCxUAIj4YqJ3PsGwoXOhJPQAvicmu6RqOww/0, createdBy=a0bf1958339, createdName=七九九八, createdTime=Mon Dec 25 20:22:39 CST 2017, time=2017-12-25, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=272183, encodeId=73092e218303, content=继续努力.加油加油!, beContent=null, objectType=article, channel=null, level=null, likeNumber=66, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/ajNVdqHZLLCcmyvDOwDu2ZpuYyovmsD12IBHmUCwr5bZqNic2p50ogCxUAIj4YqJ3PsGwoXOhJPQAvicmu6RqOww/0, createdBy=a0bf1958339, createdName=七九九八, createdTime=Sun Dec 24 17:39:51 CST 2017, time=2017-12-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=270371, encodeId=47e02e03713a, content=继续努力, beContent=null, objectType=article, channel=null, level=null, likeNumber=66, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/ajNVdqHZLLCcmyvDOwDu2ZpuYyovmsD12IBHmUCwr5bZqNic2p50ogCxUAIj4YqJ3PsGwoXOhJPQAvicmu6RqOww/0, createdBy=a0bf1958339, createdName=七九九八, createdTime=Mon Dec 18 18:39:18 CST 2017, time=2017-12-18, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=262896, encodeId=789326289678, content=学习一下谢谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/jW482SpianMayicTRbRZ5RzaiabIFicf63hKvVMjL6aJlx4yX4UcXmzX9DyibCRcicq0QtCRWMaQKh8MYjE1D3EKUBopgiaGWYuqbeR/0, createdBy=0c2b1999806, createdName=三生有幸9135, createdTime=Mon Nov 20 18:44:19 CST 2017, time=2017-11-20, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=262887, encodeId=23cf26288eef, content=学习了.谢谢., beContent=null, objectType=article, channel=null, level=null, likeNumber=66, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/xkvRjYnJzzRJ2R0y7VhicibvkjPWHjIMmu4ZpKnE9cWAu4lVicS3uDUCfF4M4JWvrkrYfpb2KehPV0Ee29tPqyPEebBUibiaKhnvD/0, createdBy=f8412001184, createdName=惠映实验室, createdTime=Mon Nov 20 18:34:52 CST 2017, time=2017-11-20, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=262885, encodeId=3d442628857d, content=不是并不代表傻逼吧删吧, beContent=null, objectType=article, channel=null, level=null, likeNumber=35, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/PiajxSqBRaEKXpC8j9TaoCpG4yIB3skibUZ0ricmVQ6KKIVpqYQxINrmlibsPZSia7GOUupzuINf8UITMib8awsjjkPw/0, createdBy=abbf1713451, createdName=zt123, createdTime=Mon Nov 20 18:27:12 CST 2017, time=2017-11-20, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=262884, encodeId=cad626288441, content=^_^^_^^_^^_^, beContent=null, objectType=article, channel=null, level=null, likeNumber=24, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/vi_32/Swp2ehfHpiciczeTntUyQOlIGMzQ705ic67sdMTVMbwTv7KbmlUvxrGvRSvQZjPkz7ib5tEDfIaORgczPtmHDpwojg/0, createdBy=c54b2163125, createdName=wqkm, createdTime=Mon Nov 20 18:20:10 CST 2017, time=2017-11-20, status=1, ipAttribution=)]
    2017-11-20 惠映实验室

    学习了.谢谢.

    0

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    2017-11-20 zt123

    不是并不代表傻逼吧删吧

    0

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    2017-11-20 wqkm

    ^_^^_^^_^^_^

    0

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