松果体区肿瘤的临床病理研究

目的 研究松果体区肿瘤(PRTs)的病理分布特点及不同病理类型的临床特点.方法 回顾性分析南方医院神经外科自2000年1月至2009年1月经手术治疗并取得完整病理资料的133例PRTs患者的临床资料及病理特点.结果 133例患者中生殖细胞肿瘤61例(45.9%),松果体实质肿瘤17例(12.8%),神经上皮肿瘤28例(21.1%),其他肿瘤27例(20.2%).生殖细胞肿瘤中男女比例为14.25:1,平均15.3岁.松果体实质肿瘤中男女比例为2.4:1,平均24.7岁.神经上皮肿瘤中男女比例为1.15:1,平均28.1岁.有33例血清免疫学检查异常,除1例为转移瘤外,其余均为生殖细胞肿瘤.结论 PRTs病理类型多样,以生殖细胞肿瘤为主;影像学检查、血清免疫学检查及活检均不能准确判断病理类型,积极手术获得完整病理标本对于PRTs的病理研究有重要意义.     

松果体区肿瘤的临床病理研究

目的 研究松果体区肿瘤(PRTs)的病理分布特点及不同病理类型的临床特点.方法 回顾性分析南方医院神经外科自2000年1月至2009年1月经手术治疗并取得完整病理资料的133例PRTs患者的临床资料及病理特点.结果 133例患者中生殖细胞肿瘤61例(45.9%),松果体实质肿瘤17例(12.8%),神经上皮肿瘤28例(21.1%),其他肿瘤27例(20.2%).生殖细胞肿瘤中男女比例为14.25:1,平均15.3岁.松果体实质肿瘤中男女比例为2.4:1,平均24.7岁.神经上皮肿瘤中男女比例为1.15:1,平均28.1岁.有33例血清免疫学检查异常,除1例为转移瘤外,其余均为生殖细胞肿瘤.结论 PRTs病理类型多样,以生殖细胞肿瘤为主;影像学检查、血清免疫学检查及活检均不能准确判断病理类型,积极手术获得完整病理标本对于PRTs的病理研究有重要意义.     

松果体区混合性生殖细胞肿瘤临床与病理分析

目的 总结松果体区混合性生殖细胞肿瘤的临床特点、病理组织学构成,并探讨其相关的治疗策略.方法 对经手术切除,且病理检查详尽的14例松果体区混合性生殖细胞肿瘤进行回顾性分析.结果 14例均为男性,平均年龄17.3岁.其中13例包含生殖细胞瘤,其他成分即可能是单一的成熟畸胎瘤,也可能是多种高度恶性的生殖细胞肿瘤.血清肿瘤标志物升高基本反映了肿瘤中包含有相应的肿瘤成分.结论 松果体区混合性生殖细胞肿瘤临床表现无明显特殊.肿瘤大多包含有生殖细胞瘤成分,术前全面准确的定性诊断困难.先手术切除肿瘤以获取完整的病理诊断,而后采取针对性综合治疗似乎更合理.当血清肿瘤标志物正常,且影像学上肿瘤密度或信号混杂时,“试验性”放疗应慎重.     

松果体区肿瘤致男性性早熟8例临床分析

目的探讨男性松果体区肿瘤患儿性早熟的原因和临床特征。方法对8例有性早熟表现的男性松果体区肿瘤患儿的临床资料进行回顾性分析,并在肿瘤病理和生殖细胞肿瘤标志物方面与11例无性早熟表现的患儿进行比较。结果本组8例性早熟患儿占同时期、同年龄段男性松果体区肿瘤患儿的42%。3例性早熟为首发表现,5例为临床表现之一。性早熟多早于其它症状出现,但常被忽视。性早熟患儿的松果体区肿瘤均为恶性生殖细胞肿瘤,且均能分泌β-人绒毛膜促性腺激素（β-HCG）。结论男性松果体区肿瘤患儿的性早熟是假性性早熟,肿瘤分泌HCG导致血清HCG异常增高是性早熟的主要原因。     

手术治疗松果体区肿瘤临床分析

目的 观察松果体区肿瘤患施行神经外科手术治疗的效果及其预后。方法 对1985—2001年收治的30例松果体区肿瘤患的病理类型、临床资料、治疗方法及预后进行回顾性分析。结果 30例患中，典型Parinaud综合征患6例；X线检查显示颅内高压3例；CT及MRI均示第三脑室后部占位性病变，两侧脑室及第三脑室扩大。全部患均施行手术治疗，经枕下幕上入路19例，幕下小脑上入路11例；19例同时行第三脑室后部造瘘；肿瘤全切除11例，次全切除8例，部分切除8例，活检加内分流3例；病理检查结果显示，生殖细胞肿瘤15例，松果体实质肿瘤5例，脑膜瘤3例，室管膜瘤1例，星形细胞瘤2例，其它病变4例。随访11例(1—6年)，其中生存5年以上7例；复发4例，其中3例死亡。结论 松果体区最常见的肿瘤是生殖细胞肿瘤，临床表现多无特异性。手术既可切除肿瘤，解除梗阻性脑积水，又可获得充足的组织标本明确肿瘤性质，以指导下一步的治疗。松果体区肿瘤患的预后大多良好。     

含成熟畸胎瘤的松果体区肿瘤病理分析及诊治

目的探讨如何全面诊断和恰当治疗包含成熟畸胎瘤的松果体区肿瘤。方法对14例含成熟畸胎瘤的松果体区肿瘤的临床资料、诊治过程、病理构成进行总结分析。结果单纯成熟畸胎瘤9例,成熟畸胎瘤+生殖细胞瘤4例,成熟畸胎瘤+绒毛膜癌1例。8例手术前曾接受过不同形式的放疗。术后均恢复良好,但2例在鞍上出现新的肿瘤,考虑为种植转移。结论含成熟畸胎瘤的松果体区肿瘤诊断及鉴别诊断困难,手术是首选治疗方法,术前放疗应慎用,术后处理应规范。     

松果体区非生殖细胞瘤性恶性生殖细胞肿瘤的治疗

目的 探讨松果体区非生殖细胞瘤性恶性生殖细胞肿瘤(NGMGCTs)的临床特点、治疗和预后.方法 回顾性分析了2000年1月至2010年1月经病理证实的34例高度恶性NGMGCTs 患者的临床特点、血清肿瘤标记物检测、治疗方法及预后.所有患者均行枕部经小脑幕(Poopen)入路显微手术切除肿瘤,并行辅助放化疗.结果 全切除32例,近全切除2例,术后病理示未成熟畸胎瘤11例,畸胎瘤恶性变2例,胚胎癌2例,卵黄囊瘤l例,绒毛膜上皮癌6例,混合性生殖细胞肿瘤12例.共随访31例患者,随访时间6个月至10年,1年生存率为97％,3年生存率为62％,5年生存率为44％.结论 多数松果体区NGMGCTs根据临床表现、影像学资料和肿瘤标记物可在术前定性,以手术为主术后辅以化疗和放疗的综合治疗可以获得良好疗效.     

松果体区生殖细胞肿瘤放射外科治疗的合理性探讨

目的 探讨立体定向放射外科作为一线方案治疗松果体区生殖细胞肿瘤的合理性.方法 回顾性分析14例经伽玛刀或X-刀治疗的松果体区生殖细胞肿瘤患者的临床资料,重点研究肿瘤放射外科治疗后变化及复发、转移情况.结果 14例患者于放射外科治疗后11 d至106个月再次入院.肿瘤放射外科治疗后缓慢增大1例,无明显变化2例,缩小后再增大1例,基本消失后原位复发2例,消失后周边复发4例,原位复发并种植转移2例,原位消失但出现种植转移2例.病理为:生殖细胞瘤5例、混合型生殖细胞肿瘤4例、成熟畸胎瘤3例、非成熟畸胎瘤和卵黄囊瘤各1例.结论 放射外科不能作为松果体区生殖细胞肿瘤的单一治疗方法.假使已采用放射外科治疗,则应依据肿瘤标记物结果和治疗后肿瘤的变化情况,及时手术或综合治疗.

Abstract：

Objective To discuss the rationality of the stereotaxic radiosurgery as the first therapy strategy on pineal region germ cell tumors.Methods To retrospectively analyze the clinical materials of 14cases which received the treatment of gamma knife or X- knife.The condition of tumor changing,recurring and metastasize post- radiosurgery were emphasized.Methods All the patients of 14 cases were admitted again from 11 days to 106 months after which had received radiosurgery.The tumor increasing slowing with 1cases,no marked change with 2 cases,repeated increasing after diminution with 1 case,situ recur after essential disappear with 2 cases,ambitus recur after disappear with 4 cases,situ recur and implantation metastasis 2 cases,situ disappear but implantation metastasis with 2 cases.The pathology results were 5germinomas,4 mixed germ cell tumors,3 mature teratomas,1 unmature teratoma and 1 yolo sac tumor.Conclusion Radiosurgery cant be regarded as the exclusive treatment for pineal region germ cell tumors.If the patients received the radiosurgery,they should be operated or combined therapy in time according the results of tumor marker and the condition of tumor changing.     

伽玛刀治疗松果体区肿瘤

目的 探索伽玛刀治疗松果体区肿瘤的具体方法，进一步提高其疗效。方法 使用伽玛刀治疗松果体区肿瘤42例，肿瘤边缘剂量10-15Gy，平均14.1Gy，中心剂量24-37.5Gy，平均30.2Gy。随访4个月-3年。结果 肿瘤消失19例，明显缩小13例，缩小7例，肿瘤不变3例，均无特殊并发症。结论 伽玛刀是治疗松果体区肿瘤的一种安全有效的方法，对有明显梗阻性脑积水，可先行脑脊液分流术再行伽玛刀治疗。     

松果体区乳头状肿瘤临床病理分析

研究背景松果体区乳头状肿瘤是世界卫生组织于2007年新定义和描述的一种少见的中枢神经系统神经上皮组织肿瘤,可能起源于下连合器的特殊室管膜细胞。组织学以胞质宽大、胞界清楚的柱状上皮样肿瘤细胞排列成乳头状或实体片状结构为特点。因缺乏特征性临床和影像学表现,术前明确诊断困难且极易误诊。本文报告1例典型的松果体区乳头状肿瘤患者的临床病理学特征,以期提高对该病的鉴别诊断能力。方法与结果男性患者,57岁,头痛6个月、加重1个月入院。MRI显示幕上中线松果体区和第三脑室界限清楚的占位性病变,呈高信号,增强后病灶均匀强化。术中可见病灶位于松果体区,突入第三脑室,无囊性变,无包膜,与周围组织界限清楚,手术全切除。肿瘤组织由乳头状结构和实体片状区域构成,乳头状结构含明显的纤维血管轴心,周围包绕数层柱状上皮样肿瘤细胞,大小形态较一致,均富嗜酸性胞质,界限清楚;实体片状区域细胞排列较密集,可见围血管"假菊形团"和室管膜"菊形团"结构,肿瘤细胞浸润周围脑实质。肿瘤细胞广谱细胞角蛋白和S-100蛋白呈弥漫强阳性,胶质纤维酸性蛋白和上皮膜抗原呈阴性。术后未予药物化疗和放射治疗,随访1年,肿瘤未复发。结论松果体区乳头状肿瘤是中枢神经系统鲜见病变,由于缺乏特征性影像学表现,以及组织学形态与其他乳头状肿瘤相似,术前难以明确诊断且在鉴别诊断时有一定困难。临床应提高对松果体区乳头状肿瘤的警惕性,并注意与其他具有相似组织学构象的中枢神经系统原发性或转移性肿瘤相鉴别。     

Poppen入路切除松果体区肿瘤手术间隙的临床研究

目的讨论改良Poppen入路切除松果体区肿瘤时手术间隙的应用。方法16例松果体区肿瘤均采用改良Poppen入路显微手术，观察分析16例肿瘤的松果体区的深静脉引流及各静脉间隙，特别是基底静脉的发育情况，统计各静脉间隙的使用情况。结果术中联合第3、4间隙切除肿瘤最多采用，15例达到镜下全切除。结论松果体区肿瘤采用改良Poppen入路切除优点较多。     

松果体区肿瘤的手术治疗(附158例报告)

目的 探讨松果体区肿瘤的手术治疗策略和技术要点.方法 对采用枕部经小脑幕入路切除的158例松果体区肿瘤患者进行回顾性研究,总结分析其临床资料、手术方法及术后随访结果.结果 肿瘤全切除137例,次全切除19例,部分切除2例.病理证实生殖细胞肿瘤83例,松果体实质肿瘤18例,神经上皮肿瘤29例,其他类型肿瘤28例.术后死亡3例(1.9％),出现同向偏盲、眼球运动障碍等永久性并发症29例(18.4％).19例出现早期或迟发性脑积水.结论 手术切除是松果体区肿瘤的必要治疗手段,合理的手术入路、对松果体区蛛网膜及深静脉系统的深入了解以及娴熟的显微操作技巧,是保障手术安全有效的关键因素.     

Treatment of germ cell tumors in the pineal region

The authors retrospectively analyzed 107 patients with primary intracranial germ cell tumor (GCT), who were treated at the Department of Neurosurgery, Yonsei Medical Center between January 1986 and January 1996. The incidence of GCT was 2.8% in pediatric patients with intracranial tumor. Of the 107 tumors, 60 were located in the pineal region, 30 in the suprasellar region, 16 in basal ganglia or the thalamic region, and 1 in the posterior fossa. The 60 pineal GCT consisted of 39 germinomas (29 pure germinomas, 6 germinomas with STGC, 4 germinomas mixed with teratoma), 5 mature teratomas, and 16 nongerminomatous GCT. Thirty patients underwent surgery: their operations took the form of total resection in 14 cases, subtotal resection in 10, and biopsy in 6. Thirty patients (27 with germinomas, 3 with endodermal sinus tumors) were managed without surgery on the basis of radiological findings and tumor markers. The 5-year survival was 91% for 39 patients with germinomas, 80% for 5 with mature teratomas, and 49% for 16 with nongerminomatous GCT. Univariate analysis of prognostic factors with the Kaplan-Meier survival curve showed that histological tumor type, radiological findings, results of tumor marker studies, and response to trial radiation or chemotherapy were highly correlated with outcome. Chemotherapy was beneficial as the method of trial treatment in pineal GCT and treatment in recurrent tumors. The administration of trial chemotherapy or radiotherapy without tissue biopsy is well justified as a treatment modality in pineal GCT suspected on the basis of radiological findings and tumor marker studies. Aggressive multimodality approaches with surgery, radiotherapy, and chemotherapy are necessary to improve the outcome in these tumors. We propose new protocol for treatment of germ cell tumors in the pineal region, which is based on a minimally invasive approach.     

Management of hydrocephalus secondary to pineal region tumors

Background

Hydrocephalus is often secondary to pineal region tumors. Hydrocephalus can lead to high intracranial pressure, which in turn results in disturbance of consciousness, cerebral hernia, and even death. Hydrocephalus management is important in the treatment of pineal region tumors. It is still controversial regarding to when and how to treat hydrocephalus secondary to pineal region tumors. The objective of this study is to investigate the management of hydrocephalus secondary to pineal region tumors.

Methods

We retrospectively analyzed records for 51 patients admitted to the department of Neurosurgery, Jinling Hospital from April 1997 to September 2010 with hydrocephalus secondary to pineal region tumors treated through occipital transtentorial approach.

Results

Preoperative ventricular drainage was performed on one patient, and ventriculoperitoneal shunts were performed on two patients. Intraoperative ventriculocisternal shunts were performed on 35 patients (the remission rate was 88.6%), no treatments on 15 patients (the remission rate was 46.7%), and ventricular drainages on three patients. VP shunts were performed on 12 patients with no remission after the operation.

Conclusion

Pineal region tumors resection usually should be performed before shunting, unless there is an acute obstructive hydrocephalus. The posterior third ventricle should be opened after tumor resection. Intraoperative third ventriculostomy and ventriculocisternal shunt are reliable ways to manage hydrocephalus secondary to pineal region tumors.     

Experience with pineal region tumors

The results are reported of a retrospective review of the presentation and outcome of 43 pineal region tumors treated from 1982 to 1996, including 20 identified tumors: 5 germinomas, 8 teratomas, 2 embryonal carcinomas, 1 endodermal sinus tumor, 2 pineocytomas and 2 pineoblastomas. Of the 43 tumors reviewed, 36 were located in the pineal region, 5 in the suprasellar, and 2 in both the pineal and suprasellar regions. Twenty patients underwent surgical resection: total in 6 and partial in 10, while only a biopsy was taken in 4 cases. Fifteen patients were managed on the basis of serum CSF tumor markers and radiation response. Twenty-three patients with germinomas received radiotherapy (RT) and had a 5-year survival rate of 87%. Fifteen patients with non-germinomatous germ cell tumors received RT and chemotherapy following direct surgery, and 5 died (mortality rate of 33.3%). The overall survival rate of the 43 patients with pineal tumors was 79.1% (34/43) and the death rate was 20.9% (9/43). It is now recognized that the wide variety of tumor types found in the pineal region necessitates different modes of treatment, and improved microsurgical and stereotactic surgical techniques have made mortality and morbidity rates acceptably low. Because the radiation response and CSF cytology are not enough to determine optimum treatment, a tissue diagnosis should be obtained in all patients.