Phase II Trial of Pathology-based Tripartite Treatment Stratification for Patients with CNS Germ Cell Tumors: A Long-term Follow-up Study.

Background: A previous Phase II clinical trial, conducted from 1995 to 2003, evaluated CNS germ cell tumors (GCTs) using a three-group treatment stratification based on histopathology. The primary objective of the study was to assess the long-term efficacy of standardized treatment regimens, while the secondary objective focused on identifying associated long-term complications.

Methods: Total 228 patients were classified into three groups for treatment: germinoma (n=161), intermediate prognosis (n=38), and poor prognosis (n=28), excluding one mature teratoma case.

Adenoid glioblastoma: Stromal hypovascularity and secretion of chondromodulin-I by tumor cells.

The case of a 75-year-old man with a glioblastoma of the right frontal lobe showing features of adenoid glioblastoma is reported. The tumor consisted of two components: the adenoid component, in which large, cohesive, polygonal cells with vesicular nuclei and abundant basophilic cytoplasm showed nest-like, trabecular, or tubular growth on the myxoid matrix and formed a multinodular configuration; and the subsidiary component, in which short spindle cells showed compact fascicular growth. The features of ordinary glioblastoma were also found in a small area.

[A case of young onset cerebral amyloid angiopathy associated with dural grafting].

A 47-year-old man was admitted to our hospital because of sudden-onset motor aphasia and right hemiplegia. His past medical history was notable for left craniotomy and hematoma evacuation following a traumatic brain hemorrhage approximately 40 years earlier, for which dural grafting was performed. He also had a history of three lobar hemorrhages in the left hemisphere since the age of 42 years.

H3 K27-altered diffuse midline glioma of the thalamus with formation of glio-fibrillary globular structures.

A case of diffuse midline glioma (DMG), H3 K27-altered, that arose in the right thalamus of a 14-year-old boy is reported. The patient died of tumor spread after a progressive clinical course of approximately 13 months. Histopathologically, the tumor consisted of a mixture of loose proliferation of stellate cells and compact fascicular growth of spindle cells showing a "piloid" feature.

Giant cell glioblastoma with lipogenic differentiation in a patient with neurofibromatosis type 1: A case report.

A 45-year-old woman with neurofibromatosis type 1 (NF1) developed a tumor in the left frontal lobe that showed features of giant cell glioblastoma (GC-GB). In addition to the typical GC-GB features, the tumor showed lipogenic differentiation, with many atypical lipoblasts and mature adipocytes. Tumor cells, including the lipogenic cells, were immunoreactive for GFAP, S-100 protein, ATRX, and p53.

5-Aminolevulinic acid fluorescence-guided endoscopic surgery for deep-seated intraparenchymal tumors.

Aim: The usefulness of 5-aminolevulinic acid (5-ALA) fluorescence-assisted surgery for maximum resection of malignant gliomas has been established. However, its usefulness when combined with endoscopic surgery for deep-seated tumors has not been well established. In this study, whether 5-ALA photodynamic diagnosis (PDD) is feasible and useful for endoscopic surgery was investigated.

Distinct patterns of copy number alterations may predict poor outcome in central nervous system germ cell tumors.

We have previously reported that 12p gain may predict the presence of malignant components and poor prognosis for CNS germ cell tumor (GCT). Recently, 3p25.3 gain was identified as an independent predictor of poor prognosis for testicular GCT.

Survey of medications for myelomeningocele patients over their lifetime in Japan.

Background: This study aimed to investigate medication prescriptions for patients with myelomeningocele (MMC) across different age groups, particularly in adulthood and after middle age.

Methods: The Japan Medical Data Center (JMDC) database, based on medical claims data, was utilized for this analysis. Patients were divided into 10-year age groups, and prescriptions for analgesics, anticonvulsants, psychotropic drugs, lifestyle disease-related drugs, drugs for urinary incontinence, and laxatives were examined.

Survival and process outgrowth of human iPSC-derived cells expressing Purkinje cell markers in a mouse model for spinocerebellar degenerative disease.

Purkinje cells are the sole output neurons of the cerebellar cortex and play central roles in the integration of cerebellum-related motor coordination and memory. The loss or dysfunction of Purkinje cells due to cerebellar atrophy leads to severe ataxia. Here we used in vivo transplantation to examine the function of human iPS cell-derived cerebellar progenitors in adult transgenic mice in which Purkinje-specific cell death occurs due to cytotoxicity of polyglutamines.

Refractory Delayed Pneumocephalus after Transsphenoidal Cyst Drainage for Rathke's Cleft Cyst in a Patient with a Cerebrospinal Fluid Shunt.

A 75-year-old man presented with bilateral lower limb weakness to our hospital from another clinic. Radiological examinations implied the possibilities of idiopathic normal pressure hydrocephalus (iNPH) and a suprasellar cyst, but both were observed conservatively at that time. Due to the progressive gait disturbance, a lumboperitoneal shunt was implanted 1 year later.

A case of acute hydrocephalus due to a giant prolactinoma rescued by transventricular neuroendoscopic tumorectomy.

Background: A giant prolactinoma extending to the suprasellar area and causing hydrocephalus may be life-threatening and should be treated promptly. A case of a giant prolactinoma with acute hydrocephalus that underwent transventricular neuroendoscopic tumor resection followed by cabergoline administration is presented.

Case Description: A 21-year-old man had a headache lasting for about a month.

Comparison of intraoperative neurophysiological monitoring methods for lumbosacral lipoma surgery in infants.

Background: Stable intraoperative neurophysiology monitoring of infants, especially very young infants, is challenging. In this study, motor evoked potentials (MEPs), the bulbocavernosus reflex (BCR), and somatosensory evoked potentials (SEPs) were simultaneously monitored in infants with lumbosacral lipomas, and these methods were compared retrospectively.

Methods: A total of 21 surgeries performed for lumbosacral lipoma in patients less than 1 year old were studied.

The effect of shunt removal on the quality of life in patients with congenital hydrocephalus.

Background: Although there have been reports investigating the quality of life of patients who underwent ventriculoperitoneal shunting or endoscopic third ventriculostomy (ETV) for congenital hydrocephalus, there have been no studies of the quality of life of patients after ventriculoperitoneal shunt (VPS) removal. In the present study, a survey of pediatric and congenital hydrocephalus patients was conducted to compare the quality of life of patients with a remaining VPS with that of patients who had the shunt removed.

Methods: Between February 2020 and November 2021, an outpatient survey was administered to patients 8 years of age and older who had undergone VPS due to a diagnosis of congenital hydrocephalus.

Randomized phase III study of high-dose methotrexate and whole-brain radiotherapy with/without temozolomide for newly diagnosed primary CNS lymphoma: JCOG1114C.

Background: The goal was to determine whether the addition of temozolomide (TMZ) to the standard treatment of high-dose methotrexate (HD-MTX) and whole-brain radiotherapy (WBRT) for primary central nervous system lymphoma (PCNSL) improves survival.

Methods: An open-label, randomized, phase III trial was conducted in Japan, enrolling immunocompetent patients aged 20-70 years with histologically confirmed, newly diagnosed PCNSL. After administration of HD-MTX, patients were randomly assigned to receive WBRT (30 Gy) ± 10 Gy boost (arm A) or WBRT ± boost with concomitant and maintenance TMZ for 2 years (arm B).

5-Aminolevulinic acid fluorescence-guided endoscopic surgery for intraventricular tumors.

Background: In recent years, the efficacy of 5-aminolevulinic acid photodynamic diagnosis (5-ALA PDD) has been reported for various types of brain tumors, including malignant glioma. In addition, many reports have been published on the usefulness of neuroendoscopic surgery for intraventricular lesions. However, no systematic report is available on the combined use of 5-ALA PDD and neuroendoscopy for various intraventricular tumors.

Primary central nervous system lymphoma of the tectal plate in adult.

Background: Primary central nervous system lymphoma (PCNSL) originating in the brainstem is uncommon. In particular, PCNSL confined to the tectal plate in adults has never been reported in the past. The case of a 53-year-old man who was diagnosed with PCNSL in the tectal plate is reported.

Current status and challenges of neurosurgical procedures for patients with myelomeningocele in real-world Japan.

Background: Little is known about the real-world status of neurosurgical treatment of myelomeningocele patients.

Objective: To investigate the real-world status of neurosurgical treatment of myelomeningocele patients, medical claims data provided by the Japan Medical Data Center (JMDC) were analyzed.

Methods: The health claims data of 556 patients with myelomeningoceles from January 2005 to March 2020 were examined.

5-ALA fluorescence-guided resection of pediatric low-grade glioma using the ORBEYE 3D digital exoscope: a technical report.

Objective: A case of low-grade glioma in which 5-aminolevulinic acid (5-ALA) fluorescence was visualized by a digital exoscope is presented.

Case Presentation: A 14-year-old girl with recurrent paroxysmal episodes of a strange smell and nausea underwent magnetic resonance imaging (MRI) for further investigation. The MRI showed a tumor with an enhanced nodule in the right temporal lobe.

Newborn with severe supratentorial subdural hematoma due to laceration of the tentorium cerebelli.

Purpose: A rare case of a newborn girl born by a normal vaginal delivery who developed a severe supratentorial subdural hematoma due to a laceration in the tentorium cerebelli is presented.

Methods And Results: The girl, born by normal vaginal delivery at 39 weeks and 4 days of gestation, showed an intermittent decrease in oxygen saturation and bulging of the anterior fontanelle. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a subdural hematoma centered in the left occipital region with a midline shift.

Treatment of refractory giant suprasellar arachnoid cyst by endoscopic expansion of a fenestrated stoma.

Background: Although endoscopic ventriculo-cysto-cisternostomy is considered to be effective for suprasellar arachnoid cysts, we encountered a giant suprasellar arachnoid cyst that recurred despite surgery using this technique.

Case Description: The patient was a 9-month-old boy. Magnetic resonance imaging revealed a huge suprasellar arachnoid cyst extending from the suprasellar region to the anterior skull base and both middle cranial fossa.

Abusive Head Trauma in Infants and Children in Japan.

Subdural hematoma in infants can be caused by abuse, and is thought to be more likely if subdural hematoma is associated with retinal hemorrhage and cerebral edema. In Japan, few doctors disagree that cases of subdural hematoma with retinal hemorrhage and cerebral edema with multiple findings on the body are more likely to have been caused by abuse rather than by household accident. On the other hand, in cases where there are no other significant physical findings, only subdural hematoma and retinal hemorrhage, there is a difference of opinion as to whether the injury was caused by an accident or abuse.

Number of surgeries performed during the lifetime of patients with myelomeningocele.

Objective: Patients with myelomeningocele often require multiple surgeries, but no study has clarified the kind of treatment given to these patients throughout their lives. The authors analyzed the type of surgery that was performed and at what age for Japanese patients with myelomeningoceles.

Methods: The Japanese health claims data of 556 patients with myelomeningocele for the period from January 2005 to March 2020 provided by the Japan Medical Data Center Co.

Transcriptome and methylome analysis of CNS germ cell tumor finds its cell-of-origin in embryogenesis and reveals shared similarities with testicular counterparts.

Background: CNS germ cell tumors (GCTs) predominantly develop in pediatric and young adult patients with variable responses to surgery, radiation, and chemotherapy. This study aimed to examine the complex and largely unknown pathogenesis of CNS GCTs.

Methods: We used a combined transcriptomic and methylomic approach in 84 cases and conducted an integrative analysis of the normal cells undergoing embryogenesis and testicular GCTs.

12p gain is predominantly observed in non-germinomatous germ cell tumors and identifies an unfavorable subgroup of central nervous system germ cell tumors.

Background: Central nervous system (CNS) germ cell tumors (GCTs) are neoplasms predominantly arising in pediatric and young adult populations. While germinomas generally respond to chemotherapy and radiation, non-germinomatous GCTs (NGGCTs) require more intensive treatment. This study aimed to determine whether 12p gain could predict the prognosis of CNS GCTs.