Targeted embolisation for coexisting conus medullaris arteriovenous malformation and cauda equina arteriovenous fistulas with a varix on a shared drainer†.

BACKGROUND: The coexistence of vascular malformations in the conus medullaris and cauda equina has been rarely reported, and the complex angioarchitecture in multiple arteriovenous lesions remains poorly understood. CASE DESCRIPTION: A 17-year-old woman presented with a sudden-onset, stepwise worsening of weakness and pain in the bilateral legs. Angiography revealed conus medullaris arteriovenous malformation and cauda equina arteriovenous fistulas. One of the drainers was shared between the coexisting lesions and harboured a varix. Targeted embolisation of a fistulous point in the conus lesion was performed with precaution to prevent occluding the common drainage route, which led to symptom improvement with angiographical diminishment of the varix. CONCLUSIONS: Recognising that communications between drainers can be observed in multiple spinal arteriovenous lesions is important in facilitating a safe embolisation. Cautious assessment of angiogram with fusion images of cone-beam computed tomography and volumetric T2 magnetic resonance imaging can help in establishing the diagnosis and treatment strategy.

Combined transarterial and transvenous embolization of multi-hole pial arteriovenous fistula with large varix.

BACKGROUND: Pial arteriovenous fistula (AVF) is a vascular fistulous disease in which the cerebral pial artery and vein are directly connected without the intervening nidus within a sub-pial space. Multi-hole pial AVFs, wherein multiple feeders flow into one drainer, are usually formed with complex angioarchitecture and are difficult to treat. METHODS: A rare case of an adult patient with hereditary hemorrhagic telangiectasia and multi-hole pial AVF was described. A 23-year-old woman was referred to our hospital. She was previously diagnosed with left cerebellar pial AVF with multiple feeders (bilateral superior cerebellar artery and common trunk of the left anterior inferior cerebellar artery and posterior inferior cerebellar) and large varix that had been untreated for 9 years. The enlargement of the large varix with the new second varix formation was revealed by angiography. Although asymptomatic, considered to be a risk for future hemorrhage was the continuous high hemodynamic stress. RESULTS: Endovascular embolization was performed by securing safe transarterial n-butyl-2-cyanoacrylate injection by transarterial and transvenous coil placement to the shunt points and feeders, resulting in total occlusion of the fistula without any complications. Extensive transvenous coil placement inside the varix allowed safe embolization of the entire fistula with multiple high-flow feeders. Moreover, bidirectional (transarterial and transvenous) retrograde coil embolization enabled firm obliteration of the shunt points. CONCLUSIONS: Performing combined transarterial and transvenous embolization in a well-balanced manner was considered to treat multi-hole pial AVF to provide a safe and effective embolization.

The oligodendroglial histological features are not independently predictive of patient prognosis in lower-grade gliomas.

The relevance of oligodendroglial histological features to patient prognoses is controversial. 93 LrGGs resected for about 2 decades were re-assessed based on WHO2007 with special interest to pure oligodendroglial diagnosis (oligodendroglioma or anaplastic oligodendroglioma) and presence of CFO features. Those histological features, patients OS, and tumor chromosomal/genetic characteristics were correlated each other in each of the 3 IDH-1p/19q-based molecular groups. There was significant association between 1p19q status with the oligodendroglial histological diagnosis as well as presence of CFO in the entire cohort. The oligodendroglial diagnosis was associated with longer OS in IDHmut/codel group; however, this association was not significant in the multivariate analyses. In IDHmut/noncodel and IDH-wildtype groups, the oligodendroglial diagnosis was not associated with patient OS. Presence of CFO was not associated with patient OS in any molecular groups. Gain of 8q was associated with the oligodendroglial diagnosis in IDHmut/noncodel group. Neither the oligodendroglial diagnosis nor CFO was predictive for the methylation status of the MGMT gene in any molecular groups. The oligodendroglial histological features are not independently predictive of either patient prognosis or chemotherapeutic response in LrGGs, leaving the possibility of marginal favorable association only in IDHmut/codel tumors.

Buddy Catheter Technique: A Method of Guiding the Mo.Ma Ultra into a Left Common Carotid Artery That Branches off the Aortic Arch at a Steep Angle.

Objective: The Mo.Ma Ultra is an embolic protection device used in carotid artery stenting (CAS). In cases of left internal carotid artery stenosis (ICS) in which the common carotid artery (CCA) branches off the aortic arch at a steep angle, insertion of the Mo.Ma Ultra into the CCA is sometimes difficult. We introduce a "buddy catheter technique" that helps guide the Mo.Ma Ultra into the CCA, with an additional 4 Fr catheter into the external carotid artery. Case Presentation: An 84-year-old man with left ICS whose CCA also branched off the aortic arch at a steep angle also underwent CAS. The "buddy catheter technique" was used, and the Mo.Ma Ultra was inserted smoothly. The buddy catheter technique displaces the left CCA upward. Displacement straightens the vessels anatomically, and the ledge effect can be prevented by aligning the course of the vessels with the wire. Nevertheless, this technique requires bilateral femoral puncture, and so, complications can occur. Conclusion: The buddy catheter technique may be considered in cases in which the left CCA branches off the aortic arch at a steep angle.

Pterygovaginal artery as a target of embolization before endoscopic skull base surgery.

BACKGROUND: The pterygovaginal artery (PtVA), a recurrent branch of the internal maxillary artery (IMA), can be a feeder of skull base tumors. Preoperative embolization can help endoscopic resection of hypervascular lesions, which is performed under a narrow surgical space with restricted instrumental maneuverability. METHODS: We performed preoperative embolization in five cases with hypervascular skull base lesions supplied by the PtVA, four of which were resected via endoscopic endonasal approach. In two cases, selective PtVA embolization through the distal IMA was successfully conducted. RESULTS: In all the cases, intraoperative bleeding during endoscopic resection was easily controlled. The medial and lateral origins of the PtVA from the IMA were demonstrated by cone-beam CT images reconstructed from three-dimensional rotational angiography, and anastomoses around the eustachian tube and soft palate were visualized by superselective angiography. CONCLUSIONS: The PtVA embolization can be an effective strategy before endoscopic skull base tumor resection. When embolizing through the PtVA, clinicians should be aware of its anatomical variations and dangerous anastomoses. Understanding the surrounding angioarchitecture by angiographic techniques helps ensure safe embolization.

Angio-anatomical study of the pterygovaginal artery based on cone-beam computed tomography.

PURPOSE: To investigate the anatomical characteristics and clinical implications of the pterygovaginal artery (PtVA), a recurrent branch from the distal internal maxillary artery (IMA), which courses through the pterygovaginal canal that connects the pterygopalatine fossa and nasopharynx. METHODS: Eighty-two patients with 90 sides of cone-beam computed tomography (CBCT) reconstructed from rotational angiography of the external or common carotid artery with a field of view covering the pterygopalatine fossa were retrospectively reviewed. The origin from the IMA, branching type, distribution, and anastomoses was evaluated. The underlying lesions were 36 hypervascular lesions with possible supply from PtVA (17 cavernous sinus arteriovenous fistulas (AVFs), 6 anterior condylar AVFs, and 13 nasopharyngeal, parasellar, or paraclival tumors) and 46 other diseases. RESULTS: PtVA was identified in 75 sides (83%). It originated from the pterygopalatine segment of the IMA in 45 sides (60%) and from the pterygoid segment in 30 sides (40%). It arose independently (77%), sharing the common trunk with the Vidian artery (15%) or with other branches. It ran posteromedially through the pterygovaginal canal to supply the mucosa over the nasopharyngeal roof, the choanae, and the pharyngeal ostium of the eustachian tube. It anastomosed with the ascending pharyngeal artery (n=37), the accessory meningeal artery (n=7), and the mandibular artery from the petrous internal carotid artery (n=2). It served as a feeder of osseous AVFs and skull base tumors. CONCLUSION: PtVA was often identified by CBCT even in normal anatomy. Its detailed angio-anatomy could be evaluated in the presence of parasellar or paraclival hypervascular lesions.

Efficacy of a topical gelatin-thrombin hemostatic matrix, FLOSEAL®, in intracranial tumor resection.

BACKGROUND: Hemostasis plays an important role in safe brain tumor resection and also reduces the risk for surgical complications. This study aimed to evaluate the efficacy of FLOSEAL®, a topical hemostatic agent that contains thrombin and gelatin granules, in brain tumor resections. METHODS: We evaluated the hemostatic effect of FLOSEAL by scoring the intensity of bleeding from 1 (mild) to 4 (life threatening). We assessed the rate of success of hemostasis with 100 patients who underwent intracranial tumor resection. We also investigated the duration of the operation, the amount of intra- and postoperative bleeding, the number of hospital stays, and adverse events in patients who used FLOSEAL compared with those who did not use FLOSEAL. RESULTS: FLOSEAL was applied to a total of 109 bleeding areas in 100 patients. A total of 95 bleeding areas had a score of 1 and 91 (96%) showed successful hemostasis. Thirteen bleeding areas scored 2 and 8 (62%) showed hemostasis with the first application of FLOSEAL. The second application was attempted with five bleeding areas and four showed hemostasis. About 94% (103/109 areas) of bleeding points successfully achieved hemostasis by FLOSEAL. Moreover, FLOSEAL significantly decreased the amount of intraoperative bleeding and postoperative bleeding as assessed with computed tomography on 1 day postoperatively compared with no use of FLOSEAL. There were no adverse events related to FLOSEAL use. CONCLUSION: Our results indicate that FLOSEAL is a reliable, convenient, and safe topical hemostatic agent for intracranial tumor resection.

Tumor immune microenvironment is associated with the growth of intracranial germinomas.

INTRODUCTION: The role of immune checkpoint molecules and the tumor immune microenvironment in the development of intracranial germ cell tumors remains unclear. METHODS: We investigated the expression of programed cell death-1 (PD-1), programed cell death ligand-1 (PD-L1), and tumor-infiltrating lymphocytes (TILs) in 8 patients who had intracranial germinomas with sufficient tumor tissue by immunohistochemistry, to analyze the associations between their clinical courses and radiological features. The 8 patients were categorized based on the duration between symptom onset and pathological diagnosis into the long-term onset (LTO) group (> 1 year of symptoms) and the short-term onset (STO) group (< 1 year of symptoms). RESULTS: Three patients belonged to the LTO group and 5 patients to the STO group. Compared with STO tumors, LTO tumors were significantly associated with a lower ratio of PD-L1-positive tumor cells (p = 0.012), higher number of infiltrating CD3- and CD8-positive lymphocytes (p = 0.016, 0.003, respectively), and lower ratio of PD-1-positive cells per CD8-positive lymphocytes (p = 0.047). LTO germinomas were significantly smaller in size than STO tumors, not associated with hydrocephalus, and tended to be present in patients with older age at diagnosis and atypical tumor location. CONCLUSIONS: Our data suggest that the tumor immune microenvironment, including PD-1/PD-L1 signaling, is associated with the growth of intracranial germinomas.

Trigeminal Neuralgia Due to Petrosal Bone Deformity.

BACKGROUND: There have been a few previous reports of trigeminal neuralgia caused by bony structures. We report a rare case of trigeminal neuralgia caused by petrous bone deformity. CASE DESCRIPTION: A-43-year-old man with facial pain in the left maxillary and mandibular divisions of the trigeminal nerve was referred to our hospital. Computed tomography showed bone hyperplasia protruding into the left cerebellopontine cistern, compressing the left trigeminal nerve. Administration of carbamazepine ameliorated facial pain, but the effect was unsatisfactory, and microvascular decompression was performed through the anterior transpetrosal approach. The protruding petrous bone was drilled out to release compression of the trigeminal nerve, thus straightening the deviated trajectory of the nerve. The superior cerebellar artery adjacent to the thickened petrosal bone seemed to have compressed the trigeminal nerve and was also transposed. The facial pain disappeared completely after microvascular decompression surgery. CONCLUSIONS: The anterior transpetrosal approach is an appropriate and essential strategy for trigeminal neuralgia caused by petrous bone deformity of the petrous apex.

Association Between CD133, CD44, and Nestin Expression and Prognostic Factors in High-Grade Meningioma.

BACKGROUND: Meningioma is the most common primary brain tumor. It is graded as I, II, or III based on the World Health Organization (WHO) classification of central nervous system tumors. Meningiomas, especially those classified as grade II-III, have an aggressive history and a high recurrence rate. Cancer stem cells (CSCs) represent a small subset of tumor cells and are considered to be involved in tumor initiation, growth, and/or recurrence. To date, the CSCs of meningioma have not been well established. METHODS: We assessed 51 grade II/III meningiomas using immunohistochemistry to determine if a correlation exists with the prognosis by investigating CD133, CD44, and nestin expression as possible CSC markers and age, gender, initial WHO tumor grade, Simpson grade, and the use of adjuvant radiation therapy. RESULTS: The median overall survival was 7.1 years, and the median progression-free survival (PFS) was 1.8 years. Univariate analysis using Cox proportional hazards regression revealed a negative correlation between CD133 and nestin expression and PFS (P = 0.0176 and P = 0.0138, respectively), and high expression of CD44 demonstrated a tendency toward a shorter PFS (P = 0.0563), as did the initial WHO grade and Simpson grade found at the initial operation (P = 0.0166 and P 0.0333, respectively). Multivariate analysis showed relevance between CD133 and nestin expression and PFS. CONCLUSIONS: CD133 and nestin expression, initial WHO grade and Simpson grade were associated with PFS in patients with grade II/III meningioma. These findings might suggest that these molecules are representative of CSCs in meningioma regarding the aspect of clinical course.

Primary Intracranial Rhabdomyosarcoma in the Cerebellopontine Angle Resected After Preoperative Embolization.

BACKGROUND: Primary intracranial rhabdomyosarcoma is a very rare malignant tumor. Owing to moderately vascular and firm characteristics of the tumor, complete removal without complications is often difficult. In pediatric patients, the volume of total blood is less than in adults, and minimal intraoperative hemorrhage is desirable. CASE DESCRIPTION: A 6-year-old boy presented with ataxia and was found to have a large cerebellopontine angle tumor and hydrocephalus. The patient underwent preoperative coil embolization of the anterior inferior cerebellar artery to decrease tumor vascularity, and gross total removal was achieved on the same day via a suboccipital approach. Pathologic diagnosis was rhabdomyosarcoma. CONCLUSIONS: Successfully resection of primary intracranial rhabdomyosarcoma was achieved after preoperative embolization. Tumor vascularity was significantly reduced, and gross total removal was achieved safely with less hemorrhage.

Association between programmed cell death ligand-1 expression and extracranial metastasis in intracranial solitary fibrous tumor/hemangiopericytoma.

BACKGROUND: Intracranial solitary fibrous tumor/hemangiopericytoma (SFT/HPC) often shows extracranial metastasis, and treatment options are very limited. Immune-checkpoint molecules have not been studied well in SFT/HPCs, and their role in intracranial SFT/HPCs remains unclear. METHODS: We investigated the expression of programmed cell death-1 (PD-1), programmed cell death ligand-1 (PD-L1), and tumor-infiltrating lymphocytes (TIL) in 16 patients of intracranial SFT/HPC by immunohistochemistry to determine if correlation with prognosis exists. RESULTS: Median overall survival (OS) of 16 patients was 9.2 years, and median follow-up of alive patients was 9.9 years. Recurrence was observed in 13 (81.3%) patients, and extracranial metastasis were observed in 6 (37.5%). PD-L1 expression was observed in all 16 tumors, whereas PD-1 expression was observed in 2. CD3 and CD8 expressions were observed in TILs in 12 and 13 patients respectively. Although the ratio of PD-L1 positive-tumor cells was not associated with OS, progression-free survival, or metastasis-free survival (MFS), diffuse staining of PD-L1 showed a trend toward shorter time to treatment failure (TTF: time to either extracranial metastasis or death) (p = 0.072). Similarly, the intense staining of PD-L1 was associated with shorter MFS (p = 0.0084) and TTF (p = 0.033). CD3 or CD8 expression was not associated with any of the prognostic parameters. In the combined analysis of PD-L1 and CD8, diffuse PD-L1 staining coupled with no or sparse CD8 expression was significantly associated with a shorter TTF (p = 0.005) and showed a trend toward shorter MFS (p = 0.0611). CONCLUSIONS: PD-L1 is frequently expressed in intracranial SFT/HPCs, and diffuse or intense PD-L1 expression might be associated with the early occurrence of extracranial metastases.

Crowned Dens Syndrome Occurred in a Patient after Simple Drainage for Chronic Subdural Hematoma: A Case Report.

Crowned dens syndrome (CDS) is a rare disease which presents with neck pain and rigidity. A 74-year-old man with right chronic subdural hematoma (CSDH) underwent hematoma drainage. After the operation, he complained of neck pain and laboratory test revealed elevation of C-reactive protein (CRP) and white blood cell (WBC). Suspecting localized infection, wound irrigation was performed. Neck pain relieved after irrigation, but we could not find the source of infection. CDS was diagnosed by computed tomography (CT). CDS is frequently misdiagnosed as meningitis and localized infection. CT is useful for diagnosis. Neurosurgeons need to be aware of CDS after operation.

Transvenous Embolization of the Basilar Plexus Before Endoscopic Transsphenoidal Transclival Surgery.

BACKGROUND: The endoscopic transclival approach is a method for reaching the anterior side of the brainstem. In making this approach, bleeding from the basilar plexus is often observed when cutting the dura overlying the clivus. Controlling such bleeding is essential. CASE DESCRIPTION: We performed a transvenous embolization of the basilar plexus before making the transclival approach. After embolization, bleeding from the basilar plexus during the operation decreased considerably. CONCLUSIONS: To our knowledge, this is the first report of transvenous embolization of the basilar plexus. If the basilar plexus is well developed on preoperative computed tomographic venography or digital subtraction angiography, this procedure is useful for controlling bleeding during endoscopic transclival surgery.

Significant antitumor response of disseminated glioblastoma to bevacizumab resulting in long-term clinical remission in a patient with encephalocraniocutaneous lipomatosis: A case report.

The prognosis of recurrent and disseminated glioblastoma is very poor. Bevacizumab is an effective established therapy for recurrent glioblastoma following treatment with radiotherapy plus temozolomide. However, the efficacy of bevacizumab is limited to prolonging progression-free survival, without significant prolongation of the overall survival. We herein report a case of glioblastoma in a 32-year-old female patient with encephalocraniocutaneous lipomatosis (ECCL) that had disseminated following surgical resection and subsequent treatment with temozolomide and radiation therapy. The disseminated tumors disappeared completely after five courses of bevacizumab therapy. Surprisingly, the patient has remained in clinical remission for >2.5 years after dissemination by continuing this therapy. To the best of our knowledge, this is the first case of long-time clinical remission following glioblastoma dissemination and treatment with bevacizumab. In the present case, bevacizumab exerted an atypically strong antitumor effect against disseminated glioblastoma after multidisciplinary treatments had already been applied. Moreover, this is the first report of ECCL associated with a malignant brain tumor.

The outermost "dura-like membrane" of vestibular schwannoma.

BACKGROUND: The membranous structure of vestibular schwannoma is an important factor in its surgical treatment. Herein, we report intraoperative and microscopic findings relating to an outermost dura-like membrane in cases of vestibular schwannoma and the importance of these findings. METHODS: Intraoperative findings of 16 cases of vestibular schwannoma treated with an initial surgery were studied with an aim to determine if the cases had a dura-like membrane. Then we studied microscopic findings of the dura-like membrane using hematoxylin and eosin, Masson trichrome, and immunohistochemical staining in 2 cases. RESULTS: The dura-like membrane was observed in 8 out of 16 cases. The average tumor size of the cases that had a dura-like membrane was 30 ± 8.1 mm, and Koos grading 4 was in 7 out of 8 cases, and one was grade 3. In cases without a dura-like membrane, these values were significantly smaller, with an average tumor size of 12.8 ± 5.2 mm, and Koos grading 4 was only in 1 of 8 cases, grade 3 was in 2 cases, and other 5 cases were grade 2. The outermost dura-like membrane enveloped the vestibular schwannoma around the internal acoustic meatus and was continuous with the dura mater. Reactive angiogenesis was observed in the dura mater. Microscopic findings proved its continuity with the dura mater. In one case, the facial nerve was damaged before it was identified during subcapsular dissection. In that case, the dura-like membrane negatively affected our ability to identify the facial nerve. CONCLUSIONS: A dura-like membrane sometimes envelops vestibular schwannoma around the internal acoustic meatus. Recognition of this membranous structure is important for the surgical preservation of facial and acoustic nerves.