A novel needle electrode for intraoperative fourth cranial nerve neurophysiological mapping.

OBJECTIVES: Trochlear nerve (CN-IV) mapping method has not been confirmed to date. The compound muscle action potential (CMAP) of CN-IV cannot be recorded because of the low mapping sensitivity and anatomical characteristics of the superior oblique muscle (SOM). The aim of this study was to evaluate the effectiveness of a novel needle electrode (NNE), for the intraoperative mapping of CN-IV. MATERIALS AND METHODS: The NNEs were inserted in the target extraocular muscles in 19 patients. We compared the CMAP amplitude of the NNE with that of the conventional needle electrode (CNE). Furthermore, we investigated the dissimilarity between the CMAP of the CN-IV and other extraocular cranial nerves (ECNs) and the correlation between the readings of the CN-IV mapping and its postoperative functional outcome. RESULTS: The CMAP of CN-IV has been measured in nine patients (47.4%). The CMAP of CN-IV was distinguishable from other ECNs. The CMAP of the NNE was found to be three times higher than that of the CNE. Although the NNE has shown the potential to record the CN-IV's CMAP, 4 cases ended up having a CN-IV postoperative dysfunction. CONCLUSIONS: For the first time, we confirmed the possibility of intraoperative mapping the CN-IV using an NNE inserted into the SOM. The NNE can also be useful for other neurophysiological monitoring methods.

Secondary brain tumors after cranial radiation therapy: A single-institution study.

AIM: To study the probability of developing secondary brain tumors after cranial radiotherapy.Background Patients treated with cranial radiotherapy are at risk for developing secondary brain tumors. PATIENTS AND METHODS: We planned an institutional survey for secondary brain tumors in survivors after cranial irradiation and reviewed the 30-year duration data. Event analysis and cumulative proportion curves were performed to generally estimate the cumulative proportion of developing secondary brain tumors, cavernoma and meningioma at different periods of time. RESULTS: Secondary brain tumors occurred in 21% of cases: 10% were cavernomas, 6% were meningiomas, 3% were skull osteomas, and 1% were anaplastic astrocytoma. The cumulative proportion of developing secondary brain tumor was 6% at 10 years and 20% at 20 years, while the cumulative proportion for developing cavernomas and meningiomas was 16% and 7% at 20 years, respectively. CONCLUSION: Our study shows that patients who received cranial irradiation were at risk of secondary brain tumors such as cavernomas and meningiomas. Thus, a meticulous follow-up of cancer survivors with history of cranial irradiation by an annual MRI scan is justifiable. This will help clinicians to detect secondary brain tumors early and make its management much easier.

Bioinformatic Analysis of Gene Expression Related to Sialic Acid Biosynthesis in Patients With Medulloblastoma.

Background Sialic acid, a critical component for cell membrane integrity, undergoes complex biosynthesis involving enzymes like sialyltransferases (STs), impacting cancer progression. Aberrant sialylation by STs is implicated in cancer growth, invasion, and therapy resistance. Medulloblastoma (MB), a pediatric brain tumor with distinct subgroups and variable genetic alterations, poses uncertainty regarding the implications of sialylation. Methodology This study employs bioinformatic analyses on bulk and single-cell RNA-sequenced samples to explore atypical gene expressions linked to sialic acid metabolism in MB. A list of sialic biosynthesis-related genes was compiled using the STRING database. Data of MB samples from bulk and single-cell RNA sequencing were obtained from open-source repositories and were differentially analyzed, focusing on molecular subgroups (WNT, SHH, Group 3, and Group 4). The study employed survival analyses, specifically Cox regression, to analyze the overall survival (OS) data obtained through bulk RNA sequencing. Results Thirty-eight genes/proteins related to sialic acid metabolism were identified. Differential expression analysis between WNT and Group 3 and WNT and Group 4 revealed significant differences in seven and eleven genes, respectively, with consistent ST6GAL2 expression disparities (false discovery rate [FDR] P-value < 0.01, log2FC > 0.58). Elevated ST6GAL2 expression correlated with improved OS, with mortality risk reductions ranging from 26% to 48% (P-value < 0.006, Bonferroni-corrected threshold). Conclusions Elevated ST6GAL2 expression correlated with improved OS in diverse MB sample subsets, suggesting potential mechanisms in inhibiting tumor progression and enhancing immune response, requiring experimental validation.

Gender Differences in the Cortical Distribution of Corpus Callosum Fibers.

Introduction Research on gender-based disparities in human brain structure has spanned over a century, yielding conflicting results and ongoing debate. While some studies indicate minimal distinctions, others consistently highlight differences in the corpus callosum (CC), even after accounting for average brain size. Methods Diverging from previous approaches, this study examines the morphology of the entire CC fiber rather than solely focusing on its midsagittal structure. Utilizing advanced neuroimaging techniques and generalized Q-imaging tractography, CC streamlines were constructed to assess gender differences in fractional anisotropy (FA), volume ratio, and cortical distribution. Student's t-test was employed to examine the disparities in FA between gender groups, while gender-based distinctions in the normalized volume of the CC and its segments were assessed using analysis of covariance (ANCOVA), with absolute whole white matter volume serving as a covariate. Results No significant gender-based disparities were found in either FA or normalized CC volume. While females exhibited consistently larger normalized volume CC streamlines than males, these differences lost statistical significance after adjusting for absolute total white matter volume as a covariate. Nonetheless, CC streamlines in females displayed a broader spatial distribution, encompassing various cortical regions, including the bilateral prefrontal cortex (medial and lateral surfaces), as well as medial parietal and temporal regions. Conclusion This study elucidates gender-related variations in the morphology of the brain's white matter pathways, indicating a more widespread cortical distribution of CC fibers in females compared to males. However, the study underscores the need for further investigations into connectivity patterns to fully elucidate these gender-based disparities.

Bifocal germ cell tumor of pineal germinoma and neurohypophyseal embryonal carcinoma: illustrative case.

BACKGROUND: Bifocal germ cell tumors, with primarily identical tissue composition, occur concurrently in the neurohypophyseal and pineal regions. OBSERVATIONS: A 16-year-old male patient exhibited increased intracranial pressure symptoms, with concurrent tumors in the pineal and neurohypophyseal regions, causing obstructive hydrocephalus. His serum human chorionic gonadotropin level was elevated, measuring 506.6 mIU/mL. Upon gross endoscopic examination, the pineal tumor appeared white, whereas the neurohypophyseal tumor appeared red and hemorrhagic. Because of the limited sample size of the latter, a frozen section biopsy was feasible only for the pineal lesion, which indicated the presence of a germinoma. Subsequently, carboplatin and etoposide were administered, resulting in the reduction of the pineal tumor, but no effect was observed in the neurohypophyseal tumor. Histopathological analysis confirmed the pineal lesion as a germinoma, whereas the neurohypophyseal lesion was an embryonal carcinoma. Thus, the treatment was altered to ifosfamide, carboplatin, and etoposide (ICE), leading to a response in both tumors. The patient underwent three additional cycles of ICE therapy and high-dose chemotherapy, followed by whole craniospinal irradiation, achieving complete remission. LESSONS: Although most bifocal germ cell tumors share the same histological tissue, occasional differences may arise, necessitating separate biopsies for accurate assessment.

Current progress in genomics and targeted therapies for neurofibromatosis type 2.

Neurofibromatosis type 2 (NF2), a multiple neoplasia syndrome, is a manifestation of an impaired expression of the merlin protein, exerting inhibitory effects on cell proliferation signals due to abnormalities of the NF2 gene located on chromosome 22. About half of patients inherit a germline mutation from a parent, and nearly 60% of de novo NF2 patients are estimated to have somatic mosaicism. The development of technical methods to detect NF2 gene mutation, including targeted deep sequencing from multiple tissues, improved the diagnostic rate of mosaic NF2. With improved understanding of genetics and pathogenesis, the diagnostic criteria for NF2 were updated to assist in identifying and diagnosing NF2 at an earlier stage. The understanding of cell signaling pathways interacting with merlin has led to the development of molecular-targeted therapies. Currently, several translational studies are searching for possible therapeutic agents targeting VEGF or VEGF receptors. Bevacizumab, an anti-VEGF monoclonal antibody, is widely used in many clinical trials aiming for hearing improvement or tumor volume control. Currently, a randomized, double-masked trial to assess bevacizumab is underway. In this randomized control trial, 12 other Japanese institutions joined the principal investigators in the clinical trial originating at Fukushima Medical University. In this review, we will be discussing the latest research developments regarding NF2 pathophysiology, including molecular biology, diagnosis, and novel therapeutics.

A Collision Tumor of Pit-1/SF-1-positive Double Pituitary Adenoma and a Craniopharyngioma Coexisting with Graves' Disease.

Double or multiple pituitary adenomas expressing different types of transcription factors and collision tumors of pituitary adenomas and craniopharyngiomas are rare. In this report, we present a case of pituitary adenoma of two different cell populations, Pit-1 and SF-1, and an adenoma and craniopharyngioma collision tumor with coexisting Graves' disease. The patient had a 16-mm pituitary tumor with pituitary stalk calcification and optic chiasm compression but no visual dysfunction. Based on hormonal profile results, the tumor in the sella was considered a nonfunctioning pituitary adenoma; nevertheless, the pituitary stalk was invaded by a different lesion, which was later confirmed to be a craniopharyngioma. Using an endoscopic endonasal approach, the pituitary adenoma was removed; however, a small remnant remained medial to the right cavernous sinus. Because the pituitary stalk lesion was isolated from the pituitary adenoma, it was preserved to maintain pituitary function. Three years after the initial surgery, the patient suffered from Graves' disease and was treated with antithyroid medications. However, the intrasellar residual and pituitary stalk lesions gradually increased in size. A second surgery was performed, and the residual intrasellar and stalk lesions were completely removed. As per the initial and second histopathologies, the pituitary adenoma comprised different cell groups positive for thyroid-(TSH) and follicle-stimulating hormones, and each cell group was positive for Pit-1 and SF-1. The pituitary stalk lesion was an adamantinomatous craniopharyngioma. We believe that TSH-producing adenoma was involved in the development of Graves' disease or that treatment for Graves' disease increased TSH-producing adenoma.

A Ruptured Spetzler and Martin Grade V Arteriovenous Malformation in a Child Treated With Radiotherapy Followed by Embolization: A Case Report and Literature Review.

Treatment of ruptured high-grade Spetzler-Martin (S&M) arteriovenous malformation (AVM) is challenging and requires a multidisciplinary treatment approach. Here, we report a case of ruptured giant callosal Grade V AVM in a child initially treated with stereotactic radiotherapy followed by endovascular embolization with Onyx; a management approach recently described in a few reports on the "postradiosurgical embolization" method. Complete obliteration was achieved 20 months after stereotactic radiotherapy and embolization. In this article, we discuss the usefulness and significance of postradiosurgical embolization, particularly for high-grade AVMs. To our knowledge, this is the first case with a giant Spetzler-Martin Grade V AVM treated with a postradiosurgical embolization method.

Mechanical Thrombectomy for Acute Ischemic Stroke Caused by Prosthetic Aortic Valve Endocarditis Due to Exophiala dermatitidis Infection: A Case Report.

Prosthetic valve endocarditis (PVE) can cause large cerebral vessel occlusion. Many reports suggested that mechanical thrombectomy (MT) is effective and useful for early diagnosis from the histopathological findings of thrombus. We present the case of a 62-year-old man, with a history of prosthetic aortic valve replacement and pulmonary vein isolation for his atrial fibrillation, who developed a high fever and an acute neurological deficit, with left hemiplegia and speech disorder. He was diagnosed as having an acute right middle cerebral artery embolism and underwent an MT. The embolic source was found to be a PVE vegetation. However, histopathological analysis of the thrombus could not detect the actual diagnosis. Although he was treated for bacterial endocarditis, his blood culture revealed a rare fungal infection with Exophiala dermatitidis not until >3 weeks after admission. Subsequently, a ß-D-glucan assay also indicated elevated levels. Although he underwent an aortic valve replacement on day 36, MRI showed multiple minor embolic strokes till that day. Early diagnosis of fungal endocarditis and detection of the causative pathogen are still challenging, and the disease has a high risk of occurrence of early and repeated embolic stroke. In addition to clinical findings and pathological studies, ß-D-glucan assay might be a good tool for the diagnosis and evaluation of fungal endocarditis.

Intraoperative transcranial facial motor evoked potential monitoring in surgery of cerebellopontine angle tumors predicts early and late postoperative facial nerve function.

OBJECTIVE: We propose a novel method that predicts facial nerve function (FNF) calculated from the drop and recovery of facial motor evoked potential (FMEP) amplitude ratio during the surgery of cerebellopontine angle tumors. METHODS: We enrolled 73 patients with cerebellopontine angle tumor, and used a biphasic, constant current, and suprathreshold stimulation (BCS) protocol to record FMEP of the orbicularis oris. We measured the intraoperative minimum-to-baseline amplitude ratio (MBR), the final-to-baseline amplitude ratio (FBR), and the recovery value (RV). RV was measured by subtracting MBR from FBR. Using those values, we evaluated FNF both at early postoperative (EP) and late postoperative (LP) periods. RESULTS: We successfully obtained 62 FMEP readings. Facial palsies occurred in 22 patients during the EP period, and 14 patients recovered during the LP period. Both MBR and FBR showed a significant correlation with FNF in the EP period. RV showed a good predictive power of FNF recovery during the LP period for the first time. CONCLUSIONS: RV is a new and useful predictor of FNF recovery. MBR can be an intraoperative predictor of FNF in the EP period. SIGNIFICANCE: FNF outcome in the early and late postoperative periods can be predicted by FMEP.

Rationale and Design of BeatNF2 Trial: A Clinical Trial to Assess the Efficacy and Safety of Bevacizumab in Patients with Neurofibromatosis Type 2 Related Vestibular Schwannoma.

Neurofibromatosis type 2 (NF2) causes bilateral vestibular schwannomas (VSs), leading to deafness. VS is treated by surgery or radiation, but neither treatments prevent hearing loss. Bevacizumab was found to be effective in suppressing the tumor's growth and may help to improve hearing. We are conducting a randomized, double-blind, multicenter clinical trial to verify the efficacy and safety of bevacizumab in NF2-related VS. The primary objective is to evaluate the efficacy of bevacizumab in improving hearing in the affected ear. One of the secondary objectives is to evaluate bevacizumab's efficacy in rechallenge treatment in relapsed cases. Sixty patients will randomly receive either bevacizumab or a placebo and will be clinically observed for 48 weeks in the initial intervention phase. In the first half (24 weeks), they will receive either 5 mg/kg of bevacizumab or a placebo drug. In the second half, all patients will receive 5 mg/kg of bevacizumab. If hearing function deteriorated in a patient who had shown improvement during the first phase, a rechallenge dose with bevacizumab would be offered.

The superior frontal longitudinal tract: a connection between the dorsal premotor and the dorsolateral prefrontal cortices.

A few studies have identified the structural connection between the premotor area and the lateral prefrontal cortex (DLPFC) as the frontal longitudinal system (FLS). This study investigated the existence of a direct segment (none U-fibre) of the superior part of the FLS (sFLS), which connects the dorsal premotor cortex (PMd) and DLPFC and analysed its asymmetry and termination point patterns. A dataset of diffusion-weighted images from 48 subjects was used for generalised q-sampling imaging tractography. Additionally, a white-fibre dissection was conducted in two right hemispheres. An analysis of spatial location, termination points, laterality, and correlation with the subjects' gender or handedness was performed. The sFLS was found to have a deeper longitudinal bundle directly connecting the PMd and DLPFC. The bundle is referred to hereafter as the superior frontal longitudinal tract (SFLT). The SFLT was reconstructed in 100% of right and 88% of left hemispheres. It exhibited variable patterns in different subjects in their posterior terminations. In addition, it was found to possess a complicated spatial relationship with the adjacent bundles. The SFLT was revealed successfully in two cadaveric right hemispheres, where the posterior terminations were found to originate in the PMd independent of the superior longitudinal fasciculus.

Bevacizumab Therapy of Neurofibromatosis Type 2 Associated Vestibular Schwannoma in Japanese Patients.

We conducted a feasibility study to investigate the therapeutic effect of bevacizumab on vestibular schwannomas (VS) associated with neurofibromatosis type 2 (NF2) in a sample of Japanese patients. Ten NF2 patients were selected between 2013 and 2018: nine women and one man, with ages ranging from 12 to 45 years (mean: 29.4). Bevacizumab was administered intravenously in 5 mg/kg doses four times, with an inter-dose interval of 2 weeks. Seventeen tumors were followed for 3-72 months (mean: 39). A reduction from baseline tumor volume of at least 20% was considered a therapeutic radiologic response. Maximum reduction in tumor volume was identified in the 3rd month in 11 tumors, and in the 6th month in three tumors. Three tumors did not show any response to bevacizumab. A radiologic response was detected in seven tumors (41%). There was a significantly lower tumor volume mean in the 3rd month in comparison to the baseline for the entire sample. Tumors in patients aged 25 and above showed a significant reduction in volume in the 3rd month and significantly lower tumor-volume-to-baseline ratio than younger patients in both the 3rd and 6th months. The interaction between 'time' and 'age group' factors significantly affected the therapeutic outcome of bevacizumab on tumor volume. This study investigated the therapeutic effects of bevacizumab on NF2-associated vestibular schwannomas in Japanese patients. Bevacizumab appears to be a useful therapeutic choice in NF2 cases to control the growth of VS. Therefore, a randomised control trial to prove this assumption is necessary.

Acute paradoxical brain herniation after decompressive craniectomy for severe traumatic brain injury: A case report.

BACKGROUND: Sinking skin flap syndrome or paradoxical brain herniation is an uncommon neurosurgical complication, which usually occurs in the chronic phase after decompressive craniectomy. We report a unique case presenting with these complications immediately after decompressive craniectomy for severe traumatic brain injury. CASE DESCRIPTION: A 65-year-old man had a right acute subdural hematoma (SDH), contusion of the right temporal lobe, and diffuse traumatic subarachnoid hemorrhage with midline shift to the left side. He underwent an emergency evacuation of the right SDH with a right decompressive frontotemporal craniectomy. Immediately after the operation, his neurological and computed tomography (CT) findings had improved. However, within 1 h after the surgery, his neurological signs deteriorated. An additional follow-up CT showed a marked midline shift to the left, i.e., paradoxical brain herniation, and his skin flap overlying the decompressive site was markedly sunken. We immediately performed an urgent cranioplasty with the right temporal lobectomy. He responded well to the procedure. We suspected that a cerebrospinal fluid leak had caused this phenomenon. CONCLUSION: Decompressive craniectomy for severe traumatic brain injury can lead to sinking skin flap syndrome and/or paradoxical brain herniation even in the acute phase. We believe that immediate cranioplasty allows the reversal of such neurosurgical complications.

Palliative shunt surgery for patients with leptomeningeal metastasis.

OBJECTIVES: Leptomeningeal metastasis (LM) is associated with poor prognosis and affects the quality of life (QOL) of end-stage cancer patients. Severe headache associated with hydrocephalus causes reduced QOL. We investigated the clinical value of surgical treatment for hydrocephalus in LM patients. PATIENTS AND METHODS: The medical records of 11 consecutive patients who underwent lumboperitoneal shunt (LPS) or ventriculoperitoneal shunt (VPS) at our institution between 2007 and 2016 were investigated. Primary brain tumor patients were excluded. We assessed the neurological status and therapeutic effects at 1 month after the shunt surgery. RESULTS: The patients were three males and eight females with a median age of 58 years (interquartile range [IR] 52-68 years). The median preoperative neutrophil-to-lymphocyte ratio was 6.4 (IR 4.8-9.2). Symptom improvement was observed in nine patients, and severe headache was relieved in seven (88%) out of eight patients. The median Karnofsky performance status scale increased from 40 to 60, and the median overall survival after primary malignancy diagnosis was 27.4 months (IR 19.6-63.1 months). The median survival after the diagnosis of brain parenchymal metastasis, LM, and shunt surgery were 7.2 months (IR 5.1-14.1 months), 3.9 months (IR 3.5-6.3 months), and 3.3 months (IR 2.9-5.7 months), respectively. CONCLUSION: Shunt surgery for hydrocephalus could offer an effective palliative surgical option for symptom relief especially relief of severe headache, contributing improvement of QOL in LM patients.

A Novel Intraoperative Laser Light Imaging System to Simultaneously Visualize Visible Light and Near-Infrared Fluorescence for Indocyanine Green Videoangiography.

BACKGROUND: Intraoperative indocyanine green videoangiography (ICG-VA) has been reported to be utilized in various cerebrovascular surgeries, wherein the blood flow is noticeably shown in white with a black background. ICG flow alone, but not other structures, can be observed using ICG-VA. We developed a novel high-resolution intraoperative imaging system using laser light source for simultaneously visualizing both visible light and near-infrared (NIR) fluorescence images of ICG-VA. METHODS: We used a novel system for 14 cerebrovascular cases. The operative field was illuminated via an operating microscope using a novel laser light source with four bands at 464 (blue), 532 (green), 640 (red), and 785 nm (NIR region). The observed light from the operative field was split using a beam splitter cube into visible (420- 660 nm) and NIR fluorescence emission light (832-900 nm). Images from the color video and NIR fluorescence emission windows were merged for visualization on a monitor screen simultaneously. Laser light was compared with xenon light, and both setups were tested for cerebrovascular surgeries. RESULTS: Laser light has numerous advantages over xenon light. The present setup clearly visualized the color operative field with enhanced blood flow. Complete clipping or incomplete clipping with neck remnant or remnant flow into an aneurysm was confirmed in aneurysm surgeries. Feeding arteries and draining veins were easily distinguished in case of arteriovenous malformation. CONCLUSIONS: Using the present setup, we can observe the color operative field and enhanced blood flow using ICG in real time. This setup could facilitate various cerebrovascular surgeries.

Utility and safety of a novel surgical microscope laser light source.

OBJECTIVE: Tissue injuries caused by the thermal effects of xenon light microscopes have previously been reported. Due to this, the development of a safe microscope light source became a necessity. A newly developed laser light source is evaluated regarding its effectiveness and safety as an alternative to conventional xenon light source. METHODS: We developed and tested a new laser light source for surgical microscopes. Four experiments were conducted to compare xenon and laser lights: 1) visual luminance comparison, 2) luminous and light chromaticity measurements, 3) examination and analysis of visual fatigue, and 4) comparison of focal temperature elevation due to light source illumination using porcine muscle samples. RESULTS: Results revealed that the laser light could be used at a lower illumination value than the xenon light (p < 0.01). There was no significant difference in visual fatigue status between the laser light and the xenon light. The laser light was superior to the xenon light regarding luminous intensity and color chromaticity. The focal temperature elevation of the muscle samples was significantly higher when irradiated with xenon light in vitro than with laser light (p < 0.01). CONCLUSION: The newly developed laser light source is more efficient and safer than a conventional xenon light source. It lacks harmful ultraviolet waves, has a longer lifespan, a lower focal temperature than that of other light sources, a wide range of brightness and color production, and improved safety for the user's vision. Further clinical trials are necessary to validate the impact of this new light source on the patient's outcome and prognosis.

Role of transcranial sphenoidotomy in skull base surgery: classification of surgical techniques based on the surgical anatomy of the sphenoid sinus.

OBJECTIVEAlthough sphenoidotomy is more commonly performed via the transnasal approach than via the transcranial approach, transcranial sphenoidotomy (TCS) remains indispensable for en bloc resection of locally advanced sinonasal malignant tumors (SNMTs) extending to the skull base. TCS also enables transsphenoidal transposition of the temporoparietal galeal flap (TPGF) to compensate for the lack of vascularized reconstructive tissue after endoscopic transnasal skull base surgery. The objective of this study was to review the authors' surgical experience using TCS with an emphasis on the surgical anatomy of the sphenoid sinus and on the purpose of TCS. Relevant anatomy is further illustrated through cadaveric dissection and photo documentation.METHODSThe authors reviewed the records of 50 patients who underwent TCS at the Nagoya University Hospital, Fukushima Medical University Hospital, or Aichi Medical University Hospital over the course of 7 years (between January 2011 and November 2017). The authors also performed cadaveric dissection in 2 adult cadaveric skull base specimens.RESULTSOf the 50 patients included in this study, 44 underwent craniofacial resection (CFR) for en bloc resection of SNMTs involving the anterior and/or lateral skull base, and 6 underwent transsphenoidal transposition of the TPGF flap. The authors categorized the TCS procedures according to the portion of the sphenoid sinus wall involved (i.e., superior, lateral, and superolateral). Superior sphenoidotomy was used in patients requiring anterior CFR. Lateral sphenoidotomy was further divided into 2 subtypes, with type 1 procedures performed for the transsphenoidal transpositioning of the TPGF, and type 2 procedures used in patients requiring lateral CFR. Superolateral sphenoidotomy was used in anterolateral CFR.CONCLUSIONSTCS still represents a useful tool in the armamentarium of neurosurgeons treating central skull base lesions. The newly proposed surgical classification facilitates a profound understanding of TCS and how to incorporate this technique into clinical practice.

Jugular Foramen Collision Tumor (Schwannoma and Plasma Cell Pseudotumor), a Probable IgG4-Related Disease.

BACKGROUND: Lower cranial nerve sheath tumors are relatively rare. Cases of schwannoma collision tumors have rarely been reported, with most of the reported cases describing schwannoma and meningioma collision tumors. CASE DESCRIPTION: We report a very rare case of a cerebellopontine angle collision tumor of the ninth cranial nerve schwannoma with an IgG4 plasma cell pseudotumor. IgG4 plasma cell pseudotumors comprise a group of diseases called IgG4-related diseases (IgG4-RDs). These diseases usually affect organs such as the pancreas and salivary gland. Few cases of nervous system IgG4-RDs have been reported. Under intraoperative microscopy, the tumor in our case did not appear different from usual cases of schwannoma, but histopathology showed significant infiltration of IgG4 plasma cells. CONCLUSION: IgG4-RDs have a distinctive histopathologic pattern; however, their pathophysiology remains unclear. Special attention must be paid to the diagnosis of such diseases because they mimic other diseases and can be missed.