醫(yī)學(xué)交流課件：海綿竇海綿狀血管瘤治療策略的演變

1、海綿竇海綿狀血管海綿竇海綿狀血管瘤治瘤治療策略療策略 的演變的演變 The evolution of treatment strategies for cavernous hemangioma in cavernous sinus 海綿竇海綿狀血管瘤的特點(diǎn)海綿竇海綿狀血管瘤的特點(diǎn) 罕見(jiàn)，海綿竇腫瘤的罕見(jiàn)，海綿竇腫瘤的2-3%2-3% 多數(shù)為亞洲女性多數(shù)為亞洲女性 起病隱匿，起病隱匿，進(jìn)展緩慢進(jìn)展緩慢，平，平 均均病程病程3 3年年 血血供供豐富豐富 Extremely rare, 2% to 3% of CS tumors Predominance in Middle aged women I

2、nsidious onset, slow progression, mean disease duration of 3 years propensity for profuse bleeding Pathology: true vascular neoplasms Type A: sponginess-shaped with an intact pseudocapsule Type B: mulberry-shaped w/wt the pseudocapsule Morphological subtypes(by Zhou et al from Huashan Hospital) Chen

3、 L, Zhou LF, et al. Surg Neurol, 2003; 60:31-8. Chen L, Zhou LF, et al. Surg Neurol, 2003; 60:31-8. A AB B Chuang CC, et al. J of Clinical Neuroscience, 2006; 13:672-5. 病理病理 無(wú)膠質(zhì)成分 與腦實(shí)質(zhì)內(nèi)的海綿狀血管瘤相似 由結(jié)締組織分隔的薄壁血管組成 血管腔內(nèi)充滿(mǎn)漿果樣物 血管壁缺乏彈力層和肌層 癥狀及體征癥狀及體征- -Symptoms 壓迫癥狀壓迫癥狀 頭暈，頭痛，眼球突出，肢體乏力， 癲癇 顱神經(jīng)功能障礙顱神經(jīng)功能障礙 面

4、部麻木，眼瞼下垂，視力減退， 復(fù)視、眼球活動(dòng)障礙 內(nèi)分泌紊亂內(nèi)分泌紊亂 月經(jīng)紊亂,泌乳素升高 Direct tumor compression Dizziness, headache, eye protrusion, limb weakness, epilepsy Neurological deficits Facial numbness, ptosis, vision loss, diplopia, eye movement disorder Endocrine disorders Menstrual disorders, elevated prolactin 影像學(xué)表現(xiàn)影像學(xué)表現(xiàn) 磁共振特

5、征磁共振特征 T2WI及T2-FLAIR均勻超高信號(hào) 顯著均勻強(qiáng)化 腫瘤啞鈴樣 病變累及鞍區(qū) 血管造影血管造影 動(dòng)脈期腫瘤無(wú)染色 Kangmin He, et al Eur Neurol 2014;72:116124 MRI characteristics T2 weighted ultrahigh uniform signal Significant uniform enhancement dumbbell-like appearance infiltration of the sellar region DSA no tumor staining on artery phase A：MRI

6、 T1 等信號(hào)等信號(hào) B：MRI T2 均勻極高信號(hào)均勻極高信號(hào) C：Flair 均勻高信號(hào)。均勻高信號(hào)。 D： 增強(qiáng)增強(qiáng)MRI 漸進(jìn)性強(qiáng)化漸進(jìn)性強(qiáng)化 E 和和F： DSA 動(dòng)脈期無(wú)染色動(dòng)脈期無(wú)染色 影像學(xué)表現(xiàn)影像學(xué)表現(xiàn) 鑒別診斷鑒別診斷 腦膜瘤腦膜瘤 血管平滑肌瘤血管平滑肌瘤 神經(jīng)鞘瘤神經(jīng)鞘瘤 垂體瘤垂體瘤 Differential diagnosis Meningioma Vascular leiomyoma Neurilemmoma Pituitary adenoma 腦膜瘤腦膜瘤 神經(jīng)鞘瘤神經(jīng)鞘瘤 皮樣囊腫皮樣囊腫 動(dòng)脈瘤動(dòng)脈瘤 治療方式的選擇治療方式的選擇 Optimum treat

7、ment strategy-controversial Microsurgical resection(35papers，145cases） Gama knife （22 papers） Fractionated radiation therapy （4 papers） Cyberknife（3 papers） 最佳治療模式尚未確立最佳治療模式尚未確立 顯微外科手術(shù)(35篇，145例） 伽瑪?shù)斗派渫饪疲?2篇） 常規(guī)放療（4篇） 射波刀治療（3篇） 手術(shù)手術(shù) 全切可治愈但極具挑戰(zhàn)全切可治愈但極具挑戰(zhàn) 術(shù)中出血 周?chē)馄式Y(jié)構(gòu)復(fù)雜 全切率低全切率低 僅64% 術(shù)后并發(fā)癥發(fā)生率高術(shù)后并發(fā)癥發(fā)生率高

8、短期 71%. 晚期 18% Curative potentially but extremely challenging Severe intraoperative hemorrhage Complicated neurovascular structures Low total removal rate Only 64% High Postoperative cranialneuropathies rate Postoperative complications 71% LT morbidity 18% SurgerySurgery Fraser JF , et al. Skull bas

9、e. 2008 Sep;18(5):309-15. 手術(shù)治療手術(shù)治療 YI-Heng Yin, et al. Journal of clinical neuroscience.2013,20,128-133 1150ml19 /22 (86.4%)8 /22 (36.4%)Total 18(81.8%) subtotal 3(13.6%) 伽瑪?shù)顿が數(shù)?Gamma Knife In 1999 Iwai A 40-year-old female Treatment Partial removal with severe bleeding Gamma knife marginal dose 12

10、Gy ，50% isodose. Results Dramatically decreased tumor size Unchanged cranial nerve deficits 1999年 Iwai 40歲女性 治療治療 部分切除導(dǎo)致嚴(yán)重出血 伽瑪?shù)?12Gy，50% 等劑量線 效果效果 腫瘤明顯縮小 顱神經(jīng)癥狀無(wú)改善 立體定向放射外科立體定向放射外科 Stereotactic radiosurgeryStereotactic radiosurgery Safe and effective Remarkable tumor shrinkage Symptom improvement Mi

11、nimal complications Gamma knife Single-fraction Small- or medium sized CSH Cyberknife Multi-fractions Large or giant CSH 安全有效安全有效 腫瘤體積明顯縮小 神經(jīng)癥狀改善 治療相關(guān)并發(fā)癥極少 伽瑪?shù)顿が數(shù)?單次 小或中等大小腫瘤 射波刀射波刀 分次 大型或巨大型腫瘤 51 75 71% 36/51 73.3% 55/75 37 50 Resolved 22% Improved 46% Stable 32% Resolved 48% Improved 13% Stable 36

12、% New 15% YI-Heng Yin, et al. Journal of clinical neuroscience.2013,20,128-133 放射外科放射外科 vsvs 手術(shù)手術(shù) Radiosurgery-Meta analysis 20122012年年 1010篇相關(guān)報(bào)道篇相關(guān)報(bào)道 5959例例 患者資料患者資料 女37/59 (62.7%) 男22/59 (37.3%) 49.2 歲(1478) 腫瘤大小9.6 cm3 （ 1.551.4 cm3 ） 隨訪 49.2 月(6156月) Wangxin, et al. Journal of neuro-oncology.201

13、2,107,239-245 2012 10 papers 59 patients clinical materials 37 (62.7%) female and 22 (37.3%) male 49.2 years (range 14-78 years) mean tumor size 9.6 cm3 （1.5-51.4cm3） Mean follow-up: 49.2 months Radiosurgery-Meta analysis 腫瘤退縮 明顯退縮 67.8% 部分退縮 25.4% 不變 6.8% 癥狀改善 無(wú)癥狀 13例 完全緩解 7例 部分改善 28例 維持原狀 11例 無(wú)治療相

14、關(guān)并發(fā)癥 伽瑪?shù)吨委熤行⌒虲SH 安全有效 Wangxin, et al. Journal of neuro-oncology.2012,107,239-245 Tumor shrinkage remarkable 67.8% partial 25.4% no change 6.8% Symptom changes no cranial nerve impairments 13 patients complete resolution 7 patients improvement 28 patients unchanged 11patients No treatment-related com

15、plications GK is safe and effective for small or medium sized CSH 華山醫(yī)院射波刀治療中心華山醫(yī)院射波刀治療中心 顱底顱底組織組織放放射射劑量劑量耐受性耐受性 動(dòng)眼神經(jīng)（III）- 16Gy？ 滑車(chē)神經(jīng)（IV） - 18Gy 外展神經(jīng)（VI） - 18Gy 眼支（V1） - 16Gy？ 上頜支（V2） - 16Gy？ 視交叉（II） - 8-10Gy 垂體- 15-160Gy 頸內(nèi)動(dòng)脈- 20Gy？ 面神經(jīng)- 12-16Gy 后組顱神經(jīng)- 14Gy 腦干- 12-20Gy 耳蝸- 4.5Gy 垂體漏斗部遠(yuǎn)端- 17Gy 顱神經(jīng)對(duì)射

16、線的耐受性研究：特殊感覺(jué)神經(jīng)軀體感覺(jué)神經(jīng)80% 癥狀改善 33例改善 2例加重 未產(chǎn)生與治療相關(guān)的新癥狀 Xuqun Tang, et al. Acta Neurochir.2015,157,961-969. GK-small or medium sized CSH 53 patients（2007-2012） Female 38patients，Male15patients 52 years tumor volume13.2 cm3（141cm3） follow up 34m(273m). Tumor shrinkage 60.2%（16.5-89.2%） 80% in 55% patien

17、ts Symptom changes improvement 28 patients aggravation 2 patients No treatment-related complications blurred visiondizziness. fatiguedizziness 鞍內(nèi)為主型鞍內(nèi)為主型 diplopia diplopia, blurred vision 鞍旁為主型鞍旁為主型 headache 混合型混合型 患者女性，61歲 ，頭暈發(fā)現(xiàn) CHCS 伽瑪?shù)吨委熀箢^ 暈好轉(zhuǎn)，21個(gè)月 后復(fù)查MRI示腫 瘤縮小。 GKS21月后 患者女性，47歲 ，復(fù)視及視物模 糊發(fā)現(xiàn)CHCS 伽

18、瑪?shù)吨委熀? 月癥狀消失，45 個(gè)月后復(fù)查MRI 示腫瘤縮小 GKS45月后 患者女性，40歲 ，復(fù)視和外展麻 痹發(fā)現(xiàn)CHCS 伽瑪?shù)吨委熀? 月復(fù)視消失，46 個(gè)月后復(fù)查MRI 示腫瘤縮小 GKS46月后 大型大型CSHCSH II期前瞻性研究 腫瘤體積 20 cm3，21Gy/3Fx 14例 腫瘤體積50.2 cm3（22.696.3cm3） 隨訪15月(636月) 腫瘤平均退縮77% （44-99%） 6例治療前有顱神經(jīng)癥狀 1例緩解 5例改善 未產(chǎn)生與治療相關(guān)的新癥狀 Wangxin, et al. Internation journal of radiation oncology b

19、iology physics .2012,157,961-969. 我院經(jīng)驗(yàn)我院經(jīng)驗(yàn) Our experience Large CSH Phase II study Tumor volume 20cm3，21Gy/3Fx 14patients Tumor volume 50.2 cm3（22.696.3cm3） Follow up 15m(636m) Tumor shrinkage 77% （44-99%） 6 patients with cranial nerve impairments complete resolution 1 patients improvement 5 patient

20、s No treatment-related complications 巨大型巨大型CSHCSH II期前瞻性研究 腫瘤體積 40cm3，18-22Gy/3-4Fx 31例 腫瘤體積64.4cm3 (40.9-145.3cm3) 隨訪30月(678月) 腫瘤平均退縮88.1% (62.3%-99.4%) 30例治療前有顱神經(jīng)癥狀 19例完全緩解 11例改善 副作用 輕度頭痛 10例 嘔吐 5例 癲癇 1例 Wangxin, et al. Journal of neurosurgery. Accepted 我院經(jīng)驗(yàn)我院經(jīng)驗(yàn) Our experience Giant CSH Phase II s

21、tudy Tumor volume40cm3，18-22Gy/3-4Fx 31patients Tumor volume 64.4cm3（40.9145.3cm3） Follow up 30m(678m) Tumor shrinkage 88.1% (62.3%-99.4%) 30 patients with cranial nerve impairments complete resolution 19 patients improvement 11 patients Treatment-related complications mild headache 10 patients vomi

22、ting 5 patients seizure 1 patient Summary of dosing schedule，biologically equivalent doses and single-dose equivalents Dosing scheduleBED Total dose(Gy)No. of fractions/=3S i n g l e - d o s e equivalent (Gy) No. of patients% Prescribed dose of tumor 18445.0010.22516.1 20453.3311.2326.5 20.8456.911.

23、6526.5 19.5361.7512.2013.2 22462.3312.251135.5 20.4366.6012.7113.2 21370.0013.00929.0 Dmax of Optic nerve (mean) 13.70334.568.801135.6 15.22434.528.792064.5 Dmax of optic chiasm (mean) 15.86343.8110.061135.6 16.34438.599.362064.5 Dmax of brain stem (mean) 19.08359.5311.951135.6 20.10453.7711.292064.

24、5 照射劑量照射劑量 DoseDose 腫瘤控制腫瘤控制 Tumor response Symptom changes Summary of post-treatment changes in symptoms Symptoms pretreatment Cranial nerve deficitsHeada che DizzyNauseaSuppress ed menstrua tion Walking instabilit y Hand numbn ess No. deficits Total (%) no. of patients1410244992231131 Recovery6102

25、33782131019 (61.3%) Improved80011210100011 (35.5%) Average time to recovery (months) 622638621033 大型大型CSHCSH三年以上長(zhǎng)期結(jié)果三年以上長(zhǎng)期結(jié)果 40例 腫瘤體積 48.6 cm3 (11.3-140.1 cm3) 隨訪53月(3690月) 腫瘤平均退縮85% (41%-100%) 30例有顱神經(jīng)癥狀 19例完全緩解 11例改善 副作用 輕度頭痛 10例 嘔吐 5例 癲癇 1例 我院經(jīng)驗(yàn)我院經(jīng)驗(yàn) Our experience Long-term outcomes of large CSH 4

26、0 cases Tumor volume 48.6 cm3 (11.3-140.1 cm3) Follow up 53 months (3690 months) Tumor shrinkage 85% (41%-100%) 30 patients with cranial nerve impairments complete resolution 19 patients improvement 11 patients Treatment-related complications mild headache 10 patients vomiting 5 patients seizure 1 p

27、atient 大型大型CSHCSH三年以上長(zhǎng)期結(jié)果三年以上長(zhǎng)期結(jié)果 Table 2 Clinical response after multi-fraction SRS for CSHs Pre-treatment symptoms and signsResponse No: (%) No.Complete resolved: NoImprovement: No. Visual impairment 146(42%)8(58%) Diplopia65(83.3%) Facial numbness77(100%) Slight headache1211(91.6) Dizziness77(100

28、%) Ptosis119(81.8)1(9%) Amennorrhea33(100%) Exophthalmos11(100%) Decreasing hearing1no Numbness of contralateral limbs 11(100%) Patients 2008.1-2014.12 102例 14例手術(shù)后殘留或復(fù)發(fā) 男26例； 女76例 年齡： 51（ 26-80 ） 腫瘤最大直徑：40mm（ 23-75） 腫瘤體積： 39.1cm3 （ 2.9-145.1） 患者資料患者資料 2008.1-2014.12 102patients Residual or recurrenc

29、e after surgery in 14 cases Male 25.5% ； Female74.5% Age： 51 （ 26-80 ） Tumor diameter：40mm（ 23-75） Tumor volume： 39.1cm3 （ 2.9-145.1） 劑量方案劑量方案 Dose schedule Tumor volumeDose schedule 513.0Gy /1fx 5V 1017.8-19.0Gy/2fx 10 40 18.0-22Gy/4fx The equivalent of Gamma knife 10-13Gy/1fx 3 18 56 25 0 10 20 30

30、 40 50 60 1234 治療 次數(shù) 例數(shù) dose curvingdose curving 華山醫(yī)院射波刀治療中心華山醫(yī)院射波刀治療中心 dose curvingdose curving 7m after CK18m after CK45m after CK Tumor volume 13.5cm3 21Gy/3Fx Before CKBefore CK 12m after CK 12m after CK Tumor Tumor shringkageshringkage 95 95Treatment planTreatment plan 4 years after CK4 years a

31、fter CKMRI FlairMRI T2 Before CK 7m after CK Tumor shrinkage 95% 2y after CK Tumor shrinkage 99% 1 y 1 y 2 y2 y Tumor diameter 6.3cm Tumor volumn 80cm3 Before CK 2y after CK4y after CK 7m after CKTumor diameter 7.5cm Tumor volume 140cm3 7m after CK tumor shrinkage 75%3y after CK tumor shrinkage 90%

32、腫瘤生長(zhǎng)速度 1998 Tumor volume：20cm3 2003 Tumor enlarged significantly 2010 2010 Tumor volume：76cm3 2011/01 CK 6m after CK，Tumor shrinkage 80% ,vision improved 42m after CK，Tumor shrinkage 95% 2002 biopsy decreased visual acuity and ptosis 2011 Tumor enlarged，Tumor diameter 7.5cm ,Tumor volume 140cm3 Treatmen