Diagnosis and treatment of intramedullary hem angioblastom a of cervical spinal cord
X U Qiwu 徐启武,BAO Weimin 鲍伟民and PANGLi 庞 力
K eywords : cervical spinal cord ・intramedullary hemangioblastoma ・MRI ・surgery
最新日韩剧Department of Neurosurgery ,Huashan H ospital ,Fudan University ,Shanghai 200040,China (Xu QW ,Bao W M and Pang L )
C orrespondence to :Dr 1X U Qiwu ,Department of Neurosurgery ,Huashan H ospital ,Fudan University ,Shanghai 200040,China (T el :86221262489999ext 66191Fax :862212624994121Email :xuqiwuc @online 1sh 1cn )
O bje ctive To inve stigate the diagno sis and surgical technique s of intramedullary hemangiobla stoma of the
托福听力算分
cervical spinal cord 1
Met h o ds MR imaging and the methods and re sults of surgery were analyzed in 21patients 1
Re s ults The tumors were divided into three type s on MR imaging 1①Syringeal type ,where the tumor
varied in size and wa s accompanied by syringobulbia and syringomyelia ;②Cystic type ,where the tumor
pre nted a s a cyst with a small mural node ;and ③Solid type ,where the tumor wa s revealed a s a huge solid ma ss 1All tumors were totally removed and diagno sis wa s confirmed by histological study 1Po st 2operative neurological status wa s improved in 20patients and aggravated in 11
Co nclusio ns The localization and the nature diagno sis of the tumor can be made by cervical MR imaging 1
Operative methods vary with tumor type s 1I t is the mo st important that the tumor is discted along the right interface and removed after deva scularization 1
Chin Med J 2002;115(7):101021013
C entral
cruel什么意思nerv ous system hemangioblastoma is often en in
the posterior cranial fossa ,while it is rare in the spinal canal 11 There were few m onographs published about intramedullary hemangioblastoma in the cervical spinal cord (IHCSC )1We surgically treated 21concutive patients with IHCSC from August 1989to December 20001All patients underwent total tum or rem oval with satis factory results 1
pok
METH ODS
Sixteen men and five w omen participated in this study ,age from 16to 52years ,with an average of 3214years 1The period between the ont of sym ptoms and admission ranged from 20days to 82m onths ,with an average of 3619m onths 1The main clinical prentations were paresthesia in 17patients ,hypoaesthesia in 16,spontaneous pain in 10,weakness in 16,my oatrophy in 13,fasciculation in 9,sphincter dys function in 6,positive pathological sign in 13,cranial nerve deficit in 5,ataxia in 2,dyspnea in 3and headache in 11Erythrocytes ranged from 317×1012/L to 516×1012/L 1Haematoglobin ranged from 110g/L to 168g/L 1T w o cas had family histories 1One ca was com plicated by multiple intra 2cranial hemangioblastomas and tw o cas prented with multiple intramedullary hemangioblastomas of the spinal cord 1One ca su ffered from tonsil cancer 8m onths after rem oval of the
hemangioblastoma 1T w o cas underwent myelography and post 2myelography spinal CT scan 1One had a spinal CT and six had spinal digital subtraction angiography 1All cas underwent spinal MRI 1Surgical treatment was performed in all patients 1
RESU LTS
In all patients ,MRI identified the localization and nature of the tum or 1There were 26intramedullary tum ors (one patient had 2tum ors ,another had 5),including 8in the medullo 2cervical region ,16in the cervical spinal cord ,2in the cervico 2thoracic region 1In 21cas ,the tum or prented as a syringeal type (Fig 11A )in 18,cystic (Fig 12A )in 2and s olid (Fig 13A )in 11Characteristic flow v oid in the tum or region was en in 14patients 1The ass ociated lesions included condary syring obulbia in 14patients and syring omyelia in 181S pinal digital subtraction angiography (DS A )showed the tum or brush ,as well as the feeding arteries and the draining veins of the tum or (Fig 14)1
Fig 111(A)Preoperative contrast MR imaging
showing syringeal type hemangioblastoma with syring obulbia and syring omyelia ;(B)P ostoperative contrast MR imaging showing total tum or rem oval 1Fig 121(A)Preoperative contrast MR imaging showing cystic type hemangioblastoma ,which prents a cyst with a mural nodule ;(B)P ostoperative contrast MR imaging showing total tum or rem
oval 1
T otal tum or rem oval was achieved in 21patients 1All rected tum ors were histologically con firmed to be hemangioblastomas 1The diameter of the tum ors ranged from 012-810cm ,with an average of 214
cm 1Patients were hospitalized for 12-38days after surgery ,with an average of 1710days 1Three patients prented tem porary limb hyperpathia 4-14days after surgery 1On discharge ,neurological status was im proved in 20patients and deteriorated in one 1Fifteen patients were followed up for 7-134m onths ,with an average of 8219m onths 1F ourteen patients showed im provement ,of whom 13went back to w ork 1One patient deteriorated com pared to his pre 2operative status ,but he was able to do s ome light w ork 1P ost 2operative MRI was performed in 14patients and all showed the tum ors totally rem oved ,and the condary syring obulbia and syring omyelia diminished or disappeared (Figs 11B ,2B and 3B )1
DISCUSSION
According to early reports ,hemangioblastoma occurs in frequently at the intra 2spinal canal and rarely at the
Fig 131(A)Preoperative MR T 12weighted imaging showing s olid type hemangioblastoma ,which prents a giant s olid mass with low signal flow v oids ;(B)P ostoperative contrast MR imaging showing total tum or rem oval 1
Fig 141S pinal DS A showing the tum or brush ,and the feeding arteries and the draining veins of the tum or 1(A)Lateral projection ;(B)Anteroposterior projection 1
intramedullary cervical spinal cord 11 This is becau the IHCSC is difficult to be diagnod preoperatively 1Tum ors have been found much m ore often with advances in MR imaging 1Signs of the tum ors on MR imaging have been reported 12,3 MR imaging can identify the localization and nature of the tum or 1According to the findings of MR imaging ,tum ors can be divided into three types 1①Syringeal type :the tum or varies in size and is ass ociated with syring obulbia and syring omyelia 1There were 18patients of such type in our study ;②Cystic type :the tum or showed a cyst with a small mural nodule 1There were tw o patients of this type in our study 1③S olid type :the tum 如梦令翻译
or revealed a giant s olid mass 1There was one patient of this type in our study 1
S pinal DS A is als o a great help to the diagnosis of the tum or becau it can show the tum or brush 1In addition ,spinal DS A helps to establish the surgical plan becau it can show the feeding arteries and draining veins of the tum or ,as well
as their location and direction1
Differential diagnosis
Becau it often prents with condary syring obulbia and syring omyelia,as well as with an untypical clinical prentation,the tum or is often con fud with other dias1Besides hydromyelia,intramedullary spinal cord ependym oma and vascular malformation of the spinal cord,3 the tum or should als o be distinguished from the following dias1
Thoracic outlet syndrome
IHSCS may be misdiagnod as thoracic outlet syndrome4 when it is at early stages and only caus pain,paresthesia, weakness and my oatrophy in the unilateral upper limb (especially with the sym ptoms being m ore vere in the ulnar side of the upper limb)1The reas on is that nerve ty
pe thoracic outlet syndrome is frequently caud by pressure on the brachiplex lower trunk(C8,T1)1The patients als o prent with numbness and pain,paresthesia,weakness, dys function in fine m ovement and my oatrophy in the ulnar side of the upper limb and hand1F our patients in this study were once diagnod as having this syndrome becau of the above2mentioned sym ptoms and tw o of them even underwent unnecessary in fra2axillary exploration lysis1The differences between the tw o dias are as follows:in thoracic outlet syndrome,the paresthesia is con fined to the unilateral upper limb1There is positive Ads on sign1Radioimaging shows no abnormality except for cervical ribs1In IHCSC,the sym ptoms and signs prent in both upper and lower limbs on both sides with the ev olution of the dia,Ads on sign is negative,and MR imaging can show typical signs of the hemangioblastoma1
Cervical spinal spondylopathy
IHSCS could be misdiagnod as cervical spinal spondylopathy when it caus pain and numbness only in the shoulder2neck and upper limbs with wave2like aggravation, and als o ass ociates with regressive signs such as the disappearance of cervical spine physical lordosis,narrowed interspinal space,or osteophyma on the posterior edge of the centrums on cervical spine X2ray1F our patients were once misdiagnod as having spondylopathy in this study1 Differences between them are as fo
llows:cervical spinal spondylopathy,except for the spinal cord type,does not generally cau spinal com pression sym ptoms with a definite level of ns ory disturbance,and only caus mild sphincter dys function1Though there could be s ome spinal com pression sym ptoms in cervical spinal spondylopathy of the spinal cord type,the com press or locates at the interspinal space and connects with the centrum,and there is no condary syring omyelia on MR imaging1In IHCSC,spinal com pression sym ptoms w ould prent with the ev olution of the dia1MR imaging can directly show the tum or and the condary syring omyelia and/or syring obulbia1Location of the tum or is not con fined to interspinal space1
T reatment
Intramedullary spinal cord hemangioblastoma is a benign tum or,which is innsitive to radiotherapy1Patients with the tum or can be cured if the tum or is totally rem oved1M ost authors claim to do total tum or rem oval for the tum or11,3,5 Intramedullary tum or in the cervical spinal cord,even inv olving medullar oblongata,is als o amenable to aggressive surgery1628 Therefore,we adv ocate doing total tum or rem oval for IHCSC1In this ries,total tum or rem oval was achieved in21(100%)and postoperative im provement was achieved in20(9512%)1The results show that IHCSC is als o amenable to aggressive surgery1
The timing of surgery for IHCSC,in our opinion,should depend on the tum or type found in MR images1F or cystic and syringeal type tum ors,surgery should be performed immediately after diagnosis becau it is relatively easy and g ood results are likely1F or s olid type tum ors,it is preferable to operate when patient has m oderate neurologic dys function1I f the patient is in g ood neurologic status,it is wi to discuss management methods with the patient or his/ her kin and to follow their decisions1This is becau surgery for the tum or is very difficult and often can cau disastrous results1It is difficult for both patients and surgeons to accept deterioration after surgery with prior g ood neurologic status1 Surgical techniques for different tum or types are as follows: In the cystic type tum or,careful disction along the nodule will result in success ful rem oval1The cystic wall should not be discted1In the syringeal type tum or that is vascular and prents with big tortuous drainage veins on the surface of and around the tum or,the dors o2lateral feeding arteries are first shown,electro2coagulated and divided1The syrinx wall is incid at the end of the tum or without the draining vein or with no dominant2draining vein1The syrinx cavity is then entered and tum or is discted along the right interface1 While it is electrocoagulated and shrunk,the tum or is parated from the surrounding spinal cord from rostral2 caudal or from caudal2rostral1When they are found(in13 patients of this ries),the ventral feeding arteries should als o be coagulated and divided1The main draining vein is divided last and the tum or is totally rem oved as en bloc as possib
le1F or large tum ors that are difficult to rem ove en bloc,the surgeon could first rem ove the part of the tum or which is com pletely devascularized and parated1Then the m ore rostral or caudal part of the tum or is discted and rem oved with the above2mentioned methods after tem porarily stopping bleeding at the cut end of the tum or1It is forbidden to electrocoagulate the main draining vein and to rem ove
piecemeal the tum or before com plete devascularization1 Otherwi,it will hinder rem oving the tum or and increa spinal cord injury1Syrinx shunt is unnecessary if the tum or is totally rem oved1There were18patients with syringeal type tum ors in this ries1On discharge,17patients who were operated on with the above2mentioned techniques were im proved1One patient g ot w orned becau the draining vein was electrocoagulated before com plete devascularization of the ventral feeding arteries1This rapidly caud enlargement and protrusion of the tum or,which led to acute spinal cord com pression and incread operative injury due to difficult tum or rem oval1Resultantly,postoperative neurological status deteriorated1Therefore,if disction of the dorsal tum or were hindered simultaneously by the dorsal root of the spinal nerve and the draining vein,surgeons should divide the former rather than electrocoagulate the latter before com plete devascularization of the ventral feeding arteries1Marks of devascularization include decread bulk
and tension of the tum or,obvious collap of the draining vein,and neither the enlargement nor the protrusion of the tum or if the main draining vein is interrupted tem porally1
In the s olid type tum or that is highly vascular and has no condary syrinx,surgery is m ore difficult becau operative space w ould be very small1Surgical techniques for the tum or are generally similar to tho for the syringeal type hemangioblastoma1It is m ore im portant to repeatedly electrocoagulate the tum or capsule using bipolar cautery with appropriate power1Thus,the tum or is shrunk and operative space is incread1Disction w ould extend to the ventral aspect of the tum or and the m ore ventral feeding arteries tend to be visible and managed1When the feeding arteries ventral to the tum or are com pletely divided and the tum or is totally parated from the surrounding spinal cord,the main draining vein is cut and total tum or rem oval is achieved1During surgery,normal saline is irrigated interruptedly in the operative area in order to wet the parched dry tum or capsule and to im prove its conductivity,which further facilitates the shrinkage and prevents its rupture1 Interoperative irrigation with normal saline can als o decrea the regional tem perature,preventing heat injury of the adjacent spinal cord1When it simultaneously inv olves the cervical spinal cord and medulla oblongata,the tum or should be rem oved with the above2mentioned techniques first in the cervical spinal cord area,and then in the medulla oblongata area1
毕业论文答辩开场白REFERE NCES
11S petzer U,Bertalan ffy H,Hu ffmann B,et al1 Hemangioblastomas of the spinal cord and the brainstem: diagnostic and therapeutic features1Neurosurg Rev1996;19:1472 1511
21Rebner M,G ebarski SS1Magnetic res onance imaging of spinal cord hemangioblastoma1Am J Neuroradiol1985;6:28722891
31Xu QW,Bao W M,Mao R L,et al1Magnetic res onance imaging and microsurgical treatment of intramedullary hemangioblastoma of spinal cord1Neurosurgery1994;35:67126761
41Chen LP,Li C Q,Sun X M,et al1The clinical and anatomic study of thoracic outlet syndrome1Chin Orthop J1991;11:1241 51Murota T,Sym on L1Surgical management of hemangioblastoma of the spinal cord:a report of18cas1Neurosurgery1989;25: 69927071
儿童谜语大全3到6岁61Cristante L,Herrmann H D1Surgical management of intramedullary hemangioblastoma of the spinal cord1Acta Neurochir1999;141:33323391
71E pstein F,Wis off J1Intra2axial tum ors of the medullocervical junction1J Neurosurg1987;67:48324871
81San ford RA,Smith RA1Hemangioblastoma of the medullocervical junction:report of three cas1J Neurosurg1986;64:31723211
(Received April19,2001)
本文编辑曹琳冰 王 德
NYK CRFPEI 孵育的外周血单个核白细胞经照射后作为抗原呈递细胞，每隔7天刺激HCC 病人自体外周血单个核
白细胞一次，共4次后作为效应T 淋巴细胞，应用流式细胞仪检测培养前后淋巴细胞的表型变化，应用乳酸脱氢酶法检测效应T 细胞对靶细胞的杀伤效应。
结果 HCC 细胞株BE L7405M AGE 21和H LA 2A24均阳性表达，可用作杀伤实验的阳性靶细胞,H LE 等其它7种细胞不能双表达，可用作杀伤实验的阴性对照细胞；培养28天，淋巴细胞增加31倍；培养28天,C D3+T 和C D8+T 分别增加16%和20%；在效∶靶为10∶1时，效应T 细胞对M AGE 21抗原九肽NYK CRFPEI 孵育的自体淋巴母细胞、BE L7405的杀伤效应分别为6215%和40125%，均明显高于对自体淋巴母细胞(17188%)、H LE (19155%)及QGY 7701的杀伤效应(116%)；在效∶靶为313∶1时，效应T 细胞对肽孵育的自体淋巴母细胞的杀伤效应为5316%，明显高于对自体淋巴母细胞(1516%)、H LE (13%)和QGY 7701的杀伤效应(1%)。
结论 本实验结果表明，应用M AGE 21抗原肽NYK CRFPEI ，在体外从HCC 病人的外周血单个核白细胞中成功地诱导出具有特异性杀伤能力的效应T 细胞，为应用M AGE 21抗原肽对HCC 病人进行免疫治疗提供了理论基础。关键词 黑色素瘤抗原 细胞毒T 淋巴细胞 肝细胞癌 免疫治疗
F as 配体表达对同种胰岛移植的影响actualize
The effect of F asL expression on pancreatic islet allografts
Chin Med J 2002;115(7):100621009
中山医科大学附属第一医院外科广州510080 詹文华 蔡世荣 汪建平 何裕隆 郑章清 彭俊生
目的 探究睾丸细胞Fas L 表达能否对共移植的胰岛移植物提供免疫豁免作用以及胰岛细胞Fas L 基因转染对同种胰岛移植的影响。
方法 将同种大鼠胰岛及睾丸细胞同时移植于糖尿病受体，重组腺病毒AdV 2Fas L 感染胰岛细胞后移植，观察移植物存活情况、胰岛功能，并检测移植物内浸润淋巴细胞以及基因转染胰岛细胞凋亡情况。
结果 单纯移植胰岛组平均存活期为(613±016)d 。与胰岛细胞同时移植的睾丸细胞数增加至1×107时，
emm什么意思存活期大于60d (P <0105)。表达Fas L 的睾丸细胞在移植物内诱导浸润淋巴细胞凋亡。Fas L 基因转染组出现排斥加速，存活期缩短至(314±012)d 。Fas L 转染的胰岛细胞在移植后24h 见Fas L 表达,48h 表达增强，移植后Fas L 转染胰岛细胞凋亡。
结论 表达Fas L 的睾丸细胞与胰岛同时移植可诱导活化的淋巴细胞凋亡，使胰岛移植物获得免疫豁免、存活期延长，但通过Fas L 基因转染使胰岛细胞直接表达Fas L 引起胰岛细胞凋亡和粒细胞浸润，导致排斥加速。关键词 胰岛/移植 Fas 配体 免疫豁免 基因治疗
颈髓髓内血管母细胞瘤的诊断与治疗
Diagnosis and treatment of intramedullary hem angioblastom a of cervical spinal cord
Chin Med J 2002;115(7):101021013
复旦大学附属华山医院神经外科上海200040 徐启武 鲍伟民 庞 力
目的 探讨颈髓髓内血管母细胞瘤的诊断与治疗。
神探夏洛克 字幕
・6011・Chine Medical Journal 2002;115(7):110121117
