颅内生殖细胞瘤38例临床与影像学分析

目的：总结颅内生殖细胞瘤的临床表现及影像学特点。方法：对38例颅内生殖细胞瘤患者的临床资料和影像学改变进行分析。结果：38例中年龄小于18岁32例（84％），偏瘫14例，尿崩症11例。头痛、呕吐8例，视力减退8例，视乳头水肿8例，丘脑底部综合征7例，复视4例，性早熟3例。肿瘤位于松果体区13例，鞍区11例，基底节区14例。位于松果体区或鞍区的生殖细胞瘤，CT表现为圆形或类圆形病灶，增强扫描后呈均匀或不均匀强化，钙化不多见；磁共振表现为不规则形病灶，T1、T2加权像呈均匀等信号，增强扫描后病灶明显强化。基底节区生殖细胞瘤在CT上表现为形态不规则病灶；磁共振上表现为T1加权像混杂低信号，T2加权像呈混杂高信号。结论：颅内生殖细胞瘤患者以儿童、青少年多见，病变多位于松果体区、鞍区或基底节区。临床主要表现为颅内压升高、尿崩症、视功能障碍或偏瘫，结合影像学检查有助于其诊断。     

颅内生殖细胞瘤围放疗期的护理

颅内生殖细胞瘤是一种少见的胚胎性肿瘤,多见于青少年,好发于松果体区,其次为鞍上区、丘脑、基底节区.由于生殖细胞瘤对放疗十分敏感,单纯放疗存活期较长,5年存活率高于70％[1],但放疗中可能给患者带来较大的不良反应和放射性损伤.为提高临床预后和患者生活质量,2009年2月至2012年6月我科对收治的9例颅内生殖细胞瘤患者行围放疗期护理,效果满意,现报道如下.     

松果体区非典型中枢神经细胞瘤1例并文献复习

目的探讨松果体区非典型中枢神经细胞瘤的临床特征、诊断及治疗方法。方法回顾性分析1例松果体区非典型中枢神经细胞瘤患者的临床资料,结合文献复习,总结其临床表现、诊断、显微外科手术效果,并探讨术后进一步治疗的必要性。结果手术近全切除,术后结合放疗,随访18个月,肿瘤无复发。结论松果体区非典型中枢神经细胞瘤,手术全切除是最佳治疗治疗手段,未全切除和/或术后病理显示肿瘤恶性级别较高患者,术后放疗或化疗可减少肿瘤复发率。     

颅内非松果体区生殖细胞瘤的影像诊断与鉴别诊断

生殖细胞瘤起源于胚生殖细胞，也称胚生殖细胞瘤，占颅内原发瘤的1％，好发于松果体区，也可以发生于松果体以外部位，如第三脑室、鞍上、下视丘、基底节、额叶、颞叶深部，也可发生于幕下，第四脑室。这些发生于松果体以外部位的生殖细胞瘤称为非松果体区生殖细胞瘤，过去称为异位松果体瘤，以鞍区和基底节区多见。本文收集8例非松果体区生殖细胞瘤，所有病例均经我院手术病理证实。     

松果体区肿瘤手术治疗方法的选择

目的探讨手术切除松果体区肿瘤的意义、肿瘤切除的程度、手术入路的选择以及脑积水的处理方法。方法我院收治松果体区肿瘤患者23例,为缓解脑积水,先后行脑室一腹腔（V-P）分流术13例,术中同时行终板造瘘术7例,其中2例术后仍需行V—P分流术。经胼胝体．透明隔．穹隆间。第三脑室入路切除肿瘤13例;经枕下小脑幕上入路（Poppen入路）切除肿瘤8例;经幕下小脑上入路（Krause入路）2例。结果肿瘤全切除11例,次全切除＋大部切除8例,部分切除4例。术后单纯放疗9例,术后放疗＋化疗8例,6例未行放、化疗。死亡2例。结论肿瘤偏大,术前诊断＋术中病理诊断为生殖细胞瘤者可不必完全切除肿瘤,但需解决脑脊液循环通路问题,包括V．P分流术、第三脑室底经终板造瘘术,如肿瘤较小且易于切除,估计对周围脑组织影响不大,第Ⅲ、Ⅳ脑室间能保持通畅者可不行分流术或造瘘术,术后放、化疗。其他病理类型肿瘤争取肿瘤全切除,以提高疗效,特别是良性肿瘤。     

颅内生殖细胞瘤复发原因分析

目的探讨颅内生殖细胞瘤治疗后复发的原因。方法回顾性分析79例颅内生殖细胞瘤患者的临床资料。结果治疗后3个月评价近期疗效,各种治疗方法均获得]00％有效率。随访2a,1l例（13．9％）复发和转移,转移或复发中位时间12．6个月;复发者松果体区病变（63．6％）高于病情稳定者（42．7％）,症状与发病部位有关;稳定组72．1％患者采用放、化疗联合治疗,复发组72．7％患者以单一治疗为主,3例7刀治疗患者复发后再行放、化疗联合治疗,均获得理想效果。结论早期诊断和规范手术治疗并合理联合放、化疗可有效控制颅内生殖细胞瘤,减少复发及转移。     

颅内生殖细胞瘤治疗失败3例分析及规范建议

颅内生殖细胞瘤是儿童及青少年好发的颅内恶性肿瘤,常见于鞍上下丘脑、松果体及基底节区。临床表现有中枢性尿崩症、垂体前叶功能低下、梗阻性脑积水及偏瘫等。颅内生殖细胞瘤对放射治疗非常敏感,放射治疗是主要的根治手段,治愈率高。本科室曾报道1999—2007年间收治的74例颅内生殖细胞瘤患者,其1、5、10年总生存率和无复发生存率分别为99％和97％、96％和90％、93％和83％。     

颅内生殖细胞瘤的MRI影像表现及诊断研究

目的:研究颅内生殖细胞瘤的MRI影像表现及诊断效果。方法:按照入院顺序随机抽取2015年2月至2017年2月收治的颅内生殖细胞瘤患者60例,对其进行MRI影像诊断,观察影响表现并分析诊断结果。结果:60例中2例是多发、58例是单发。2例同时位于松果体区以及鞍上、3例位于三脑室内、15例位于鞍上、40例位于松果体区。共发现62个病灶,其中21个呈圆形、41个呈不规则形。最小肿瘤直径是1.6cm×1.6cm,最大肿瘤直径是3.8cm×4.2cm。结论:颅内生殖细胞瘤患者进行MRI检查,可以对病变部位及其周围组织做出清晰的反映,具有较高的诊断价值,值得借鉴和推广。     

36例松果体区生殖细胞瘤的治疗

目的比较松果体区生殖细胞瘤的不同治疗方法间疗效的差异。方法回顾分析36例松果体区生殖细胞瘤，病理诊断13例，临床诊断23例。治疗方法：局部放疗15例，全脑放疗9例，全脑全脊髓放疗10例。应用spss10．0软件包分析各组的差别。结果总体3年无瘤生存率62．41％，5年无瘤生存率54．61％。结果显示全脑全脊髓放疗组有最低的转移率及最高的长期生存率，但无统计学差异。结论推荐全脑全脊髓放疗为松果体区生殖细胞瘤的首选治疗方法。     

鞍区肿瘤手术并发症的观察与护理

鞍区肿瘤主要有各种类型的垂体腺瘤、颅咽管瘤。异位松果体瘤，视神经胶质瘤等。鞍区肿瘤以手术切除为主。由于手术切除体积大的肿瘤易损伤鞍区正常组织，特别是可能牵拉损伤丘脑下部，引起各种神经症状甚至导致死亡。据报道的常见并发症有视力、视野障碍加重，意识障碍，...     

松果体区肿瘤的治疗策略研究

目的：探讨松果体区肿瘤的治疗策略。方法：我院自2003年12月至2007年12月收治的58例松果体区肿瘤,按肿瘤标志物甲胎蛋白（AFP）和人β-促绒膜性腺激素（hCG-β）将其分为标志物增高组和正常组,回顾分析两组临床资料和治疗体会。结果：肿瘤标志物正常组36例,其中27例行手术切除肿瘤,9例行伽马刀治疗（其中5例行伽马刀加脑室腹腔分流治疗）。术后4例因病理报告示生殖细胞瘤行伽马刀补充治疗,3例因梗阻性脑积水行脑室腹腔分流。增高组22例,均选用伽马刀治疗（其中17例合并梗阻性脑积水者行伽马刀加脑室腹腔分流）。治疗后3例因症状缓解不佳行手术切除肿瘤术。手术病例中24例采用经胼胝体入路,4例经枕部小脑幕入路,2例经幕下小脑上入路。手术全切24例,次全切5例,部分切除1例,术后住院期内死亡者1例。病理诊断：生殖细胞瘤14例,畸胎瘤5例,中枢神经细胞瘤4例,室管膜瘤2例,表皮样囊肿2例,脑膜瘤1例,胶质瘤1例,皮样囊肿1例。放射外科治疗病例中28例术后3个月临床症状明显改善,4例随访期内肿瘤无明显变化,3例肿瘤增大。2例放疗后出现鞍区转移。结论：松果体区肿瘤组织类型多样,应尽可能先明确肿瘤性质后选择合理治疗策略。     

Diabetes insipidus with impaired vision caused by germinoma and perioptic meningeal seeding: A case report

BACKGROUNDGerminoma is a type of germ cell tumor that most frequently arises in the midline axis of the brain. Impaired vision is a clinical manifestation of germinnoma. Although rare, intracranial germinoma seeding to the perioptic arachnoid space is one cause of visual acuity decrease.CASE SUMMARYAn 11yearold girl who presented with polyuria and polydipsia and subsequently developed diminution of vision. Imaging showed bilateral heterogeneous enhancement of the optic nerve sheaths and atrophy of the optic nerve, and transsphenoidal biopsy revealed a germinoma. The patient experienced poor visual recovery following chemotherapy and radiotherapy. Germinomas are rare and they are mostly identified in children and adolescents. The manifestations include diabetes insipidus, pituitary dysfunction, visual complaints, etc. The mechanisms that lead to visual loss include intracranial hypertension, compression of optic chiasma, and tumor invasion. A literature review was performed to summarize the cases with a tumor infiltrating the optic nerve. Most of the reported patients were adolescents and presented with anterior pituitary hormone deficiency. Enhancement of optic nerve sheaths and optic disc pallor could be identified in most of the cases. The purpose of this report is to provide awareness that in cases where a germinoma is associated with visual loss, though rare, perioptic meningeal seeding should be taken into consideration.CONCLUSIONThe case report suggests that children with diabetes insipidus need a complete differential diagnosis.     

X-刀治疗颅内疾病44例疗效分析

Objective to investigate therapeutic methods and effect of X-knife for on intracranial diseases. Method Recent effect of 44pqtients with cranial diseases by X-knife was observed. Radiological follow-up was performed on 40 cases with mean 5.65 months of followup time. Result 92.5% of tumors were controlled locally, stability and recovery rate was 90.0%. The local control of metastatic tumors of brain was higher, but most patients with metastatic tumors died of primary lesion. New metastatic lesions appeared in patients without panencephalic radiotherapy in 1～5months. Tumors of pineal region were sensitive to X-knife. Conclusion X-knife has a definite effect on intracranial diseases. For patients with tumors of pineal region complicated by serious hydrocephalus, shunting should be conducted before X-knife treatment. For patients with mild or morderate hydrocephalus, X-knife chould be utilized only under correct interventions such as dehydration. The local control rate of intracranial metastatic tumors was high, but survival time postoperation depended on panencephalic radiotherapy or control of primafry lision. For tumors with diameter＞ 3cm, pituitary tumors, brains stem tumors and tumors in cerebellopontine angle region repeated X-knife were suggested, which could improve cure rate and decrease complications.     

Does the rapid response to Cisplatin-based chemotherapy justify its use as primary treatment for intracranial germ-cell tumours?

We reviewed presentation, diagnostic problems and outcome ofeight cases of primary intracranial germcell tumour (4 germinoma,4 teratoma) treated with Cisplatin-based chemotherapy at ourcentre over the last ten years. Three patients received primarychemotherapy with Cisplatin-based regimens followed by radiotherapyfor subsequent relapse, two were treated with a combinationof chemotherapy and external radiotherapy, and three receivedchemotherapy for relapse after radiotherapy. The response toCisplatin-based chemotherapy was rapid, with some patents exhibitingsymptomatic improvement within 24 h. Four patients achievedcomplete remission within 21 days, and three of these have remainedprogression-free. Four patients in total have survived for 32to 128 + months. Six of seven patients tested pre-treatmenthad central diabetes insipidus and five had partial anteriorpituitary failure. The endocrine deficit progressed in two,with no recovery in any patient. It is arguable that chemotherapyshould be the primary therapy in all such cases diagnosed onthe basis of tumour markers and imaging, with surgery and/orradiotherapy as later options. As these tumours are rare, suchquestions can only be answered by collaboration studies.     

松果体区肿瘤的显微手术治疗

目的：探讨显微手术治疗松果体区肿瘤的手术入路和技术。方法：采用显微手术技术和幕下小脑上入路切除9例松果体区肿瘤;其中生殖细胞瘤5例、混合性生殖细胞肿瘤2例（分别为生殖细胞瘤合并恶性畸胎瘤和胚胎癌）、脑膜瘤和良性畸胎瘤各1例;1例术后脑积水行三脑室底造瘘术。结果：所有病例全切除肿瘤。术后早期结果全部良好,随访1～7年：良好7例,死亡2例。结论：松果体区肿瘤手术难度较大,但选择适合的手术入路和熟练的显微手术技术可以取得满意的疗效。     

松果体区生殖细胞瘤扩散的MRI研究

目的分析松果体区生殖细胞瘤扩散的途径和MRI表现。方法24例松果体区生殖细胞瘤均经手术和病理证实,其中13例经临床和诊断性放疗证实为松果体区生殖细胞瘤的扩散。结果16例沿脑脊液(CSF)种植;9例沿室管膜下扩散;13例向邻近脑组织浸润蔓延。侧脑室旁扩散为等T1长T2或稍长T2,多个结节或连接成片;小脑幕、大脑镰和软脑膜扩散多表现为“绳索”样增粗,也可为多个或单个结节;环池、第4脑室和小脑上蚓池多为强化结节。除3例透明隔和1例脊髓马尾瘤灶外,MRI均显著均匀强化。结论扩散除具有恶性肿瘤扩散的共性———直接浸润蔓延和沿CSF种植外,还可沿室管膜下扩散,MRI增强扫描可清楚显示多发的和较小的转移灶。     

133例松果体区肿瘤病人的术后护理

目的探讨松果体区肿瘤病人术后的护理体会。方法回顾性分析本院2000年1月-2009年6月收治的133例松果体区肿瘤病人的临床资料,并总结术后的观察及护理要点。结果所有病人术程顺利,手术时间196-436min,中位时间268.0min。术后并发症情况:颅内出血6例,早期脑积水6例,迟发性脑积水8例,不耐受气管插管5例,拔管后血氧饱和度下降1例;新出现眼球运动障碍13例、复视5例、视野缺损6例,死亡2例。结论对松果体区肿瘤病人术后做好生命体征及引流管的观察和护理,及时发现并处理颅内出血和脑积水,其是手术取得成功的保证。     

松果体区肿瘤的显微外科手术治疗

目的:研究比较松果体区肿瘤的显微外科手术切除不同手术入路及其优缺点。方法:回顾分析收治的48例松果体区肿瘤患者的手术情况及术后表现,并进行分析。结果:48例患者中,经枕小脑幕切开(Poppen)入路31例,幕下小脑上(Krause)入路8例,经胼胝体-穹窿间入路6例,经胼胝体后部(Dandy)入路2例,颞部侧脑室三角部入路1例。肿瘤全切除40例,次全切除5例,大部分切除3例。8例未全切者及病理证实为恶性的病变术后行放疗和(或)化疗,5例术后并发脑积水行分流术,偏盲1例,死亡2例,随访6个月～6年,KPS大于80分者约43例。结论:松果体区肿瘤的手术治疗效果较好,全切率高、死亡率低,合理的入路及体位,娴熟的显微外科手术技巧是手术成功的关键,Poppen入路和Krause入路是符合微创理念的理想入路。     

原发性皮肤间变性大细胞淋巴瘤13例临床病理分析

目的探讨原发性皮肤间变性大细胞淋巴瘤(ALCL)的临床病理形态特征、免疫组化及预后特点。方法对13例原发性皮肤ALCL进行形态学观察,免疫组化标记及随访,并结合相关文献进行讨论。结果 13例原发性皮肤ALCL中,男女之比为1.6∶1,平均年龄47岁。临床表现为皮肤斑块、结节、肿块;组织学形态多样;免疫组化示CD30均(+),大多数CD3和/或CD43(+),部分EMA、GranB和perforin(+),ALK、CKpan、CD20、CD79a、HMB45、CD68、CD15和CD117(-)。13例均经外科手术切除局部病变,再辅助以化疗和∕或放疗;9例随访时间5～55个月,1例死亡。结论原发性皮肤ALCL是低度恶性淋巴瘤,预后相对较好。诊断依赖于组织病理学及免疫组化标记。本病应与弥漫性大B细胞性淋巴瘤、黑色素瘤、低分化腺癌等鉴别。