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Open access

Mestet Yibeltal Shiferaw, Yemisirach Bizuneh Akililu, Bethelehem Yesehak Worku, Tsegazeab Laeke T/Mariam, and Abenezer Tirsit Aklilu

BACKGROUND

Multiple-site open neural tube defects (MNTDs) and multiple-site split cord malformations (MSCMs) are extremely rare congenital anomalies that are defined by the simultaneous noncontiguous occurrence of more than one neural tube defect (NTD) and split cord malformation (SCM), respectively, in a single case with normal neural tissue in between. This work shows the cooccurrence of MNTDs and MSCMs, which has never been reported in the literature.

OBSERVATIONS

A single-stage repair for a 13-day-old female neonate with a preoperative diagnosis of MNTDs (thoracic meningocele and thoracolumbar myelomeningocele) plus an additional intraoperative diagnosis of MSCMs (type 3c) of thoracic and thoracolumbar spine, and thickened filum terminale was done with a favorable smooth postoperative course.

LESSONS

The use of intraoperative meticulous surgical technique along with preoperative skin stigmata helped for anticipation, detection, and treatment of associated complex spinal MNTDs, especially in resource-limited settings, where preoperative magnetic resonance imaging is not routinely used. Whether to repair the MNTDs as a single- versus multiple-stage procedure is mainly a function of the patient’s tolerance to the duration of anesthesia and the anticipated blood loss for the patient’s age. The overall developmental biology and long-term clinical outcome of MNTDs compared to single NTD/SCM is poorly understood and needs further study.

Open access

Eri Shiozaki, Yoichi Morofuji, Fumiya Kutsuna, Daiki Uchida, Ichiro Kawahara, Tomonori Ono, Wataru Haraguchi, and Keisuke Tsutsumi

BACKGROUND

A craniocervical junction arteriovenous fistula (CCJAVF) is a rare vascular malformation, and its etiology remains unclear. Here, to the best of the authors’ knowledge, they present the first case of CCJAVF associated with thrombus formation in the ipsilateral internal jugular vein.

OBSERVATIONS

An 80-year-old man presented with a sudden occipital headache. Computed tomography revealed a subarachnoid hemorrhage surrounding the brainstem and upper cervical cord. Digital subtraction angiography showed a CCJAVF fed by the left C2 radiculomeningeal artery with ascending intracranial drainage and epidural plexus. After endovascular treatment, the authors retrospectively found that his ipsilateral internal jugular vein and innominate vein were occluded with a huge thrombus at admission.

LESSONS

This case suggested a restricted antegrade venous flow due to thrombus-induced progressive retrograde intracranial drainage causing hemorrhage. Venous hypertension should be considered one of the causes of hemorrhage due to CCJAVF as well as intracranial arteriovenous fistulas.

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Arthur Hosmann, Mohammed Jaber, Thomas Roetzer-Pejrimovsky, Gerald Timelthaler, Martin Borkovec, Barbara Kiesel, Lisa I. Wadiura, Matthias Millesi, Petra A. Mercea, Joanna Phillips, Shawn Hervey-Jumper, Anna S. Berghoff, Johannes A. Hainfellner, Mitchel S. Berger, Walter Stummer, and Georg Widhalm

OBJECTIVE

Early markers are urgently needed in low-grade glioma (LGG) evaluation to rapidly estimate the individual patient’s prognosis and to determine the optimal postoperative management. Generally, visible 5-aminolevulinic acid (5-ALA) fluorescence is present in only a few LGGs. Recently, the authors identified visible 5-ALA fluorescence as a powerful intraoperative marker for unfavorable outcome in LGG treatment. However, its precise histopathological correlate is unclear. Neoangiogenesis represents a crucial event in tumor evolution, and CD34 is an established marker for vascular endothelial progenitors potentially indicating tumor progression. The aim of this study was thus to correlate 5-ALA fluorescence and CD34 microvascularity as well as to investigate the prognostic value of CD34 in a large series of LGGs.

METHODS

In this retrospective study including 3 specialized centers, patients with histopathologically confirmed isocitrate dehydrogenase–mutated LGGs (WHO grade II) receiving 5-ALA prior to resection were included. During surgery, the presence of visible fluorescence was analyzed and one representative tumor sample from the area with the maximum fluorescence effect (tumor with focal fluorescence or nonfluorescing tumor) was selected for each LGG. All fluorescing or nonfluorescing tumor samples were stained for CD34 and semiquantitatively analyzed for microvascular proliferation patterns (physiological vessels, branching capillaries, or microvessel clusters) as well as automatically quantified for CD34 microvessel density (MVD) by standardized histomorphometry software. These semiquantitative/quantitative CD34 data were correlated to the fluorescence status and patient outcome including progression-free survival (PFS), malignant transformation–free survival (MTFS), and overall survival (OS).

RESULTS

In a total of 86 LGGs, visible fluorescence was found during surgery in 13 (15%) cases. First, the semiquantitative CD34 score significantly correlated with intraoperative fluorescence (p = 0.049). Accordingly, the quantitative CD34 MVD was significantly higher in tumors showing fluorescence (p = 0.03). Altogether, the semiquantitative CD34 score showed a strong correlation with quantitative CD34 MVD (p < 0.001). At a mean follow-up of 5.4 ± 2.6 years, microvessel clusters in semiquantitative analysis were a prognostic marker for poor PFS (p = 0.01) and MTFS (p = 0.006), but not OS (p = 0.28). Finally, quantitative CD34 MVD > 10 vessels/mm2 was a prognostic marker for poor PFS (p = 0.01), MTFS (p = 0.008), and OS (p = 0.049).

CONCLUSIONS

The data indicate that CD34 microvascularity is associated with intraoperative 5-ALA fluorescence and outcomes in patients with LGG. Thus, visible fluorescence in LGGs might indicate increased CD34 microvascularity, serving as an early prognostic marker for unfavorable patient outcome that is already available during surgery.

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Andrea D. Maier, Alessandra Meddis, Christian Mirian, Jeppe Haslund-Vinding, Jiri Bartek Jr., Sebastian M. Krog, Thi Uyen Phuong Nguyen, Aušrinė Areškevičiūtė, Linea C. Melchior, Steffen Heegaard, Bjarne W. Kristensen, Tina N. Munch, Kåre Fugleholm, Morten Ziebell, David R. Raleigh, Frantz R. Poulsen, Thomas A. Gerds, Thomas Litman, David Scheie, and Tiit Mathiesen

OBJECTIVE

Meningioma is the most common primary intracranial neoplasm. Only 1%–3% of meningiomas are malignant according to the 2016 WHO criteria (WHO grade III). High-grade meningiomas present specific gene expression signatures indicating aggressive growth or recurrence. However, changes in gene expression and in neuroinflammatory gene expression signatures in WHO grade III meningiomas and during progression from WHO grade I or II to grade III are unknown.

METHODS

The authors used a NanoString targeted gene expression panel with focus on 787 genes relevant in meningioma pathology and neuroinflammatory pathways to investigate patients with grade III meningiomas treated at Rigshospitalet from 2000 to 2020 (n = 51). A temporal dimension was added to the investigation by including samples from patients’ earlier grade I and II meningiomas and grade III recurrences (n = 139 meningiomas). The authors investigated changes in neuroinflammatory gene expression signatures in 1) grade I meningiomas that later transformed into grade III meningiomas, and 2) grade III meningiomas compared with nonrecurrent grade I meningiomas.

RESULTS

The authors’ data indicate that FOXM1, TOP2A, BIRC5, and MYBL2 were enriched and the HOTAIR regulatory pathway was enriched in grade III meningiomas compared with nonrecurrent grade I meningiomas. They discovered a separation of malignant and benign meningiomas based only on genes involved in microglia regulation with enrichment of P2RY12 in grade I compared with grade III meningiomas. Interestingly, FOXM1 was upregulated in premalignant grade I meningioma years before the grade III transformation.

CONCLUSIONS

The authors found gene expression changes in low-grade meningiomas that predated histological transformation to grade III meningiomas. Neuroinflammation genes distinguished grade III from grade I meningiomas.

Open access

Shuhei Yamada, Ryuichi Hirayama, Takamitsu Iwata, Hideki Kuroda, Tomoyoshi Nakagawa, Tomofumi Takenaka, Noriyuki Kijima, Yoshiko Okita, Naoki Kagawa, and Haruhiko Kishima

OBJECTIVE

Meningiomas are the most common primary intracranial tumors, and their clinical and biological characteristics vary by location. Convexity, parasagittal, and falx meningiomas account for approximately 50%–65% of intracranial meningiomas. Focusing only on these locations, the aim of this study was to determine the typical speed of tumor growth, to assess the growth risk, and to show the possible tumor volume that many lesions can reach after 5 years.

METHODS

Patients with radiologically suspected convexity, parasagittal, or falx meningiomas at the authors’ institution were studied retrospectively. The relative growth rate (RGR) and annual volume change (AVC) were calculated from MRI at more than 3-month intervals. Based on sex, age, and signal intensity on T2-weighted MRI, the cases were classified into three groups: extremely high-growth, high-growth, and low-growth groups.

RESULTS

The data of 313 cases were analyzed. The median RGR and AVC for this entire cohort were 6.1% (interquartile range [IQR] 2.4%–16.0%) and 0.20 (IQR 0.04–1.18) cm3/year, respectively. There were significant differences in sex (p = 0.018) and T2-weighted MRI signal intensity (p < 0.001) for RGR, and T2-weighted MRI signal intensity (p < 0.001), tumor location (p = 0.025), and initial tumor volume (p < 0.001) for AVC. The median RGR and AVC were 17.5% (IQR 8.3%–44.1%) and 1.05 (IQR 0.18–3.53) cm3/year, 8.2% (IQR 2.9%–18.6%) and 0.33 (IQR 0.06–1.66) cm3/year, and 3.4% (IQR 1.2%–5.8%) and 0.04 (IQR 0.02–0.21) cm3/year for the extremely high-growth, high-growth, and low-growth groups, respectively, with a significant difference among the groups (p < 0.001). A 2.24-times, or 5.24 cm3, increase in tumor volume over 5 years was typical in the extremely high-growth group, whereas the low-growth group showed little change in tumor volume even over a 5-year follow-up period.

CONCLUSIONS

For the first time, the typical speed of tumor growth was calculated, focusing only on patients with convexity, parasagittal, and falx meningiomas. In addition, the possible tumor volume that many lesions in these locations can reach after 5 years was shown based on objective indicators. These results may allow clinicians to easily detect lesions that require frequent follow-up or early treatment by determining whether they deviate from the typical range of the growth rate, similar to a growth chart for children.

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Eric J. Lehrer, Manmeet S. Ahluwalia, Jason Gurewitz, Kenneth Bernstein, Douglas Kondziolka, Ajay Niranjan, Zhishuo Wei, L. Dade Lunsford, Kareem R. Fakhoury, Chad G. Rusthoven, David Mathieu, Claire Trudel, Timothy D. Malouff, Henry Ruiz-Garcia, Phillip Bonney, Lindsay Hwang, Cheng Yu, Gabriel Zada, Samir Patel, Christopher P. Deibert, Piero Picozzi, Andrea Franzini, Luca Attuati, Rahul N. Prasad, Raju R. Raval, Joshua D. Palmer, Cheng-Chia Lee, Huai-Che Yang, Brianna M. Jones, Sheryl Green, Jason P. Sheehan, and Daniel M. Trifiletti

OBJECTIVE

Immune checkpoint inhibitors (ICIs) and stereotactic radiosurgery (SRS) are commonly utilized in the management of brain metastases. Treatment-related imaging changes (TRICs) are a frequently observed clinical manifestation and are commonly classified as imaging-defined radiation necrosis. However, these findings are not well characterized and may predict a response to SRS and ICIs. The objective of this study was to investigate predictors of TRICs and their impact on patient survival.

METHODS

This retrospective multicenter cohort study was conducted through the International Radiosurgery Research Foundation. Member institutions submitted de-identified clinical and dosimetric data for patients with non–small cell lung cancer (NSCLC), melanoma, and renal cell carcinoma (RCC) brain metastases that had been treated with SRS and ICIs. Data were collected from March 2020 to February 2021. Univariable and multivariable Cox and logistic regression analyses were performed. The Kaplan-Meier method was used to evaluate overall survival (OS). The diagnosis-specific graded prognostic assessment was used to guide variable selection. TRICs were determined on the basis of MRI, PET/CT, or MR spectroscopy, and consensus by local clinical providers was required.

RESULTS

The analysis included 697 patients with 4536 brain metastases across 11 international institutions in 4 countries. The median follow-up after SRS was 13.6 months. The median age was 66 years (IQR 58–73 years), 54.1% of patients were male, and 57.3%, 36.3%, and 6.4% of tumors were NSCLC, melanoma, and RCC, respectively. All patients had undergone single-fraction radiosurgery to a median margin dose of 20 Gy (IQR 18–20 Gy). TRICs were observed in 9.8% of patients. The median OS for all patients was 24.5 months. On univariable analysis, Karnofsky Performance Status (KPS; HR 0.98, p < 0.001), TRICs (HR 0.67, p = 0.03), female sex (HR 0.67, p < 0.001), and prior resection (HR 0.60, p = 0.03) were associated with improved OS. On multivariable analysis, KPS (HR 0.98, p < 0.001) and TRICs (HR 0.66, p = 0.03) were associated with improved OS. A brain volume receiving ≥ 12 Gy of radiation (V12Gy) ≥ 10 cm3 (OR 2.78, p < 0.001), prior whole-brain radiation therapy (OR 3.46, p = 0.006), and RCC histology (OR 3.10, p = 0.01) were associated with an increased probability of developing TRICs. The median OS rates in patients with and without TRICs were 29.0 and 23.1 months, respectively (p = 0.03, log-rank test).

CONCLUSIONS

TRICs following ICI and SRS were associated with a median OS benefit of approximately 6 months in this retrospective multicenter study. Further prospective study and additional stratification are needed to validate these findings and further elucidate the role and etiology of this common clinical scenario.

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Binghao Zhao, Hao Xing, Yu Xia, and Wenbin Ma

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Aysegul Esen Aydin, Seckin Aydin, Berra Bilgin, Muyassar Mirkhasilova, Nicat Bayramli, and Necmettin Tanriover

OBJECTIVE

The thalamocortical projections of the auditory system have not been detailed via microanatomical fiber dissections from a surgical viewpoint. The aim of this study was to delineate the course of the auditory radiations (ARs) from the medial geniculate body to their final destination in the auditory cortex. The authors’ additional purpose was to display the relevant neural structures in relation to their course en route to Heschl’s gyrus.

METHODS

White matter fibers were dissected layer by layer in a lateral-to-medial, inferolateral-to-superomedial, and inferior-to-superior fashion.

RESULTS

The origin of ARs just distal to the medial geniculate body was revealed following the removal of the parahippocampal gyrus, cingulum bundle, and mesial temporal structures, in addition to the lateral geniculate body. Removing the fimbria, stria terminalis, and the tail of the caudate nucleus along the roof of the temporal horn in an inferior-to-superior direction exposed the lateral compartment of the sublenticular segment of the internal capsule as the predominant obstacle that prevents access to the ARs. The ARs were initially obscured by the inferolaterally located temporopulvinar tract of Arnold, and their initial course passed posterolateral to the temporopontine fascicle of Türck. The ARs subsequently traversed above the temporopulvinar fibers in a perpendicular manner and coursed in between the optic radiations at the sensory intersection region deep to the inferior limiting sulcus of insula. The distal part of the ARs intermingled with the fibers of the anterior commissure and inferior fronto-occipital fasciculus during its ascent toward Heschl’s gyrus. The ARs finally projected to a large area over the superior temporal gyrus, extending well beyond the anteroposterior boundaries of the transverse temporal gyri.

CONCLUSIONS

The ARs can be appreciated as a distinct fiber bundle ascending between the fibers of the sublenticular segment of the internal capsule and traversing superiorly along the roof of the temporal horn by spanning between the optic radiations. Our novel findings suggest potential disruption of the ARs’ integrity during transsylvian and transtemporal approaches along the roof of the temporal horn toward the mesial temporal lobe. The detailed 3D understanding of the ARs’ relations and awareness of their course may prove helpful to secure surgical interventions to the region.

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Mariano Socolovsky, Karina Barillaro, Gonzalo Bonilla, Gilda Di Masi, and Martijn Malessy

OBJECTIVE

After brachial plexus injuries (BPIs), nerve transfers are used to restore lost muscle function. Brain plasticity underlies the process of regaining volitional control, which encompasses disconnection of the original donor nerve–related programs and reconnection to acceptor nerve programs. To the authors’ knowledge, the levels of disconnection and reconnection have never been studied systematically. In this study, the authors developed a novel 4-point plasticity grading scale (PGS) and assessed the degree of volitional control achieved, identifying clinical correlations with this score.

METHODS

Patients with BPI who underwent a phrenic, spinal accessory, median, and/or ulnar fascicle nerve transfer to restore biceps and deltoid function were asked to maximally contract their target muscle as follows: 1) by using only the donor nerve program, and 2) by activating the target muscle while consciously trying to avoid using the donor nerve, with assessment each time of the Medical Research Council (MRC) scale grade for muscle strength. The authors’ PGS was used to rate the level of volitional control achieved. PGS grade 1 represented the lowest independent volitional control, with MRC grade 4 obtained in response to the donor command and MRC grade 0 in response to the acceptor command (minimum brain plasticity), whereas PGS grade 4 was no noticeable contraction in response to the donor command and MRC grade 4 in response to the acceptor command (maximum brain plasticity).

RESULTS

In total, 153 patients were studied. For biceps restoration, the phrenic nerve was used as a donor in 44 patients, the spinal accessory nerve in 40 patients, and the median and/or ulnar fascicles in 44 patients. A triceps branch was used to restore deltoid function in 25 patients. The level of volitional control achieved was PGS grade 1 in 1 patient (0.6%), grade 2 in 21 patients (13.7%), grade 3 in 103 patients (67.3%), and grade 4 in 28 patients (18.3%). The median PGS grade did not differ significantly between the four donor nerves. No correlations were observed between age, time from BPI to surgery, duration of follow-up, or compliance with rehabilitation and PGS grade.

CONCLUSIONS

Just around 20% of the authors’ patients developed a complete disconnection of the donor program along with complete independent control over the reinnervated muscle. Incomplete disconnection was present in the vast majority of the patients, and the level of disconnection and control was poor in approximately 15% of patients. Brain plasticity underlies patient ability to regain volitional control after a nerve transfer, but this capacity is limited.