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Object: Analysis of the safety and morbidity of neuroendoscopic biopsies (NEBs), as well as the reliability in obtaining an accurate diagnosis, has until now been based on studies with relatively small sample sizes. Through the cooperative efforts of several international medical centers, authors of the present study collected data on a large number of patients to obtain better insight into this issue. When possible, they compared pathology obtained through an NEB with the "gold-standard" pathology obtained in open surgery., Methods: Thirteen randomly chosen medical centers in 9 countries collected data for patients who had undergone NEB, which were then analyzed for universal complications, bleeding, navigation technique, pathology, mismatch between biopsy results and final diagnosis, and a number of other potentially influential factors., Results: Data for 293 patients were analyzed. Sixty percent of the patients were male, and patient ages ranged from 0.1 to 78.7 years (median age 20.4 years). The most common tumor locations were pineal (33.1%), thalamic (16.7%), tectal (13%), and hypothalamic (4.4%). Fifty percent of the tumors were larger than 20 mm, 36% were between 10 and 20 mm, and 14% were smaller than 10 mm. Intraoperative bleeding was seen in 275 patients (94%). The amount of blood was noted as mild in 75%, moderate in 13%, and severe in 6%. Infection occurred in 8 patients (3%). Death occurred in 1 patient (0.3%), which was caused by severe intraoperative bleeding. Biopsies were informative in 265 patients (90.4%). Seventy-eight patients (26.6%) had open surgery following the NEB. For these patients, the pathology results from the NEB were compared with those from the open surgery that followed. In 14 cases (17.9%) there was disagreement on the pathology. Of these cases, a meaningful mismatch, in which the erroneous NEB pathology could have led to an inappropriate management decision, occurred in 9 cases (11.5%). Most of these meaningful mismatches were lesions diagnosed as low-grade or pilocytic astrocytoma on the NEB and later proved to be high-grade astrocytoma (4 cases) and 1 case each of meningioma, cavernoma, primitive neuroectodermal tumor, neurocysticercosis, and pineocytoma., Conclusions: In experienced hands, NEBs can be performed with low morbidity and mortality, providing meaningful pathological data for the majority of patients with a wide range of tumor types, locations, and presentations. These biopsies also offer other advantages, such as the ability to perform concomitant endoscopic third ventriculostomy and septum pellucidotomy. However, due caution must be maintained, since pathology obtained from an NEB, as with stereotactic biopsies, may be subject to sampling errors, especially when the results seem to indicate a low-grade glial tumor.

NY-ESO-1, one of the most immunogenic cancer/testis antigens, provides attractive targets for cancer immunotherapy. NY-ESO-1 has been demonstrated to be expressed in a range of solid tumors via DNA demethylation and/or histone modification; however, it has been rarely expressed in glioma. The reversibility of these epigenetic aberrations is potentially attractive for glioma treatment with DNA-methyltransferase inhibitors (DNMTi) and histone deacetylase inhibitors (HDACi), leading to reactivation of silenced genes. We previously demonstrated de novo induction of NY-ESO-1 in glioma cells by DNMTi. In this study, we show that an anticonvulsant, i.e., valproic acid (VPA), also acting as an HDACi, enhances induction of NY-ESO-1 in synergy with DNMTi. Chromatin assays demonstrated that combination of DNMTi and VPA elicited significant DNA demethylation, histone H3 Lys9 demethylation, and acetylation. These findings not only shed light on an epigenetic immunotherapy, but also suggest that the silencing of NY-ESO-1 is mediated by histone modification.

Rationale: Suprasellar tumors are common in the pediatric population and exhibit a wide range of pathological appearance and behavior. Although rare, pure suprasellar germinomas carry worse prognosis than other locations., Methods: We present a case of a 10-year-old girl treated with therapeutic anticoagulation for previous cardiac valvular replacement, who presented with rapidly growing giant (>4 cm) suprasellar germinoma. Neoadjuvant chemotheraphy was effective and the dilated ventricles became slit-like. The fine free-hand maneuver using a fine rigid-rod neuroendoscope. "Oi-Samii Handy Pro" made it possible to obtain the tissue diagnosis., Results: Complete remission was achieved using neoadjuvant chemotherapy, minimally invasive neuroendoscopic surgery, then followed by specific chemoradiotherapy., Conclusion: The role of neuroendoscopic procedure, performed in a difficult situation with normalized size of the ventricles, was emphasized.

Introduction: Congenital brain tumours are a rare entity that is nowadays often already recognised during pregnancy by ultrasound (US) and magnetic resonance (MR). Even though the definitive diagnosis is usually achieved by means of histological studies, in some cases the diagnosis may remain uncertain because of the malformative origin of this type of tumour., Case Report: We describe a patient with a diffuse congenital intracranial mass diagnosed by intrauterine US and foetal MR performed to further evaluate the lesion and the associated foetal hydrocephalus. After delivery by caesarean section, an endoscopic biopsy was performed. Several specimens were obtained and resulted in the diagnosis of primitive neuroectodermal tumour (PNET). Despite polychemotherapy, the tumour continued to enlarge and the patient died at 2 months of age. Post-mortem histological examination of the intracranial mass showed a diffuse intracranial teratoma., Conclusion: Endoscopic biopsy specimen examination resulted in a diagnosis that was not confirmed by post-mortem findings. The scarce differentiation of teratoma components and their high variability in histomorphology as well as the huge size of the tumoral mass may limit the interpretation of endoscopic biopsy specimens, even when multiple and obtained from different areas.

Background: Considering the separate benefits of neuronavigation and neuroendoscopy, neuroendoscopic surgery with the aid of neuronavigation systems will play an increasingly important role in the future. Bearing this in mind, the present research project was conducted to facilitate neuronavigational neuroendoscopic surgery along the pathway to the prepontine cistern using cadaver heads., Materials and Methods: A computer-aided, frameless image-guided stereotactic navigation system and a new type of handy rigid-rod neuroendoscope were used. The ideal entry point and the safest trajectory to the prepontine cistern through the foramen of Monro were defined in two formalin-fixed cadaver heads and clinical brain MRI data. Then, maneuvering of the neuroendoscope with the aid of the neuronavigation system was performed., Results: Straight trajectories from the entry point to the prepontine cistern could be designed. For the registration accuracy of the tip of the neuroendoscope, the virtual image registered a mean error distance of 5.42 mm away from the reference point along the axis of vertical line. However, free-hand maneuvering enabled the neuroendoscope to be finely manipulated without damaging brain tissues. Neuroendoscopic anatomical views of the interpeduncular and prepontine cistern were also acquired., Conclusion: Interactive use of free-hand maneuvering of the handy rigid-rod neuroendoscope together with frameless neuronavigation systems plot the way to true neuronavigational neuroendoscopic surgery in a safe and reliable manner. This pairing of the most recent technological neurosurgical options with better understanding of neuroendoscopic anatomy enables the neurosurgeon to acquire broader treatment options for central nervous system diseases.

Congenital intracranial tumors are extremely rare and account only for 0.5%-1.5% of brain tumors in children. We report a large intrauterine congenital teratoma in a female fetus at gestation weeks 37, which was diagnosed by detecting the tumor and associated craniomegaly with ultrasonography (US) and magnetic resonance (MR) imaging. The tumor had replaced the cerebral hemispheres and produced prenatal manifestations. Pathologic examination showed an immature teratoma, which was differentiated from all three germ layers. Microarray analysis revealed upregulation of ten genes and downregulation of three genes, as well as upregulation of 41 genes of ribosomal proteins in teratoma cells, compared to normal brain tissue of the patient. The data from the microarray analysis offer not only the potential to help define disease pathogenesis but may also provide clues to identify potential molecular therapeutic targets.

The therapeutic modalities for pineal region tumors in Western countries differ from those in far-eastern countries, that is, Japan and Korea, mainly because of the different patient populations. The majority of pineal region tumors in Japan and Korea are radio sensitive and/or chemosensitive, and adjuvant therapy rather than extensive surgery plays the main part in the treatment of these tumors. The authors have applied minimally-invasive preferential management in pineal region tumors in last 8 years. For the therapeutic regimen, if the tumor markers alpha-fetoprotein (AFP) and human chorionic gonadotropin (HCG) were not detected in serum and there was significant ventricular dilation visualized on neuroimages, neuroendoscopic surgery was first applied for tumor debulking with tissue diagnosis and gross morphological analysis of the tumor and the intraventricular structures, followed by third-ventriculostomy. In the results, our minimally-invasive preferential regimen clarified the precise indication for neuroendoscopic procedures, and the majority of our patients with dilated ventricles and no evidence of tumor markers were treated satisfactorily with effective neuroendoscopic procedures as the initial procedure. Then avoided unnecessary craniotomy and radiotherapy and promised excellent therapeutic outcomes. Neuroendoscopic procedures have a great advantage in the management of chemo- or radiosensitive tumors, such as germinoma, pineoblastoma, or primitive neuroectodermal tumor. The neuroendoscopic anatomy including the lateral and third ventricles with a pineal region tumor with or without tumor dissemination was described in detail, together with the neuroendoscopic surgical technique.

Object: This prospective study is based on a consecutive series of 20 patients with pineal region tumors who underwent minimally invasive preferential management. The purpose of this report is to discuss the role of neuroendoscopic procedures in the management of pineal region tumors., Methods: If the tumor markers alpha-fetoprotein and human chorionic gonadotropin were not detected in serum and there was significant ventricular dilation visualized on neuroimages, neuroendoscopic surgery was first applied for tumor debulking with tissue diagnosis and gross morphological analysis of the tumor and the intraventricular structures, followed by third ventriculostomy. Subsequent procedures were determined on the basis of verified individual tumors. For treatment of germinomas and pineoblastomas, if no tumor dissemination was confirmed by pre-, intra-, or postoperative findings, stereotactic radiotherapy or radiosurgery was performed after one course of chemotherapy with the ICE regimen (isofomid, cisplatin, and etoposide) and followed by two additional courses of chemotherapy. For treatment of malignant germ cell tumors, after extensive surgery, adjuvant chemotherapy with the ICE regimen was performed in three courses in all cases. Then radiotherapy was started using various methods, depending on the evidence of tumor dissemination. For treatment of teratomatous and neuroectodermal tumors other than pineoblastomas, extensive surgical removal was performed. As for adjuvant therapy, if the tumor was a low-grade glioma or if the patient was younger than 5 years of age, postoperative treatment did not include radiotherapy. If the tumor was a malignant teratoma or high-grade glioma, conventional focal radiotherapy was performed, followed by chemotherapy with ICE for 1 year. All but two treated patients had ventriculomegaly. Neuroendoscopic procedures were performed in six of 15 treated patients. Neuroendoscopic biopsy with tumor debulking offered enough material for tissue diagnosis, including immunohistochemical analysis and, in one case, revealed evidence of tumor dissemination undetectable on neuroimaging. With one exception, no shunt was required in any patient undergoing endoscopic third ventriculostomy. Stereotactic radiotherapy was performed in indicated cases. Favorable therapeutic outcomes were obtained in all cases of germinoma and pineoblastoma, with follow-up periods ranging from 24 months to 6.5 years., Conclusions: Our minimally invasive preferential regimen clarified the precise indication for neuroendoscopic procedures, and the majority of our patients with dilated ventricles and no evidence of tumor markers were treated satisfactorily with effective neuroendoscopic procedures as the initial procedure, avoiding unnecessary craniotomy and radiotherapy and promising excellent therapeutic outcomes. The treatment for malignant pineal region tumors remains a subject for further study.

The therapeutic modalities used for tumors of the pineal region in Western countries differ from those in Japan, mainly because of the different patient populations. An extensive survey was conducted to delineate the racial differences in Japan and in Korea in the epidemiology and recent therapeutic modalities for this tumor group. Among the members of International Society for Pediatric Neurosurgery (ISPN), 15 from Japan and 5 from Korea reported their recent findings in 118 (1-25 years of age, mean 7.38-year period) and 125 (1-12 years of age, mean 6.69-year period) histologically verified cases, respectively. The patient populations in the two countries were found to be almost identical, with an extremely high incidence of germ cell tumors representing 71.2% (in Japan) and 80.0% (in Korea) of all pineal region tumors and neuroectodermal tumors representing only 15.2% and 16.8%, respectively. The most common type of pineal region tumor was germinoma (46.6% in Japan and 47.2% in Korea). The majority of tumors were radio- and/or chemosensitive, and adjuvant therapy rather than extensive surgery played the major role in the treatment in both countries. Radical resection of the tumor was recommended as the initial procedure by only 22.2% of neurosurgeons in Japan and 16.6% in Korea. Biopsy was recommended by 38.9% and 50.0%, and radiation therapy by 38.9% and 37.5%, respectively. A minimally invasive procedure, by either a neuroendoscopic (33.3% of biopsies in Japan) or a stereotactic approach (33.3% of biopsies in Korea), was considered to be appropriate as the initial procedure. The study disclosed the almost identical epidemiology of this brain tumor in Japan and in Korea and clarified the consequent therapeutic modalities. The authors emphasize that minimally invasive tissue diagnosis with or without tumor debulking should be considered as the initial step for the treatment planning of the pineal region tumor, followed by the most commonly indicated major procedures, including radiation therapy, chemotherapy and/or radical resection with various methodologies.

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, pineoblastoma, 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 years 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.

The authors describe a case of congenital intracranial teratoma. The patient was diagnosed to be hydrocephalic at 29 weeks' gestation, and to have a huge intraventricular mass lesion at 34 weeks' gestation. Subsequently, the patient underwent subtotal resection of the mass, resulting in a significant decrease in the remarkably elevated alpha-fetoprotein in serum and cerebrospinal fluid. Histological analysis revealed a malignant teratoma, also with alpha-fetoprotein-positive elements in the immunohistochemical study.

During 1987, 23 survey forms for ependymoma cases diagnosed in the 1st year of life were selected from 88 neurosurgical institutes in Japan in collaboration with the International Society for Pediatric Neurosurgery. There were no specific perinatal episodes. The most common clinical manifestations were symptoms of intracranial hypertension and hydrocephalus. Two of seven children with infratentorial ependymomas were alive, and all of the children with such supratentorial tumors were still alive. Of those with ependymoma, three of four in the total extirpation group, and three of seven in the subtotal or partial removal group were alive. The 1-year survival rate was 54% for ependymoma and 58.3% for ependymoblastoma. For 36% of the cases with ependymoma and 14.6% with ependymoblastoma, a 2-year survival rate was observed. Since the prognosis of these tumors is poor, they should be extirpated as often as possible. Collins' law held true in approximately half of the cases.

The authors present specific clinical features of brain tumors occurring in immature brain in comparison with those of older infants and children. Twenty-six neonatal brain tumors, which accounted for 11.3% of 231 brain tumors diagnosed in the 1st year of life collected in a cooperative study in Japan, were analyzed. Although astrocytomas were invariably common tumors in each age group, comprising 25.0% for all pediatric brain tumors, teratomas were the most common type of neonatal brain tumors and accounted for 33.4% in this age group. Location of tumors in the immature brain was more striking, involving the posterior fossa region less commonly, as in 11.1% in neonatal, 29.5% in infantile, and 41.4% in all pediatric age groups. Surgical intervention was performed in 97.4% of the infantile and 79.1% of the neonatal cases, but total or subtotal resection of the mass was achieved only in 58.9% in the infants and 73.7% in the neonates. There was more strict limitation in performing the adjuvant therapies in the immature age groups. Radiation therapy and chemotherapy were given only in 32.0% and 27.6%, respectively, in the neonatal cases. The prognosis was extremely poor in the immature cases: 1-year, 5-year, and 10-year survival rates were 59.3%, 26.1%, 11.5% in the infants, and 43.5%, 23.8%, 7.1% in the neonates, respectively. Of the neonates, 76% and of the infants 73.1% were retarded. The present study delineates the characteristic clinical features of tumors involving immature brain and emphasized that establishment of more specific therapeutic modalities is urgent.

The nature and sequence of the radiation-induced changes in the brain were studied postmortem in 34 children with glioma, 22 of whom underwent central nervous system radiation therapy. Twenty received whole-brain or whole-neuroaxis radiation at a total mean dosage of 4063 cGy. Brain tissue alterations were analyzed histologically by means of various staining methods, including immunohistochemical techniques. The histological features of irradiated brains were compared with those of non-irradiated brains. Microscopic findings included demyelination (seven cases), focal necrosis (six cases), cortical atrophy (four cases), endothelial proliferation (four cases), and telangiectatic vascular proliferation with vascular thickening and oozing of a thick fluid (one case). Such findings were rare in non-irradiated patients. Demyelination was observed earliest in a patient who died 5 months after radiation therapy and was more common after 9 months. Focal necrosis was first observed 9 months post-irradiation but was more advanced and extensive after 1 year. Calcified foci were found only after 60 months. Various vascular changes such as vascular thickening and thrombosis suggested ischemic insult to the brain as a late effect of radiation injury. The results of this study suggest that the immature brain may be more sensitive to radiation than is the adult brain, and that the manifestations of radiation-induced injury depend on the time elapsed after irradiation.

Results of Computerized Axial Tomography with and without intravenous contrast (60% Conray) in 24 patients with multiple brain tumors were analyzed. Of the 24 patients, 19 had metastatic brain tumors, 4 multiple gliomas and 1 patient had multiple meningiomas. Data obtained by C.T. scan were compared to results obtained by angiography and nuclear scanning. In the diagnosis of multiple metastatic brain tumors. C.T. scans were superior to the other two procedures. however, C.T. scanning detected 4 lesions in the case of multiple meningiomas, while angiography disclosed 11 tumors in the same patient. In one patient having multiple gliomas, one (vascular) lesion only was demonstrated by angiography. Many other evaluations of Computerized Axial Tomography have been reported and the advent of this unique non-invasive radiologic technique has resulted in marked changes in the neuro-radiological diagnostic approach to brain tumors. However, it is important to recognize the weak as well as the strong points of each diagnostic procedure including the C.T. scan. At present, in our opinion, even with a positive demonstration of a brain tumor by C.T.scanning, cerebal angiography should be carried out prior to surgical intervention.

We have experienced with 50 cases of parasellar tumors, four cases of which had persistent thirst, polydipsia, polyuria, and reversible temporary hyponatremia secondary to hypernatriuresis. The mechanism of the syndrome in these four cases could not be explained by either that of the syndrome of hypernatremia or of the so-called SIADH. We tentatively named this syndrome as "CEREBRAL POLYURIC HYPONATREMIA" and the criteria of this syndrome as as follows: 1) persistent thirst, polydipsia polyuria, 2) reversible temporary hyponatremia secondary to hypernatriuresis, 3) exception of the following items--administration of uretic drugs, renal and adrenal dysfunction, hyperglycemia, hyperlipemia, overadministration of water, and poor administration of NaCl. The mechanism of this syndrome is presumed as follows: 1) compression by a tumor or surgical attack to the anterior hypothalamus, 2) disturbance of the mechanism of ADH secret-on, 3) extrasecretion of natriuretic factor possibly produced in the anterior hypthalamus, and 4) preservation of the thirst center.

Brain tumors in infants and children are different from those in adults in type and location of tumor as well as accompanying complications. Given this fact, and the fact that these patients are under development, careful consideration is required for determination of treatment planning. Thus, we have investigated the curative results of brain tumors in infants and children and factors affecting prognosis. In this study, astrocytoma which is most frequently seen in infants and children was analyzed in 181 patients. One hundred forty three of them were diagnosed as benign astrocytoma; and the other 38, as malignant astrocytoma or glioblastoma. In respect of location, supratentorial tumors were seen in 73 patients, while subtentorial tumors occurred in 61; nine tumors infiltrated into both regions. The 5-year survival rate of benign astrocytoma was 50%, compared with 6.3% in malignant astrocytoma. As for relations to location of tumor, 66% of tumors which infiltrated into the supratentorial and subtentorial regions were malignant, and the prognosis was poor: the 5-year survival rate was 14.2%. The 5 year survival rate of subtentorial tumors was 38.9%, whereas that of supratentorial tumors was 57.6%. Analysis by age revealed that malignancy increased in proportion to age over 12 months after birth; between the ages of one and six years, the incidence of malignant tumors was 9% and the 5-year survival rate was 48%, while between the ages of 12 and 16, the rates were 29% and 14%, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)