3.0T磁共振诊断腹膜后神经鞘瘤1例

患者男,46岁,体检发现腹膜后肿物,MRI扫描示左中腹部见一巨大软组织异常信号影,T2为混杂信号,其内可见不规则长T2信号,T1以等信号为主,大小约10.0 cm×7.0 cm×6.0 cm,境界清晰,周围肠管可见挤压征;增强后,该病灶均匀强化,边缘似可见包膜,延迟期病灶内部仍可见无强化区.术后病理诊断:腹膜后神经鞘瘤,瘤细胞丰富,局灶生长活跃,核分裂象1～3个/10HPF,间质内可见较多慢性炎细胞及泡沫细胞浸润.免疫组化染色显示肿瘤细胞:Bcl-2(灶状+),CD34(-),CD68(+吞噬细胞),CD99(+)、S-100(++),SMA(-),CD117(-),actin(pan)(-).     

Neurilemmoma of median nerve

Neurilemmomas constitute one of the most common tumors of peripheral nerves. Rarer amongst them is their occurrence in median nerve in the region of arm. A sixteen-year-old female presented with painless mass in right arm which was non-tender on palpation with positive Tinel''s sign and no motor or sensory deficit in the affected arm. Ultrasound examination revealed an eccentrically arising mass of size 19 × 11 mm along median nerve. MRI study revealed a mass homogenously isointense on T1 weighted images and hyperintense in T2 weighted images placed eccentrically in relation to median nerve in arm. Excisional biopsy under loupe magnification was carried out which revealed the mass to be neurilemmoma. This slowly growing benign tumor of peripheral nerves with an incidence of 5% with 14% involvement of Median nerve can be enucleated from the nerve with little or no damage. In spite of advanced imaging studies the mass cannot be differentiated preoperatively from another peripheral nerve sheath tumor neurofibroma. Both these tumors although bearing some clinical and imaging resemblance carry different intraoperative findings, histopathological features and post-operative results.     

腹膜后恶性外周神经鞘瘤1例

患者女,41岁,于2个月前出现右侧腰部疼痛,近1个月来进行性加重,伴尿频、尿急、腹胀、乏力、纳差,无发热、呕吐.CT平扫于肝脏与右肾间见不规则混杂密度肿块影,约12.6 cm×8.7 cm×18.3 cm,其内可见片状低密度区、斑片索条状软组织密度影及钙化影;增强扫描肿块不均匀强化,斑片索条状影强化明显(CT值由41 HU强化为108 HU),片状低密度区无强化(CT值约为13 HU);肿物推挤肝脏、右肾及右侧肾上腺,与周围组织边界尚清,下腔静脉受压向左移位,内可见小的充盈缺损(图1、2).     

原发性腹膜后神经鞘瘤的外科治疗

目的 探讨原发性腹膜后神经鞘瘤的诊治与预后.方法 回顾性分析1995年1月至2009年12月北京大学第一医院诊治的47例原发性腹膜后良、恶性神经鞘瘤患者的临床资料.结果 良性神经鞘瘤36例,中位年龄41岁,体检发现25例,有症状者11例;恶性神经鞘瘤11例,中位年龄38岁,体检发现5例,有症状者6例.术前CT和MRI诊断阳性率分别为36.2%(17/47)和58.3%(7/12).免疫组化染色良性组S-100阳性率100%;恶性组S-100阳性者率81.8%(9/11).47例患者均行手术治疗,良性组手术切除率100%,恶性组手术切除率90.9%(10/11),两组均无围手术期死亡患者,术后并发症5例(10.6%).良、恶性神经鞘瘤术后5年生存率分别为100%与45.5%.良性组术后复发2例;恶性组术后复发4例,远处转移3例.结论 腹膜后神经鞘瘤确诊依靠病理及免疫组化检查,手术完整切除是治疗主要方法.良性神经鞘瘤预后良好,恶性神经鞘瘤易转移和复发.

Abstract：

Objective To explore the clinical diagnosis and surgical treatment of primary retroperitoneal neurilemoma (schwannoma). Methods Clinical data of 47 patients of primary retroperitoneal schwannoma admitted and surgically treated from January 1995 to December 2009 were retrospectivelly reviewed. Results As diagnosed by pathology there were 36 cases of Benign schwannoma,with a median age at onset of 41 years, among those 11 patients were symptomatic, and 25 were asymptomatic. There were 11 malignant 11 cases, the median age was 38 years, among those 6 patients were symptomatic, and 5 were asymptomatic. The positive diagnostic rate of preoperative CT and MRI were 36. 2％ ( 17/47 ) and 58. 3％ ( 7/12 ) respectively. Immunohistochemically positive rates of S-100 were 100％ and 81.8％ (9/11) in benign and malignant group respectively. All cases underwent surgical treatment. Surgical resection rates for benign and malignant groups were 100％ and 90. 9％ (10/11)respectively. There was no perioperative death, Overall 5-year survival rates were 100％ and 45.5％ for benign and malignant tumors groups respectively. In benign group 2 cases recurred, in malignant group 4 cases recurred, and 3 had distant metastasis. Conclusions Primary retroperitoneal schwannomas are less common. It is difficult to make an accurate preoperative diagnosis. Surgery is the most effective therapy.Prognosis is good for benign and poor for malignant retroperitoneal neurilemomas.     

后腹腔镜肾上腺神经鞘瘤切除

目的探讨后腹腔镜技术治疗肾上腺神经鞘瘤的安全性和可行性。方法回顾性分析2004年1月至2011年6月单县中心医院泌尿外科经腹膜后途径腹腔镜下切除7例肾上腺神经鞘瘤的诊治。B超偶然发现2例、血压升高3例、腰腹部酸疼2例。7例均行B超及CT检查,肿瘤位于右侧肾上腺区5例、左侧肾上腺区2例。结果7例手术均获成功,无中转开放,无大出血,肿瘤直径3．0～7．0cm,平均5．0cm;腔内手术时间45～120min,平均75min;出血量20—100ml,平均50ml;术后住院7—8d;随访2～12个月,未见肿瘤复发或远处转移。结论后腹腔镜切除肾上腺神经鞘瘤,手术出血少、创伤小、恢复快,是较好方法。     

面神经肿瘤的诊断和鉴别诊断

目的回顾性分析9例面神经肿瘤的临床特征,探讨面神经肿瘤的诊断与鉴别诊断方法。方法分析9例面神经肿瘤患者的一般临床资料,主诉和常规检查所见,听力学检查和面神经功能评价,影像检查,以及手术所见和术后病理检查报告。结果面神经肿瘤生长缓慢,本组9例面神经肿瘤患者平均病程3.7年,术前有6例发现面神经功能下降,4例发现听力下降,有6例经外耳道发现肿块,影像检查表现为面神经径路上的膨胀性生长肿块,较大肿块显示界限清楚的囊实性肿块,不均匀强化,但中耳乳突腔无慢性炎症征象。肿瘤破坏侧颅底可发现肿瘤主体位于颞骨内并有茎乳孔区扩大。单纯腮腺内和桥小脑角面神经瘤术前很难明确诊断。术后病理诊断面神经鞘瘤6例,面神经血管瘤1例,面神经纤维瘤2例。结论面神经肿瘤发病率低,需结合临床症状和影像学检查进行诊断,面神经径路上的占位病变和面神经功能检查是主要的鉴别诊断方法。     

高频超声诊断外周神经鞘瘤

神经鞘瘤分布广泛,在肿瘤边缘找到与其发生神经的连接关系是超声诊断该病的要点。本研究回顾性分析31例体表神经鞘瘤的声图像特征及误诊原因,探讨其与其他常见软组织肿瘤的鉴别诊断要点。     

纵隔巨大神经纤维鞘瘤病例报道并文献复习

目的报道1例无症状纵隔巨大神经纤维鞘瘤,提高其认识。方法对2009年5月上海长海医院收治的1例纵隔神经纤维鞘瘤患者的临床、影像学及病理资料进行分析,并复习相关文献。结果患者体查发现右侧胸腔巨大占位1周就诊,经CT引导下行组织穿刺活检,病理结果证实为神经纤维鞘瘤,并行外科手术切除治疗。结论巨大纵隔神经纤维瘤发病率低,临床表现多不典型,易误诊、漏诊,多生长至体积巨大后才被发现,增加了手术风险。经皮穿刺组织活检或开胸活检病理检查可明确诊断。     

右侧腹股沟及盆腔多发神经鞘瘤1例

患者男,50岁.因"右侧腹股沟扪及包块"入院检查,既往无肿瘤病史.盆腔CT:右侧腹股沟及盆腔右侧见多个大小不等类圆形软组织影,部分融合成团,密度均匀,边界清楚;增强后轻度强化,膀胱右侧受压,其他脏器未见侵犯(图1).术前诊断为"右侧隐睾恶变,精原细胞瘤可能伴右侧腹股沟、髂内多个淋巴结转移";术后病理:肿物可见薄层完整的纤维包膜.由Antoni A区及B区结构构成,A区瘤细胞较密集,排列紧密,细胞核杆状,分化良好,B区瘤细胞较分散,排列疏松,周围水肿.病理诊断:多发神经鞘瘤.