Pathological findings in enucleated eyes of patients with neurofibromatosis type 1: report of a case with 15-year follow-up and review of 14 patients in the literature.

BACKGROUNDS: Iris nodules are frequently noted as clinical manifestations of neurofibromatosis type 1 but the other intraocular manifestations are rare. The purpose of this study is to present a patient with a phthisic eye who underwent enucleation for a cosmetic reason after 15-year follow-up and also to review 14 patients with enucleation described in the literature. CASE PRESENTATION: A 17-year-old man with neurofibromatosis type 1 from infancy underwent the enucleation of phthisic left eye and also had the resection of eyelid subcutaneous mass lesions on the left side for a cosmetic reason. He had undergone four-time preceding surgeries for eyelid and orbital mass reduction on the left side in childhood and had developed total retinal detachment 10 years previously. Pathologically, the enucleated eye showed massive retinal gliosis positive for both S-100 and glial fibrillary acidic protein (GFAP) in the area with involvement of the detached retinal neuronal layer, together with a more fibrotic lesion along the choroid which were, in contrast, negative for both S-100 and GFAP. The choroid, ciliary body, and iris did not show apparent neurofibroma while episcleral neurofibroma was present. LITERATURE REVIEW: In review of enucleated eyes of 14 patients in the literature, buphthalmic eyes with early-onset glaucoma on the unilateral side was clinically diagnosed in 9 patients who frequently showed varying extent of hemifacial neurofibromatosis which involved the eyelid and orbit on the same side. Pathologically, neurofibromas in varying extent were found in the choroid of 12 patients. One patient showed choroidal malignant melanoma on the left side and fusiform enlargement of the optic nerve on the right side suspected of optic nerve glioma. The phthisic eye in another patient showed massive retinal gliosis similar to the present patient. CONCLUSIONS: In summary of the 15 patients with neurofibromatosis type 1, including the present patient, buphthalmic or phthisic eyes with no vision were enucleated for cosmetic reasons and showed choroidal neurofibroma in most patients and massive retinal gliosis in two patients including the present patient.

Safety and Efficacy of Combined Injection of Pure-µ-Opioid Agonist with Tramadol as an Opioid Induction Agent for Opioid-Naïve Cancer Patients.

Background: Tramadol is known to provide synergistic analgesia when used in combination with morphine. Objectives: The aims of this study were: (1) to introduce an opioid combination therapy using pure-µ-opioid receptor agonist (OPI) + tramadol injections (OPI + tramadol) and (2) to elucidate safety and efficacy of this combination therapy for opioid-naïve cancer pain patients. Methods: Opioid-naïve patients referred to our palliative care team (in Japan) who were unable to take oral medications and received OPI + tramadol as opioid induction agents were retrospectively investigated on the electric medical chart. OPI + tramadol dosage was adjusted to achieve the patient's pain as Numerical Rating Scale ≤4/10 or Support Team Assessment Schedule-Japanese ≤1. Patients' demography, doses of OPI and tramadol administered, and adverse events were analyzed. Results: A total of 44 patients were included. The primary organs of malignancy were pancreas (11), stomach (5), lung (4), breast (4), liver (4), and others (13). OPI injections administered were hydromorphone (39), morphine (6), oxycodone (1), and fentanyl (1). The starting doses of OPI (morphine equivalent) and tramadol were 6.05 ± 1.63 and 67.8 ± 13.6 mg/day, respectively, and the final doses of OPI (morphine equivalent) and tramadol were 8.14 ± 3.85 and 80.0 ± 28.5 mg/day, respectively. Treatment goals were achieved in all patients. There were three patients in whom OPI was switched owing to inadequate analgesia and no new side effects other than those known to occur when OPI or tramadol is administered appeared. Conclusion: The results suggest that this innovative and unique opioid therapy can be safely and effectively introduced to opioid-naïve cancer patients who are relatively close to the end of life.

Onset of Aortic Dissection Complicated with Cushing's Disease: A Case Report and Review of the Literature.

Cushing's syndrome and Cushing's disease cause various metabolic disorders associated with high cortisol levels. Some reports have shown that Cushing's syndrome is complicated with dissecting aortic aneurysm and aortic dissection after long-term exposure to high cortisol levels. We herein report a rare case of aortic dissection complicated with Cushing's disease. Aortic dissection may occur even under relatively short periods of high cortisol conditions. This case suggests that hypercortisolemia should be treated as soon as possible in order to prevent aortic dissection in subjects with Cushing's disease.

A Case of Ruptured Cerebral Aneurysm with Asymmetric Fusion of Basilar Apex.

Objective: We report a case of a ruptured cerebral aneurysm in which a bifurcation pattern at the tip of the basilar artery was asymmetric fusion type and the superior cerebellar artery (SCA) branched from the posterior cerebral artery (PCA) on the caudal fusion type side. Case Presentation: A 45-year-old woman presented with a subarachnoid hemorrhage with a headache. Cerebral angiography revealed that the right SCA diverged from the PCA and a small cerebral aneurysm had developed at this site. This cerebral aneurysm was successfully treated by coil embolization. Conclusion: There have been no previous reports on cerebral aneurysms at the site of this normal variation.

C-reactive protein, symptoms and activity of daily living in patients with advanced cancer receiving palliative care.

BACKGROUND: The association between C-reactive protein (CRP) level, symptoms, and activities of daily living (ADL) in advanced cancer patients is unclear. METHODS: Secondary data analysis of a multicenter prospective cohort study consisted of 2426 advanced cancer patients referred to palliative care settings was conducted to examine the cross-sectional relationships between CRP level, symptoms, and ADL disabilities. Laboratory data, symptoms, ADL, and manual muscle testing (MMT) results were obtained at baseline. Participants were divided into four groups: low (CRP < 1 mg/dl), moderate (1 = < CRP <5 mg/dl), high (5 = < CRP < 10 mg/dl), and very high CRP (10 mg/dl = < CRP). The proportions of eight symptoms, five ADL disabilities, and three categories of MMT according to the CRP groups were tested by chi-square tests. Multiple-adjusted odd ratios (ORs) were calculated by using ordinal logistic regression after adjustment for age, gender, site of primary cancer, metastatic disease, performance status, chemotherapy, and setting of care. RESULTS: A total of 1702 patients were analysed. Positive rates of symptoms and ADL disabilities increased with increasing CRP level. In the very high-CRP group, rates of positivity for anorexia, fatigue, and weight loss were 89.8%, 81.0%, and 79.2%, respectively, and over 70% of patients received assistance for bathing, dressing, going to the toilet, and transfer. The grade of MMT also deteriorated with increasing CRP level. Adjusted ORs for the accumulated symptoms significantly increased with increasing CRP level in the moderate-CRP, high-CRP, and very high-CRP groups [1.6 (95% confidence interval 1.2-2.0), P < 0.001; 2.5 (1.9-3.2), P < 0.001; 3.5 (2.7-4.6), P < 0.001, respectively]. Adjusted ORs for the accumulated ADL disabilities significantly increased in the very high-CRP groups [2.1 (1.5-2.9), P < 0.001]. CONCLUSIONS: Associations between CRP level, symptoms, and ADL were observed in advanced cancer patients receiving palliative care.

A prospective, multicenter cohort study to validate a simple performance status-based survival prediction system for oncologists.

BACKGROUND: Survival prediction systems such as the Palliative Prognostic Index (PPI), which includes the Palliative Performance Scale (PPS), are used to estimate survival for terminally ill patients. Oncologists are, however, less familiar with the PPS in comparison with the Eastern Cooperative Oncology Group (ECOG) performance status (PS). This study was designed to validate a simple survival prediction system for oncologists, the Performance Status-Based Palliative Prognostic Index (PS-PPI), which is a modified form of the PPI based on the ECOG PS. METHODS: This multicenter, prospective cohort study enrolled all consecutive patients who were referred to 58 palliative care services in Japan. The primary responsible physicians rated the variables required to calculate the PS-PPI and the PPI. Patient survival in these risk groups was compared, and the sensitivity and specificity of the PS-PPI and the PPI were evaluated. Patients were subclassified as patients receiving care from in-hospital palliative care teams, palliative care units, or home-based palliative care services. Subsets of patients receiving chemotherapy were also analyzed. RESULTS: This study included 2346 patients. Survival predictions based on the PPI and the PS-PPI differed significantly among the 3 risk groups (P < .001). The PS-PPI was more sensitive, whereas the PPI was more specific. All areas under the receiver operating characteristic curves of both indices were >0.78 for predicting survival at all times, from 3 weeks to 180 days. CONCLUSIONS: In predicting the prognosis of patients with advanced cancer, the PS-PPI was as accurate as the PPI. The PS-PPI was useful for short- and long-term survival prediction and for the prediction of survival for patients undergoing chemotherapy. Cancer 2017;123:1442-1452. © 2016 American Cancer Society.

A novel quinone derived from 5-hydroxyindoleacetic acid reacts with protein: Possible participation of oxidation of serotonin and its metabolite in the development of atherosclerosis.

The modification of 5-hydroxyindoleacetic acid (5HIAA) by myeloperoxidase with a xanthine oxidase system was investigated by chromatographic analyses. Two major products were identified as a dimer and quinone (indoleacetate dione) of 5HIAA. The formation of a quinone moiety was also confirmed by chemical trapping with o-phenylenediamine. In the presence of N-acetyl-cysteine (NAC), a quinone-NAC adduct was formed. When glyceraldehyde 3-phosphate dehydrogenase was exposed to the myeloperoxidase system with 5HIAA, quinone adducts were formed on the protein molecule. A monoclonal antibody was prepared using a quinone-modified protein as an immunogen to immunochemically detect the quinone on a protein. The established antibody recognized the quinone-NAC adduct, quinone-modified poly-L-lysine, and quinone-modified low-density lipoprotein. Quinone-modified proteins in human atherosclerotic lesions were immunohistochemically observed using the established antibody to the quinone and also a monoclonal antibody to tryptamine dione-modified protein, suggesting an occurrence of in vivo oxidation of serotonin and 5HIAA, accompanied by covalent adduction to biomolecules.

Impact of industrial structure and soil exposure on the regional variations in pulmonary nontuberculous mycobacterial disease prevalence.

OBJECTIVE/BACKGROUND: The prevalence of pulmonary nontuberculous mycobacterial (pNTM) disease, including Mycobacterium avium complex (MAC), varies widely according to geographic region. However, the factors that influence regional variations in pNTM disease prevalence remain unknown. This study was undertaken to examine whether environmental or occupational factors or host traits could influence regional variations in pNTM disease prevalence. METHODS: We collected laboratory data on pulmonary tuberculosis (pTB) and pNTM from two hospitals in the West Harima area of Japan and five hospitals in Kyoto City, Japan from 2012 to 2013. We estimated microbiological pNTM disease prevalence by multiplying all pTB cases in each area with the ratio of pNTM cases and pTB cases at the survey hospitals in each area. We administered a standardized questionnaire to 52 patients and 120 patients with pulmonary MAC (pMAC) disease at Ako City Hospital and Kyoto University Hospital, respectively. RESULTS: The estimated prevalence of microbiological pNTM disease in the West Harima area (85.4/100,000 population-years) was significantly higher than that observed in Kyoto City (23.6/100,000 population-years; p<.001). According to multiple logistic regression analysis, in Ako City Hospital, primary (activities directly related to natural resources) and secondary industries (construction, mining, and manufacturing primary industry produce; odds ratio [OR]=4.79; 95% confidence interval [CI]=1.49-14.0; p=.007) and soil exposure (OR=13.6; 95% CI=4.94-45.26; p<.001) were associated with pMAC disease. CONCLUSION: Environmental factors, both industrial structures associated with occupational dust and environmental soil exposure, could influence the regional variations in pNTM disease prevalence.

Hybrid Microscopic-Endoscopic Surgery for Craniopharyngioma in Neurosurgical Suite: Technical Notes.

OBJECTIVE: The best chance of curing craniopharyngioma is achieved by microsurgical total resection; however, its location adjacent to critical structures hinders complete resection without neurologic deterioration. Unrecognized residual tumor within microscopic blind spots might result in tumor recurrences. To improve outcomes, new techniques are necessary to visualize tissue within these blind spots. We examined the success of hybrid microscopic-endoscopic neurosurgery for craniopharyngioma in a neurosurgical suite. METHODS: Four children with craniopharyngiomas underwent microscopic resection. When the neurosurgeon was confident that most of the visible tumor was removed but was suspicious of residual tumor within the blind spot, he or she used an integrated endoscope-holder system to inspect and remove any residual tumor. Two ceiling monitors were mounted side by side in front of the surgeon to display both microscopic and endoscopic views and to view both monitors simultaneously. RESULTS: Surgery was performed in all patients via the frontobasal interhemispheric approach. Residual tumors were observed in the sella (2 patients), on the ventral surface of the chiasm and optic nerve (1 patient), and in the third ventricle (1 patient) and were resected to achieve total resection. Postoperatively, visual function was improved in 2 patients and none exhibited deterioration related to the surgery. CONCLUSIONS: Simultaneous microscopic and endoscopic observation with the use of dual monitors in a neurosurgical suite was ergonomically optimal for the surgeon to perform microsurgical procedures and to avoid traumatizing surrounding vessels or neural tissues. Hybrid microscopic-endoscopic neurosurgery may contribute to safe, less-invasive, and maximal resection to achieve better prognosis in children with craniopharyngioma.

The Accuracy of Physicians' Clinical Predictions of Survival in Patients With Advanced Cancer.

CONTEXT: Accurate prognoses are needed for patients with advanced cancer. OBJECTIVES: To evaluate the accuracy of physicians' clinical predictions of survival (CPS) and assess the relationship between CPS and actual survival (AS) in patients with advanced cancer in palliative care units, hospital palliative care teams, and home palliative care services, as well as those receiving chemotherapy. METHODS: This was a multicenter prospective cohort study conducted in 58 palliative care service centers in Japan. The palliative care physicians evaluated patients on the first day of admission and followed up all patients to their death or six months after enrollment. We evaluated the accuracy of CPS and assessed the relationship between CPS and AS in the four groups. RESULTS: We obtained a total of 2036 patients: 470, 764, 404, and 398 in hospital palliative care teams, palliative care units, home palliative care services, and chemotherapy, respectively. The proportion of accurate CPS (0.67-1.33 times AS) was 35% (95% CI 33-37%) in the total sample and ranged from 32% to 39% in each setting. While the proportion of patients living longer than CPS (pessimistic CPS) was 20% (95% CI 18-22%) in the total sample, ranging from 15% to 23% in each setting, the proportion of patients living shorter than CPS (optimistic CPS) was 45% (95% CI 43-47%) in the total sample, ranging from 43% to 49% in each setting. CONCLUSION: Physicians tend to overestimate when predicting survival in all palliative care patients, including those receiving chemotherapy.

Independent validation of the modified prognosis palliative care study predictor models in three palliative care settings.

CONTEXT: Accurate prognostic information in palliative care settings is needed for patients to make decisions and set goals and priorities. The Prognosis Palliative Care Study (PiPS) predictor models were presented in 2011, but have not yet been fully validated by other research teams. OBJECTIVES: The primary aim of this study is to examine the accuracy and to validate the modified PiPS (using physician-proxy ratings of mental status instead of patient interviews) in three palliative care settings, namely palliative care units, hospital-based palliative care teams, and home-based palliative care services. METHODS: This multicenter prospective cohort study was conducted in 58 palliative care services including 16 palliative care units, 19 hospital-based palliative care teams, and 23 home-based palliative care services in Japan from September 2012 through April 2014. RESULTS: A total of 2426 subjects were recruited. For reasons including lack of followup and missing variables (primarily blood examination data), we obtained analyzable data from 2212 and 1257 patients for the modified PiPS-A and PiPS-B, respectively. In all palliative care settings, both the modified PiPS-A and PiPS-B identified three risk groups with different survival rates (P<0.001). The absolute agreement ranged from 56% to 60% in the PiPS-A model and 60% to 62% in the PiPS-B model. CONCLUSION: The modified PiPS was successfully validated and can be useful in palliative care units, hospital-based palliative care teams, and home-based palliative care services.

Covalent modification of cytoskeletal proteins in neuronal cells by tryptamine-4,5-dione.

Serotonin, 5-hydroxytryptamine, is a systemic bioactive amine that acts in the gut and brain. As a substrate of myeloperoxidase in vitro, serotonin is oxidized to tryptamine-4,5-dione (TD), which is highly reactive with thiols. In this work, we successively prepared a monoclonal antibody to quinone-modified proteins and found that the antibody preferentially recognizes the TD-thiol adduct. Using the antibody, we observed that the chloride ion, the predominant physiological substrate for myeloperoxidase in vivo, is not competitive toward the enzyme catalyzed serotonin oxidation process, suggesting that serotonin is a plausible physiological substrate for the enzyme in vivo. Immunocytochemical analyses revealed that TD staining was observed in the cytosol of SH-SY5Y neuroblastoma cells while blot analyses showed that some cellular proteins were preferentially modified. Pull-down analyses confirmed that the cytoskeletal proteins tubulins, vimentin, and neurofilament-L were modified. When pure tubulins were exposed to micromolar levels of synthetic TD, self-polymerization was initially enhanced and then suppressed. These results suggest that serotonin oxidation by myeloperoxidase or the action of other oxidants could cause functional alteration of cellular proteins, which may be related to neurodegeneration processes or irritable bowel syndrome.

[Construction and usefulness of the dose management system using DICOM radiation dose structured report (Dose SR) in angiography].

The International Commission on Radiological Protection recommends diagnostic reference levels (DRL) in each radiological examination for justification and optimization of patients' dose in medicine. The aim of our study was to propose the dose management system by utilizing dose information in diagnostic X-ray radiation dose structured report (Dose SR) in The Digital Imaging and Communications in Medicine to optimize radiation dose in institutions. Our dose management system is able to organize dose information obtained from various angiography systems and CTs. It is possible to provide this information to operators for justification and optimization of patient dose. Our system would be useful for the estimation of organ dose and could be used for the determination of local DRL (LDRL) for each radiological practice. In addition, the optimization became possible to compare LDRL with national DRL.

Assessment of a quick reference table algorithm for determining initial postoperative pressure settings of programmable pressure valves in patients with idiopathic normal pressure hydrocephalus: SINPHONI subanalysis.

BACKGROUND: Management of overdrainage complications in shunted patients with idiopathic normal pressure hydrocephalus (INPH) remains a difficult task despite the use of programmable pressure valves. OBJECTIVE: To assess the usefulness of a quick reference table (QRT) algorithm for achieving a suitable initial programmable pressure valve setting in INPH patients who participated in the Study for INPH on Neurological Improvement (SINPHONI). METHODS: One hundred registered patients diagnosed with probable INPH were treated with ventriculoperitoneal shunts using Codman-Hakim programmable valves (CHPVs). In this series, the initial CHPV setting was decided prospectively according to the QRT algorithm. Shunt effectiveness, complications, and the number of CHPV readjustments during follow-up periods were investigated. RESULTS: Eighty patients were considered better than shunt responders (more than 1 point improvement in modified Rankin scale at any follow-up period). Readjustments of CHPVs within 3 months after treatment with ventriculoperitoneal shunt were performed 56 times in 44 cases (44%, 0.56 times/patient). Low-pressure headache occurred in 9 patients, all of whom improved by readjustment alone. Nontraumatic subdural fluid collections and chronic subdural hematomas occurred in 15 cases (15%); however, most of the cases were subclinical and improved after CHPV readjustments alone. Burr hole irrigation was necessary in only 1 case. CONCLUSION: Use of the QRT algorithm was associated with a decrease in postoperative CHPV readjustments and serious overdrainage complications during the follow-up period. The QRT algorithm is an easy, safe, and effective method for determining the initial CHPV pressure setting in INPH patients.

The Effects of Percutaneous Endoscopic Gastrostomy on Quality of Life in Patients With Dementia.

BACKGROUND: To examine the effects of percutaneous endoscopic gastrostomy (PEG) on quality of life (QOL) in patients with dementia. METHODS: We retrospectively included 53 Japanese community and tertiary hospitals to investigate the relationship between the newly developed PEG and consecutive dementia patients with swallowing difficulty between Jan 1st 2006 and Dec 31st 2008. We set improvements in 1) the level of independent living, 2) pneumonia, 3) peroral intake as outcome measures of QOL and explored the factors associated with these improvements. RESULTS: Till October 31st 2010, 1,353 patients with Alzheimer's dementia (33.1%), vascular dementia (61.7%), dementia with Lewy body disease (2.0%), Pick disease (0.6%) and others were followed-up for a median of 847 days (mean 805 ± 542 days). A total of 509 deaths were observed (mortality 59%) in full-followed patients. After multivariate adjustments, improvement in the level of independent living was observed in milder dementia, or those who can live independently with someone, compared with advanced dementia, characterized by those who need care by someone: Odds Ratio (OR), 3.90, 95% confidence interval (95%CI), 1.59 - 9.39, P = 0.003. Similarly, improvement of peroral intake was noticed in milder dementia: OR, 2.69, 95%CI, 1.17 - 6.17, P = 0.02. Such significant associations were not observed in improvement of pneumonia. CONCLUSIONS: These results suggest that improvement of QOL after PEG insertion may be expected more in milder dementia than in advanced dementia.

[Usefulness of endovascular treatment for delayed massive epistaxis following endoscopic endonasal transsphenoidal surgery: a case report].

We report here a case of massive nasal bleeding from the sphenopalatine artery three weeks after endonasal transsphenoidal surgery. This 66-year-old male suffered from massive nasal bleeding with the status of hypovolemic shock. Under general anesthesia, an emergent angiography revealed an extravasation from the sphenopalatine artery. Trans-arterial embolization using coil and n-butyl-cyanoacrylate (NBCA) was performed following the diagnostic angiography. Complete occlusion of the injured artery was achieved. The patient showed good recovery from general anesthesia. Delayed nasal bleeding after endonasal transsphenoidal surgery is a rare but important complication. The sphenopalatine artery and its branch are located in the hidden inferior lateral corner of the sphenoid sinus and may be injured during enlargement of the sphenoid opening. When massive delayed nasal bleeding follows transsphenoidal surgery and damage of the internal carotid artery has been ruled out, endovascular treatment of the external carotid artery should be considered.

Guideline for management and treatment of fetal and congenital hydrocephalus: Center Of Excellence-Fetal and Congenital Hydrocephalus Top 10 Japan Guideline 2011.

INTRODUCTION: Hydrocephalus does not indicate a single clinical entity, but includes a variety of clinicopathological conditions caused by excessive cerebrospinal fluid (CSF) based on the disturbed circulation. Recent progress in prenatal neuroimagings such as MRI and ultrasound echoencephalography on fetus enables to understand clinicopathological conditions of CSF circulation disorder in conjunction with morphological changes in the central nervous system properly. It has been revealed that the CSF dynamics develop in the theory of evolution from the immature brain, as in the animals with the minor CSF pathway predominance, towards matured adult human brain together with the completion of the major CSF pathway: the "Evolution Theory in CSF Dynamics". Now, we can analyze CSF circulation dynamically and also analyze the flow velocity and direction of CSF movement. CENTER OF EXCELLENCE-FETAL HYDROCEPHALUS TOP 10 JAPAN: Along with this technical improvement, the standards of clinicopathological evaluation of hydrocephalus as well as the classification and concept of hydrocephalus shall undergo a major upgrade. Based on such remarkable improvement in the recent practical diagnostic evaluation of fetal hydrocephalus, it is now required to update the guideline for management and treatment of fetal and congenital hydrocephalus, and a nationwide study group; Center of Excellence-Fetal Hydrocephalus Top 10 Japan, was organized in 2008 in Japan. The retrospective analysis of 333 cases of congenital hydrocephalus indicated a fact that 43% of these cases were diagnosed prenatally, and the majority of cases were treated in these top 10 institutes in Japan. Now, congenital hydrocephalus diagnosed immediately after birth is regarded as to be based on embryonic stage; brain disorder in patients with congenital hydrocephalus should be considered in conjunction with neuronal mature process of embryonic stage. The fact is supported by the current trends in hydrocephalus research represented by "Perspective Classification of Congenital Hydrocephalus" and "Multi-categorical Hydrocephalus Classification". The ultimate goal of hydrocephalus treatment remains achieving arrested hydrocephalus by shunt surgeries. In the future, to achieve arrested hydrocephalus, minimum quantity of CSF to be drained should be elucidated. Consideration for accurate operative indication of ETV along with new neuroendoscopic device development and analysis of CSF circulation is expected in the future. The data in this prospective multicenter analysis in this guideline are credited in Oxford Evidence level 2b (Grade II).

Nationwide investigation of the current status of therapeutic neuroendoscopy for ventricular and paraventricular tumors in Japan.

OBJECT: The authors report their investigation on the current status of neuroendoscopic biopsy for ventricular and paraventricular tumors as well as treatment for associated hydrocephalus in Japan. METHODS: Patients who had undergone therapeutic neuroendoscopy between 2005 and 2009 were included in this study. The main items examined were age; sex; localization of tumor; pathological diagnosis using biopsy; the presence, treatment, and efficacy of treatment of associated hydrocephalus; perioperative complications; activities of daily living (ADL) before and after therapeutic neuroendoscopy; and the presence of dissemination during the postoperative course. RESULTS: Seven hundred fourteen patients from 123 sites (462 male and 252 female patients, mean age 33.3 years) were enrolled. Localization of the tumor was mainly classified into the lateral ventricle in 91 patients, the third ventricle in 339, the fourth ventricle in 18, the suprasellar region in 75, and other paraventricular areas in 191 patients. The most commonly observed tumors were germ cell tumors in the third ventricle (177 cases [39%]), cystic lesions in the suprasellar region (56 cases [75%]), and astrocytic tumors in the thalamus-basal ganglia (71 cases [38%]). Although 641 (92.8%) of 691 patients could receive neuroendoscopic diagnosis using biopsy, the diagnosis obtained with endoscopic biopsy differed from the final diagnosis based on subsequent craniotomy in 18 patients and clinical course in 3 patients. Of these 21 patients, 7 had astrocytic tumors, 4 had pineal tumors, 6 had germ cell tumors, and 4 had other tumors. The final diagnostic accuracy rate was 89.7%. Associated hydrocephalus was observed in 517 patients (72.4%), of whom 316 and 39 underwent third ventriculostomy and fenestration of the septum, respectively. The response rates were 96.2% and 89.7%, respectively. Third ventriculostomy was required for recurrence of hydrocephalus in 41 patients (13.0%), and the long-term response rate was therefore 83.2% (263 of 316 patients). Perioperative complications other than fever, such as new onset of or progressive hydrocephalus, infection due to CSF leakage, and bleeding in the ventricle or tumor, were found in 81 patients (11.3%). The median Karnofsky Performance Scale score before endoscopic surgery was 80, but it increased to 90 after surgery. The score was thus significantly increased after surgery (p < 0.0001, Mann-Whitney U-test). Activities of daily living after surgery decreased due to perioperative complications in 15 patients (2.1%). The incidence of new dissemination after endoscopic biopsy was 6.8% and not high compared with routine surgical treatment. CONCLUSIONS: The authors concluded that neuroendoscopic diagnosis using biopsy for ventricular and paraventricular tumors is adequately accurate and safe. It was demonstrated that endoscopic procedures play important roles not only in the treatment of hydrocephalus associated with intra- and paraventricular tumors but also in significantly improving ADL. Furthermore, the long-term outcome of endoscopic third ventriculostomy was clearly favorable.