Clinical and molecular features of patients with IDH1 wild-type primary glioblastoma presenting unexpected short-term survival after gross total resection.

BACKGROUND: This study investigated the factors influencing short-term survivors (STS) after gross total resection (GTR) in patients with IDH1 wild-type primary glioblastoma. METHODS: We analyzed five independent cohorts who underwent GTR, including 83 patients from Kitasato University (K-cohort), and four validation cohorts of 148 patients from co-investigators (V-cohort), 66 patients from the Kansai Molecular Diagnosis Network for the Central Nervous System tumors, 109 patients from the Cancer Genome Atlas, and 40 patients from the Glioma Longitudinal AnalySiS. The study defined STS as those who had an overall survival ≤ 12 months after GTR with subsequent radiation therapy, and concurrent and adjuvant temozolomide (TMZ). RESULTS: The study included 446 patients with glioblastoma. All cohorts experienced unexpected STS after GTR, with a range of 15.0-23.9% of the cases. Molecular profiling revealed no significant difference in major genetic alterations between the STS and non-STS groups, including MGMT, TERT, EGFR, PTEN, and CDKN2A. Clinically, the STS group had a higher incidence of non-local recurrence early in their treatment course, with 60.0% of non-local recurrence in the K-cohort and 43.5% in the V-cohort. CONCLUSIONS: The study revealed that unexpected STS after GTR in patients with glioblastoma is not uncommon and such tumors tend to present early non-local recurrence. Interestingly, we did not find any significant genetic alterations in the STS group, indicating that such major alterations are characteristics of GB rather than being reliable predictors for recurrence patterns or development of unexpected STS.

Frontotemporal craniotomy with skin incision along the superior temporal line outside the hairline in bald male patients with temporal gliomas.

A head skin incision is inevitable in neurosurgical procedures and is usually concealed within the hairline. Androgenetic alopecia (AGA) is a progressive hair loss disorder or baldness highly prevalent in men. Therefore, if bald male patients require neurosurgical procedures, skin incisions cannot be concealed, but this subject is yet to be discussed in the literature. This study presents a frontotemporal craniotomy using a skin incision along the superior temporal line, ignoring the hairline in bald male patients. Thirty-three patients with temporal gliomas underwent surgical removal between 2015 and 2022. They were divided into three groups: bald male patients with skin incisions not concealed in the hairline (minimum group, n = 13), bald and non-bald male patients with skin incisions concealed in the hairline (male group, n = 11), and female patients with skin incisions concealed in the hairline (female group, n = 9). In the minimum group, patients had no complaints regarding the incision scar. Cosmetic outcome was excellent, and no cases showed surgical site infection or peripheral facial nerve palsy. Compared with the male and female groups, the minimum group had the shortest skin incision length; however, the craniotomy size and extent of resection were similar. Skin incision for frontotemporal craniotomy cannot be hidden in bald male patients, and the preferred location for the incision is unknown. The skin incision along the superior temporal line is a cosmetically favorable, feasible, and safe procedure.

Frontotemporal Craniotomy for Clipping of Unruptured Aneurysm Using a Diamond-Coated Thread Wire Saw and Reconstruction Using Calcium Phosphate Cement without Metal Fixation.

Metal fixation systems for cranial bone flaps cut by a drill are convenient devices for cranioplasty, but cause several complications. We use modified craniotomy using a fine diamond-coated threadwire saw (diamond T-saw) to reduce the bone defect, and osteoplasty calcium phosphate cement without metal fixation. We report our outcomes and tips of this method. A total of 78 consecutive patients underwent elective frontotemporal craniotomy for clipping of unruptured intracranial aneurysms between 2015 and 2019. The follow-up periods ranged from 13 to 66 months. The bone fixation state was evaluated by bone computed tomography (CT) and three-dimensional CT (3D-CT). The diamond T-saw could minimize the bone defect. Only one wound infection occurred within 1 week postoperatively, and no late infection. No pain, palpable/cosmetically noticeable displacement of the bone flap, fluid accumulations, or other complications were observed. The condition of bone fixation and the cosmetic efficacy were thoroughly satisfactory for all patients, and bone CT and 3D-CT demonstrated that good bone fusion. No complication typical of metal fixation occurred. Our method is technically easy and safety, and achieved good mid-term bone flap fixation in the mid-term course, so has potential for bone fixation without the use of metal plates.

Endoscope-integrated indocyanine green video angiography and the detection of the fragile periventricular collaterals associated with moyamoya disease: illustrative cases.

BACKGROUND: Hemorrhagic moyamoya disease (MMD) and the fragile periventricular collaterals are known to have a causal relationship. Digital subtraction angiography and magnetic resonance angiography have shown the presence of fragile periventricular moyamoya vessels. However, dynamic fragile periventricular moyamoya vessels have never been observed under direct vision. OBSERVATIONS: The authors treated two patients with hemorrhagic MMD: a 42-year-old man with intraventricular hemorrhage and a 47-year-old woman with intracerebral hemorrhage. Endoscope-integrated indocyanine green video angiography (EICG angiography) could visualize the dynamic fragile periventricular collaterals. In particular, EICG angiography enabled visualization of invisible moyamoya vessels buried in the subependyma and characterization of the blood flow in the moyamoya vessels located inside the lateral ventricles and hematoma cavity. LESSONS: EICG angiography can confirm the fragile periventricular collaterals associated with MMD by direct visualization. The high spatial resolution and real-time imaging can help to avoid accidental hemorrhage in and after evacuation of hemorrhage in patients with MMD.

Ventricular opening and cerebrospinal fluid circulation accelerate the biodegradation process of carmustine wafers suggesting their immunomodulation potential in the human brain.

PURPOSE: Opening the ventricular system during glioblastoma surgery is often necessary, but the consequent effect on the tumor microenvironment of glioblastoma remains unknown. Implantation of carmustine wafer enables direct drug delivery to the tumor site; however, the exact mechanism of the wafer's biodegradation process is unclear, and the available data is limited to in vivo non-human mammalian studies. We hypothesized that the ventricular opening affects the degradation process of the wafer and the glioblastoma tumor microenvironment. METHODS: This study included 30 glioblastoma patients. 21 patients underwent carmustine wafer implantation during initial surgery. All patients underwent repeated surgical resection upon recurrence, allowing for pathological comparison of changes associated with wafer implantation. Immunohistochemical analyses were performed using CD68, TMEM119, CD163, IBA1, BIN1, and CD31 antibodies to highlight microglia, macrophages, and tumor vascularity, and the quantitative scoring results were correlated with clinical, molecular, and surgical variables, including the effect of the ventricular opening. RESULTS: The carmustine wafer implanted group presented significantly less TMEM119-positive microglia within the tumor (P = 0.0002). Simple and multiple regression analyses revealed that the decrease in TMEM119-positive microglia was correlated with longer intervals between surgeries and opened ventricular systems. No correlation was observed between age, methylated O6-methylguanine DNA methyltransferase promoter expression, and the extent of surgical resection. CONCLUSIONS: Our study findings strongly suggest that biomaterials may possess immunomodulation capacity, which is significantly impacted by the ventricular opening procedure. Furthermore, our data highlights the pathophysiological effects of the ventricular opening within the surrounding human brain, especially after the wafer implantation.

Molecular analyses of rosette-forming glioneuronal tumor of the midbrain tegmentum: A report of two cases and a review of the FGFR1 status in unusual tumor locations.

Background: Rosette-forming glioneuronal tumor (RGNT) is a rare tumor that arises primarily in the posterior fossa, with molecular features of FGFR1 mutation. A previous study reported that brainstem RGNT accounts for only 2.7% cases; therefore, midbrain RGNT is infrequent. Case Description: The authors encountered two cases of RGNT located in the midbrain tegmentum (Case 1: 23-year-old woman and Case 2: 18-year-old boy), both exhibiting similar cystic components with gadolinium-enhanced cyst walls on preoperative magnetic resonance imaging, surgically resected through the occipital transtentorial approach. Histological findings in both cases comprised two characteristic architectures of neurocytic and glial components, typical of RGNT. Molecular assessment revealed no FGFR1 mutation in the initial specimen, but revealed FGFR1 K656E mutation in the recurrent specimen in Case 1 and showed no FGFR1 mutation but showed TERT C228T mutation in Case 2. Neither case revealed IDH1/2, BRAF, H3F3A K27, H3F3A G34, or HIST1H3B K27 mutations. DNA methylation-based classification (molecularneuropathology.org) categorized both cases as RGNT, whose calibrated scores were 0.99 and 0.47 in Cases 1 and 2, respectively. Conclusion: Midbrain tegmentum RGNTs exhibited typical histological features but varied FGFR1 statuses with TERT mutation. RGNT in rare locations may carry different molecular alterations than those in other common locations, such as the posterior fossa.

Leber's hereditary optic neuropathy with diffuse white matter changes mimicking gliomatosis cerebri: illustrative case.

BACKGROUIND: Leber's hereditary optic neuropathy (LHON) is a mitochondrial disease characterized by bilateral severe subacute central vision loss and a mutation in the mitochondrial DNA (mtDNA). The findings on cranial magnetic resonance imaging of patients with LHON vary from subtle to multiple white matter changes. However, they rarely present with diffuse infiltrative white matter changes. OBSERVATIONS: The authors reported a case with diffuse white matter changes mimicking gliomatosis cerebri (GC). The histological findings included only mild glial hyperplasia without immunohistochemical positivity, supporting the diagnosis of glial tumors. Analysis of mtDNA obtained from the blood and brain tissue revealed mutation of m.11778G>A in the NADH dehydrogenase 4 gene, which confirmed the case as LHON. Immunohistochemistry of the brain tissue revealed 8-hydroxy-2'-deoxyguanosine positivity, suggesting the presence of oxidative stress. LESSONS: LHON is extremely difficult to diagnose unless one suspects or knows the disease. The present case brings attention not only to LHON but also to other mtDNA-mutated diseases that need to be considered with diffuse white matter changes or GC.

[Percutaneous vertebroplasty for vertebral compression fracture].

BACKGROUND: Percutaneous vertebroplasty has become an option for the treatment of painful osteoporotic compression fractures in patients in whom conservative medical management has failed. AIM: This paper assessed the effectiveness and safety of percutaneous vertebroplasty in patients with focal pain caused by compression vertebral body fractures. MATERIALS AND METHODS: Over a twenty-five-month period 63 patients underwent percutaneous vertebroplasty, and ten of these patients were subsequently retreated, for a total of 73 operations on 93 vertebrae. The patients were affected by osteoporotic compression fractures (n=57) or by benign or malignant infiltrative processes (n=6). All patients were examined at discharge and thereafter to assess the level of pain and investigate possible changes in the quality of life. The mean length of follow-up was 15.2 months. RESULTS: After treatment, almost 90% of patients reported complete disappearance or significant alleviation of the pain. In 7 of 63 of the patient (11%) there were small asymptomatic leakages of cement outside the vertebral body. A substantial number of patients with osteoporosis, 19% of the study population, experienced new fractures following treatment with vertebroplasty. DISCUSSION: Our experience confirms the effectiveness of vertebroplasty to pain caused by vertebral fractures. If the indications are strictly followed, improvement of symptoms is often immediate, allowing the return of mobility, and patient satisfaction with surgery is higher. The use of appropriate systems limits the number of complications.

Beyond conservatism and the boundaries of a medical discipline: a short history of the department of neurosurgery at kyoto university graduate school of medicine.

CONSTANT CHANGE AND the occasional fusion of two different entities can result in the creation of masterpieces, not only in art but also in neurosurgery. Chisato Araki is one of the pioneers of neurosurgery in Japan; his 2-year sojourn in the United States and Europe provided him with an extraordinary amount of experience. He traveled throughout the world at a time when it took 30 days to journey from Yokohama to New York, and he visited with most of the leading contemporary neurosurgeons and observed their operations, never abandoning his highly honed critical insights. Driven by passion and a deep sense of duty to pass on his knowledge and perspective, he became a beacon of hope and encouragement for young physicians working in a country devastated by war. His successor, Hajime Handa, established neurosurgery as one of the branches of neuroscience and fostered the collaborations and exchanges among different disciplines that have become a tradition and hallmark of our Department of Neurosurgery. Through anecdotes and glimpses of the evolution of neurosurgery at our institution, we offer insights into the unique nature of Japanese neurosurgery that may illuminate the path toward the resolution of some of the recent and enduring problems encountered in our specialty.