显微手术切除松果体区肿瘤(附34例报告)

目的 总结松果体区肿瘤显微手术切除的经验。方法 回顾34例松果体区肿瘤的临床资料和手术体会。结果 本组患者中生殖细胞瘤12例,畸胎瘤7例,胶质瘤5例,脑膜瘤4例,胆脂瘤、海绵状血管瘤各2例,松果体细胞瘤和异位垂体腺瘤各1例。所有患者均行手术切除肿瘤,采用枕部经小脑幕入路27例,幕下小脑上入路4例,侧脑室三角部入路2例,经胼胝体入路1例。肿瘤全切除28例,次全切除5例,部分切除1例。手术后无住院期内死亡者。结论 显微手术技术的提高显著降低了松果体区肿瘤直接手术切除的死亡率和并发症发生率,我们认为积极的显微外科手术治疗是松果体区肿瘤最佳的一线治疗手段。     

松果体区肿瘤的显微手术及内镜辅助作用

目的 探讨显微手术治疗松果体区肿瘤的手术入路和技术以及内镜的辅助作用.方法 采用显微手术技术通过幕下小脑上入路切除10例松果体区肿瘤,其中内镜辅助手术5例;病理结果：生殖细胞瘤5例、混合性生殖细胞肿瘤3例（生殖细胞瘤合并恶性畸胎瘤1例,合并胚胎癌2例）、脑膜瘤和成熟畸胎瘤各1例;1例术后脑积水行第三脑室底造瘘术,1例术前行第三脑室底造瘘.结果 所有病例全切肿瘤.术后早期结果全部良好,随访1个月～7年良好8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加上内镜的辅助可以取得满意的疗效.     

Intracranial germ cell tumors: pathobiological and immunohistochemical aspects of 70 cases

Seventy cases of histologically verified intracranial germ cell tumor were reviewed: 43 germinomas, 16 immature teratomas, seven mature teratomas, two embryonal carcinomas, one choriocarcinoma, and one yolk sac tumor. The male-to-female ratio was 2.6:1. The average age was 19 years in patients with germinoma, 11 years in patients with immature teratoma, and 17 years in patients with mature teratoma. Duration of symptoms averaged 19 months for germinoma, three months for immature teratoma, and 11 months for mature teratoma. Sixty-six lesions were located in the midline. Fifty-eight percent of the germinomas arose anterior to the pineal gland, whereas 29% of the immature and 14% of the mature teratomas were located anteriorly. The histologic appearance of the germinomas was indistinguishable from that of the usual testicular seminoma. The immature teratomas contained tissue from all three germ layers and exhibited morphologic features of fetal tissue. Of 14 immature teratomas, seven contained, in addition, foci of other malignant germ cell elements; thus, there were two teratocarcinomas, two lesions with germinoma and immature teratoma, two lesions with extensive rhabdomyoblastic differentiation in an immature teratoma, and one lesion with both germinoma and embryonal carcinoma in addition to immature teratoma. The seven mature teratomas consisted of fully differentiated epithelial and mesenchymal tissues. In 23 cases, immunoperoxidase stains for human chorionic gonadotropin (HCG), alpha-fetoprotein (AFP), and carcino-embryonic antigen (CEA) revealed patterns which, with minor exceptions, were essentially identical to those found in genital germ cell lesions. Survival was longest for patients with germinomas. In classifying germ cell tumors of the central nervous system, the World Health Organization's (WHO) classification of testicular germ cell tumors is preferable to its present classification of intracranial germ cell tumors.     

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.     

So-called intracranial germ cell tumours: personal experiences and a theory of their pathogenesis

We investigated 110 cases of intracranial germ cell tumours (up to the end of 1986), 56% of which showed monotypic histological patterns and 44% were shown to be mixed tumours. All these cases underwent surgery followed by radiation and/or chemotherapy. All cases of choriocarcinoma and embryonal carcinoma died within 2 yr: cases of endodermal sinus tumour also showed poor results (4 yr survival rate was 12.5%). Mature teratoma had a 5 yr survival rate (5YSR) and a 10 yr survival rate (10YSR) of 78.3% each: immature teratoma showed a 5YSR of 44.9%. Two-cell pattern tumours (PTC) showed a 5YSR of 85.8% and a 10YSR of 82.4%. They (PTC) can be divided into two groups (i) germinoma and (ii) pinealoma of pineal parenchyma origin (pineocytoma with lymphocytic infiltration) on the basis of the difference in the tumour cell-stroma relationship and also placental alkaline phosphatase stain. In the pineal region, 70% of PTC belonged to the category of pinealoma and in the suprasellar region, 92% of PTC were germinoma. Germinoma showed a 5YSR of 91.4% and a 10YSR of 91.4%, whereas those of the pinealoma were 78.2% and 68.4% respectively. This suggests that the biological characteristics of germinoma and pinealoma may be different. All these results may bring into question the validity of the germ cell theory, since germinoma, which should be the most undifferentiated according to the theory, was the most benign and choriocarcinoma and endodermal sinus tumour, which should be the most differentiated, were the most malignant in the follow-up study.(ABSTRACT TRUNCATED AT 250 WORDS)     

Surgical treatment of pineal and tectal tumors from the subtentorial and supracerebellar approach

The results of operative treatment of pineal and tectal tumours by infratentorial, supracerebellar approach were presented. There were 15 patients with pineal and 2 with tectal tumours. Clinical symptoms of the disease were as follows: signs of intracranial hypertension, tectal lesion, impairment of gait and hypoacusis. Diagnostic procedure included CT, MRI, and panangiography investigation. Application of infratentorial, supracerebellar approach enabled total resection of tumours in 14 patients (82.3%) and subtotal in 2 patients (11.8%) In 1 patient we managed to do only diagnostic biopsy (5.9%). This approach makes possible removal of pineal and tectal tumours growing in the midline toward the anterior part of III ventricle and also posterior fossa. The advantage of this approach is fact that the operation takes place below the vein of Galen. Drooping by gravity cerebellum creates enough places for microsurgical operation. During control investigation 13 patients (76%) had no announce any complaints and were without neurological disorders.     

松果体区肿瘤的影像表现及鉴别诊断

目的 分析松果体区肿瘤的影像特点,以提高诊断及鉴别诊断的准确率.方法 回顾性分析经手术及病理证实的28例松果体区肿瘤的CT和MRI表现,其中8例患者术前行1H-MRS和DWI检查.结果28例松果体区肿瘤分别为生殖细胞瘤11例,畸胎瘤4例,星形细胞瘤3例,室管膜瘤1例,小脑幕缘脑膜瘤3例,松果体细胞瘤2例,松果体母细胞瘤1例,错构瘤1例.表皮样囊肿2例.影像学主要表现为松果体区囊实性肿块,密度/信号多变,部分肿瘤有其特征的形态、密度/信号及强化方式.生殖细胞瘤及松果体细胞肿瘤绝大部分为均匀实性肿块,密度/信号均匀.胶质瘤多为囊实性,且形态多变,密度/信号不均,变化大.成熟性畸胎瘤一般为混杂囊状密度/信号.胶质瘤1H-MRS可见到典型高Cho峰及低NAA峰,脑膜瘤往往测不到NAA峰,松果体母细胞瘤Cho峰较高,无Lip峰,而生殖细胞瘤多出现Lip峰.结论 松果体区肿瘤的形态、密度/信号特征及强化方式有助于松果体区病变的诊断,1H-MRS及DWI对鉴别诊断有帮助,但部分肿瘤鉴别困难.     

松果体区肿瘤的影像表现及鉴别诊断

目的 分析松果体区肿瘤的影像特点,以提高诊断及鉴别诊断的准确率.方法 回顾性分析经手术及病理证实的28例松果体区肿瘤的CT和MRI表现,其中8例患者术前行1H-MRS和DWI检查.结果28例松果体区肿瘤分别为生殖细胞瘤11例,畸胎瘤4例,星形细胞瘤3例,室管膜瘤1例,小脑幕缘脑膜瘤3例,松果体细胞瘤2例,松果体母细胞瘤1例,错构瘤1例.表皮样囊肿2例.影像学主要表现为松果体区囊实性肿块,密度/信号多变,部分肿瘤有其特征的形态、密度/信号及强化方式.生殖细胞瘤及松果体细胞肿瘤绝大部分为均匀实性肿块,密度/信号均匀.胶质瘤多为囊实性,且形态多变,密度/信号不均,变化大.成熟性畸胎瘤一般为混杂囊状密度/信号.胶质瘤1H-MRS可见到典型高Cho峰及低NAA峰,脑膜瘤往往测不到NAA峰,松果体母细胞瘤Cho峰较高,无Lip峰,而生殖细胞瘤多出现Lip峰.结论 松果体区肿瘤的形态、密度/信号特征及强化方式有助于松果体区病变的诊断,1H-MRS及DWI对鉴别诊断有帮助,但部分肿瘤鉴别困难.     

松果体区肿瘤的影像表现及鉴别诊断

目的 分析松果体区肿瘤的影像特点,以提高诊断及鉴别诊断的准确率.方法 回顾性分析经手术及病理证实的28例松果体区肿瘤的CT和MRI表现,其中8例患者术前行1H-MRS和DWI检查.结果28例松果体区肿瘤分别为生殖细胞瘤11例,畸胎瘤4例,星形细胞瘤3例,室管膜瘤1例,小脑幕缘脑膜瘤3例,松果体细胞瘤2例,松果体母细胞瘤1例,错构瘤1例.表皮样囊肿2例.影像学主要表现为松果体区囊实性肿块,密度/信号多变,部分肿瘤有其特征的形态、密度/信号及强化方式.生殖细胞瘤及松果体细胞肿瘤绝大部分为均匀实性肿块,密度/信号均匀.胶质瘤多为囊实性,且形态多变,密度/信号不均,变化大.成熟性畸胎瘤一般为混杂囊状密度/信号.胶质瘤1H-MRS可见到典型高Cho峰及低NAA峰,脑膜瘤往往测不到NAA峰,松果体母细胞瘤Cho峰较高,无Lip峰,而生殖细胞瘤多出现Lip峰.结论 松果体区肿瘤的形态、密度/信号特征及强化方式有助于松果体区病变的诊断,1H-MRS及DWI对鉴别诊断有帮助,但部分肿瘤鉴别困难.     

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。     

Primary intracranial germ cell tumours

Summary A histological study has been made of a retrospective series of 17 primary intracranial germ cell tumours found in a collection of 3550 intracranial neoplasms (incidence of 0.48%). All, except for two differentiated teratomas (one extracerebral in a neonate and another in the lateral ventricle), were situated in the midline in persons aged 5 to 37 years (13 males, 4 females). 12 tumours were located in or originated from the (para)pineal region, two of them also invaded the hypothalamus, while three germinomas occupied the retrochiasmal (supra/intrasellar) region without pineal involvement. There were 11 rather pure tumours (7 germinomas, 4 teratomas of various differentiation) and six mixed neoplasms (2 germinomas with teratoid areas, 3 embryonal carcinomas containing elements of endodermal sinus tumour, choriocarcinoma and germinoma, and one teratocarcinoma with endodermal sinuses). Only one case showed prominent features of endodermal sinus tumour, but characteristic elements of this type were present in four other mixed tumours. All germinomas and germinomatous parts of mixed neoplasms showed an inflammatory reaction of varying intensity, in 6 cases associated with multinucleated giant cells, which may be related to the prognosis of these tumours (one patient with hypothalamic germinoma is alive 6 years after radiotherapy). The close structural similarities between the various types of intracranial and gonadal dysgerminomas and their frequent combination within the same tumour support the concept of a common histogenesis of germ cell tumours regardless of their site of origin. Difficulties of classification may arise from the rather frequent occurrence of mixed germ cell neoplasms.Dedicated to E. Frauchiger, on the occasion of his 70th anniversary.     

An unusual case of primary central nervous system germinoma with meningeal dissemination

Purpose

Central nervous system germinomas often extend or disseminate into the ventricular and subarachnoid space. We present a case of primary central nervous system germinoma consisting mainly of meningeal dissemination, which is extremely unusual.

Case report

A 16-year-old boy presented with pure germinoma, manifesting as anorexia and headache for 10 days. Radiological examinations revealed a large mass lesion in the posterior fossa and a small mass lesion on the pineal lobule with thickening of the tentorium cerebelli and falx. The patient underwent partial removal of the infratentorial tumor via open surgery. Intraoperative findings indicated extension of the dural and supracerebellar mass lesions into the epiarachnoid space. Three chemotherapy courses comprising ifosfamide, etoposide, and cisplatin were administered after the surgery. Craniospinal irradiation was administered subsequently. The adjuvant therapy had a complete response.

Conclusion

Central nervous system germinoma cells may have similar compatibility to meningeal components as they do to cerebrospinal fluid or the ventricular system. If there is a possibility of the tumor extending into the subdural space during surgical intervention or other events, a range of postdiagnostic irradiation should be considered to cover a wider range.     

幕下-小脑上入路切除松果体瘤的并发症

目的探讨经幕下-小脑上入路(Krause's approach)切除松果体瘤的有关并发症.方法总结2个医院近10年来采用经幕下-小脑上入路切除松果体肿瘤病例共24例,归纳其术后出现的并发症.结果与手术有关的主要并发症有:颅内积气5例,硬膜下积液3例,Parinaud综合征3例,大脑后动脉损伤1例,术后脑膜脑室炎3例.结论幕下-小脑上入路手术的并发症较多,手术过程中对大脑各重要结构的保护是预防并发症的关键.     

脑干海绵状血管瘤手术入路选择

目的探讨脑干海绵状血管瘤手术适应证和手术入路的选择。方法回顾分析70例手术治疗的脑干海绵状血管瘤，其中位于：中脑15例、中脑-桥脑交界8例、桥脑34例、桥脑-延髓交界5例、延髓8例。我们共采用8种手术入路：经菱形窝27例、颞下或颞-枕入路14例、远外侧经髁7例、外侧小脑上幕下7例、中线小脑上幕下8例、枕经天幕4例、乳突后2例，以及眶颧1例。结果本组病例的年出血率为2．9％（77／2364），占同期颅内海绵状血管瘤的44％（70／159）；97％的病例手术选择在亚急性或慢性期、且所有患者均有颅神经症状和（或）运动功能障碍、感觉障碍、共济失调（包括失平衡）。中脑病变手术入路选择以中线小脑上（46．7％，7／15）、颞下或颞-枕（26．7％，4／15）为主；桥脑病变多选择菱形窝（58．8％，20／34）、颞下或颞-枕（23．5％，8／34）；而病变位于延髓者以远外侧经髁（62．5％，5／8）和菱形窝入路（37．5％，3／8）为主。结论脑干海绵状血管瘤表现为进行性神经功能缺失、具有占位效应、接近脑干表面者可考虑手术治疗；个体化地选择手术入路、术中神经电生理监测以及直接的电刺激是手术成功的关键。     

Krause入路和Poppen入路对松果体区显露及损伤程度的解剖学对比研究

目的定量比较Krause入路(经幕下小脑上入路)和Poppen入路(枕下经小脑幕入路)对松果体区的显露及损伤情况。方法对6具成年国人头颈部湿性标本,模拟常规Krause入路和Poppen入路的手术操作,每个手术步骤完成后,用Horgan方法计算出显露面积,按Ammirati标准对两种手术入路显露松果体区的程度进行评分比较,同时对两种手术入路的损伤程度进行评分比较。结果Krause入路和Poppen入路的显露程度评分均为2分,显露面积分别为(1298.07±64.67)和(1041.10±50.23)mm2,损伤程度评分分别为(4.5±0.6)和(6.3±0.7)分;两组比较,经t检验,均P0.01。结论Krause入路和Poppen入路均能达到对松果体区的有效显露,但Krause入路显露面积明显大于后者,且损伤程度更小。     

Expansive mass of the posterior region of the 3d ventricle (tumors of the pineal region)

Tumours of the pineal region are rare. Clinical and CT aspects, and results of the treatment of 12 cases observed in a 5 years period (1979-1984) are described. The authors consider the supracerebellar infratentorial approach, formerly described by Krause in 1926 and reintroduced by Stein in 1971, the best for the surgical management of these tumours.     

Clinical experience with a high definition exoscope system for surgery of pineal region lesions

VITOM-90 (Karl Storz Endoscopy, Tuttlingen, Germany) is a new technology that can be used as an alternative to the operating microscope. We have found that this device substantially improves surgeon comfort during infra-tentorial supracerebellar approaches to pineal region masses, and now report our experiences. The VITOM-90 is a specially designed scope that is attached to a high definition (HD) digital camera and displayed on a HD video monitor. This system was utilized in five patients undergoing infratentorial supracerebellar approaches for pineal region lesions. Surgical outcomes and pathologies are described. The device was used by three surgeons during five procedures. Three patients underwent surgery in the sitting position and two in the modified prone (Concorde) position. Pathologies included pineocytoma, lipoma, and germinoma. Total resection was achieved in three patients and subtotal in two patients. Surgeon assessment was positive; surgeons indicated that surgery with the VITOM-90 was more comfortable than with the operating microscope. Lack of stereopsis was considered a minor drawback. The VITOM-90 permitted a natural head and neck position. Operating room personnel and residents reported improved visualization of the anatomy. Using the VITOM-90 benefited surgeons during pineal region surgery by reducing strain and allowing the surgeon to operate from a comfortable position without increased operative time or complications. The improved comfort levels may translate into safer, more accurate surgeries in this complex area.     

Infratentorial supracerebellar resection of a pineal tumor using a high definition video exoscope (VITOM®)

A telescope based high definition (HD) video system (VITOM®, Karl Storz GmBH &; Co., Tuttlingen, Germany) has recently been proposed as an alternative to the operating microscope for microneurosurgery. It remains unclear which clinical situations will benefit from its advantages. In light of the uncomfortable surgeon position and fatigue often associated with pineal region surgery, we used the VITOM® HD exoscope system to perform an infratentorial supracerebellar resection of a pineal tumor. The VITOM® dramatically improved surgeon comfort and ease of operating by permitting the surgeon to stand upright and in a comfortable position and avoid the need to extend the arm or assume an awkward. position commonly encountered when using the microscope for these approaches. The marked improvement in surgeon comfort afforded by the VITOM® exoscope indicates that this system may have significant advantages over traditional microscope based surgery for tumors of the pineal region approached using an infratentorial supracerebellar approach.     

松果体区肿瘤显微手术治疗及入路比较

目的探讨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12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。12例患者中,枕下经小脑幕入路(Poppen入路)7例,经胼胝体-穹窿间入路3例,颞部侧脑室三角部入路1例,幕下小脑上入路(krause入路)1例。结果肿瘤全切除11例。术后死于并发症1例;随访8个月至3.5年,10例恢复良好,1例生活基本自理,1例术后3月死亡。结论松果体区肿瘤的手术治疗效果较好,选择合适的手术入路及体位,熟练的显微外科手术技巧是手术成功的关键。     

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.