Metachronous neurohypophysial immature teratoma occurring 10 years after total resection of pineal mature teratoma

An 18-year-old boy presented with an immature neurohypophysial teratoma occurring 10 years after total resection of a mature pineal teratoma through an occipital transtentorial approach in 1989. Thorough histological examination had revealed a mature teratoma. He developed panhypopituitarism and diabetes insipidus in 1999. Magnetic resonance imaging revealed a suprasellar tumor occupying the third ventricle. This tumor was totally resected through a frontobasal approach. Histological examination revealed an immature teratoma. This tumor occurred in a different site from the initial tumor and was considered to be de novo and thus a so-called metachronous germ cell tumor. Patients with completely resected mature teratoma require extended follow-up, including periodic magnetic resonance imaging, because of the risk of such a metachronous germ cell tumor. Received: 19 June 2000 Revised: 10 August 2000     

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.     

松果体区畸胎瘤的诊断和治疗

目的 探讨松果体区畸胎瘤的临床特征和治疗方法.方法 对16例开颅手术的松果体区畸胎瘤的临床资料及其随访结果进行回顾性分析.结果 以颅高压起病16例,CT扫描钙化14例,MRI囊变7例,血清学甲胎蛋白(AFP)增高2例.行枕部经小脑幕入路手术,成熟畸胎瘤6例,未成熟畸胎瘤10例,无手术死亡,术后并发视野缺损3例,随访6个月至5年,复发5例,死亡3例.结论 松果体区畸胎瘤多见于儿童男性,常见临床表现是颅高压和共济失调,影像学有形态不规则、钙化、囊变、不均一增强等表现,影像学和血清学检查有助于区分成熟和未成熟畸胎瘤,初次放射治疗无效,应积极手术治疗,术后根据病理结果辅助放疗,可取得较满意的疗效.     

松果体区成熟畸胎瘤的临床特点及治疗分析

采用经枕部小脑幕入路手术治疗9例松果体区成熟畸胎瘤患者,无一例围手术期死亡,术后均经病理检查明确诊断。术后4例发生手术相关性并发症(双眼上视困难、视野缺损、癫发作)。术后共随访3个月至7年,平均3.70年。松果体区成熟畸胎瘤多见于男性儿童,以颅内高压和共济失调为主要临床表现,神经外科手术治疗可获得满意疗效。     

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.     

Controversy pertaining to therapeutic modalities for tumors of the pineal region: a worldwide survey of different patient populations

The management of tumors of the pineal region differs between Western countries and Japan. This paper reports on a worldwide survey of individual experience and regimens for treating pineal region tumors in different patient populations. Fifteen pediatric neurosurgeons from nine different countries participated in the survey, and a total of 408 pineal region tumors were evaluated. Determination of tumor histology as an initial procedure was strongly supported by the majority of neurosurgeons in North and Central America and Europe (group A), whereas all but one from Asia and Egypt (group B) emphasized initial application of the radiation test. The analysis of patient populations clearly revealed racial differences in tumor type which explain this discrepancy. Germinoma, the most radiosensitive tumor, constituted 43–70% (mean: 53.7%) of tumors in group B, followed by teratoma, pineoplastoma, and others, whereas in group A the incidence of germinoma was only 21–44% (mean: 34.7%), followed by a variety of tumors, such as astrocytoma, pineoblastoma, etc. The age distribution among intracranial germ cell tumors (GCT) obtained from data from the Brain Tumor Registry in Japan also demonstrated clear differences in the incidence of tumor types in different age groups in Japan: while germinoma constituted 70–84% of GCT in patients between the ages of 15 and 35 years, the incidence was much lower before 15 yeras and after 35 years, being 24% of tumors under 4 years and 34% of tumors after 40 years of age. The therapeutic regimen for pineal region tumors should depend on the patient population concerned, because of the differences relating to race and age distribution.     

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。     

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.     

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.     

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.     

应用改良Poppen入路切除儿童松果体区肿瘤的手术体会

目的 探讨直切口小骨窗斜行切开小脑幕技术切除儿童松果体区肿瘤的效果.方法 总结2008年1月至2011年10月环湖医院收治9例应用改进Poppen入路手术的松果体区肿瘤患儿.统计肿瘤全切除率、并发症发牛率和开关颅时间.结果 肿瘤显微镜下全切除率为77.8％,桥静脉损伤率为11.1％,一过性同向性偏盲发生率为22.2％,开关颅时间平均(25.4±4.4) min.全切除病例脑积水缓解率100％,1例取活检后行第三脑室底造瘘术,无脑室-腹腔分流病例,无手术死亡病例.结论 改进Poppen入路为松果体区肿瘤切除手术提供了充足的视野和操作空间,使手术安全性更高,并发症发生率更低.     

Immature teratoma of the pineal gland with isochromosome 12p

An immature teratoma arising in the pineal gland in a 27-year-old male was shown to present an isochromosome 12p as evidenced by cytogenetic and fluorescence in situ hybridization analysis. As i(12p) is characteristic of gonadal germ cell tumors, this case indicates that similar genetic pathways may operate in gonadal and intracranial teratomas. Received: 20 May 1997 / Revised, accepted: 23 June 1997     

松果体区肿瘤的显微外科治疗(附31例报告)

目的研究松果体区肿瘤外科治疗的方法、入路和注意事项。方法回顾分析31例松果体区肿瘤直接手术患者的临床特点、手术及预后情况。31例患者中，枕下经小脑幕入路（Poppen入路）22例，幕下小脑上入路（Krause入路）6例，经胼胝体后部入路（Dandy入路）2例。颞部侧脑室三角部入路1例。结果肿瘤全切除24例，次全切除4例，大部分切除3例。术后死于并发症2例；随访4个月至4年，19例恢复良好，5例生活基本自理，4例术后复发，1例术后1年死亡。结论松果体区肿瘤的手术治疗效果较好，合理的手术入路及体位，娴熟的显微外科手术技巧是手术成功的关键。     

Papillary tumor of the pineal region in a child: case report and review of the literature

Case report A 13-year-old boy presented with a history of intracranial hypertension. Radiologic studies revealed triventricular hydrocephalus secondary to a space-occupying lesion in the pineal region. Endoscopic third ventriculostomy was performed to treat hydrocephalus, and the lesion was biopsied. Complete resection of the tumor was subsequently performed. After a period of uncertainty, the diagnosis of papillary tumor of the pineal region was established. Tumor bed radiotherapy at a dose of 50 Gy was associated. With a follow-up of 15 months, the postoperative course was satisfactory with return to school and no residual tumor on magnetic resonance imaging. Conclusion Papillary tumors of the pineal region constitute a rare entity, with a difficult diagnosis, an uncertain prognosis, and a high risk of local recurrence; they must be treated by a combination of surgery and radiotherapy. The only identified prognostic factor is the complete or incomplete nature of surgical resection.     

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.     

Operative approaches to the pineal region tumors

Until the introduction of microsurgery, the surgical treatment of pineal region tumors had very poor results with high mortality and morbidity. However, there have been remarkable improvements with modern surgical technology and advanced knowledge. Now the main surgical approaches to pineal region tumors are the occipital transtentorial approach and the infratentorial supracerebellar approach. Recently the neuroendoscopic approach has been added. The surgical approach and any supplementary treatment should be selected in each case according to the nature of the individual tumor. The forms of treat-ment practiced by ourselves, including neuroendoscopic surgery, are presented, and the surgical treatments now applied are discussed.     

Pineal region tumours in childhood A 30-year experience

Patients aged under 16 years presenting to the Royal Children's Hospital between 1967 and 1997 with pineal region tumours were retrospectively reviewed. Thirty-seven patients were identified, with 13 germinomas, 7 nongerminomatous germ cell tumours, 6 pineoblastomas, 2 pineocytomas, and 3 astrocytomas, while in 6 patients no histopathological diagnosis was obtained. The most common presentation was with symptoms of raised intracranial pressure due to hydrocephalus. Thirty-two of the 37 patients required a shunt. Thirteen had a biopsy as a separate procedure, 3 of which were stereotactic. Tumour excision was performed in 21 patients and was complete in 4 and subtotal in 17. There were 2 perioperative deaths and 6 patients who were neurologically worse after surgery. Twenty-six patients had radiotherapy and 16 chemotherapy, with significant complications of radiotherapy in half of the patients who received it. The 5-year survival of patients with benign tumours was 75%, 5-year survival with germinomas 62% and with other malignant tumours 14%. This series demonstrates significant improvements in management of pineal region tumours in the last 30 years and highlights some of the current controversies. A collaborative research approach is necessary to determine optimal management of the varied tumour types occurring in the pineal region in childhood. Received: 29 October 1998     

松果体区肿瘤立体定向活检

报告了３５例松果体区肿瘤定向活检使用Ｌｅｋｓｅｌｌ定向仪Ｂａｃｋｌｕｎｄ螺旋活检针病理结果：生殖细胞瘤２３例，星形胶质瘤６例，松果体母细胞瘤３例，囊肿和脑脓肿各１例，阴性１例。主要并发症是出血，发生率１１．４％，其中死亡１例。为了避免活检出血，应在术时用ＤＳＡ检查协助术中穿刺避开血管。另外，尽量使用侧方开口的活检针。