A Unique Case Report of Infant-Type Hemispheric Glioma (Gliosarcoma Subtype) with TPR-NTRK1 Fusion Treated with Larotrectinib

Herein, we present a rare case of a nine-month-old boy diagnosed with infant-type hemispheric glioma (gliosarcoma subtype) at the left frontal lobe. Following subtotal resection, the patient started chemotherapy with the BABY POG protocol. We describe the clinical diagnosis, histological characteristics, radiological features, molecular aspects, and management of this tumor. A comprehensive molecular analysis on the tumor tissue showed a <i>TPR-NTRK1</i> gene fusion. The patient was treated with a TRK inhibitor, larotrectinib, and exhibited a stable disease with residual lesion following 8 months of target therapy. The present study is the first report of an infantile gliosarcoma harboring <i>NTRK1</i> rearrangement treated with larotrectinib.

Intraoperative use of heads-up display in skull base surgery

In this video, the authors highlight the applications of virtual reality and heads-up display in skull base surgery by presenting the case of a 45-year-old woman with an incidental large clinoid meningioma extending into the posterior fossa. The patient underwent preoperative endovascular tumor embolization to facilitate tumor resection and reduce blood loss, followed by a right pterional craniotomy. The use of intraoperative Doppler, intraoperative neurophysiological monitoring, and endoscope-assisted microsurgery is also featured. A subtotal resection was planned given tumor encasement of the posterior communicating and anterior choroidal arteries. No new neurological deficits were noted after the surgical procedure. The video can be found here: https://stream.cadmore.media/r10.3171/2021.10.FOCVID21177

Refractory Craniopharyngioma: Successful Treatment with LINAC Based Stereotactic Radiosurgery(SRS): First Case in Nepal

Purpose: To present first case of refractory craniopharyngioma treated successfully with SRS in Nepal.Background: Craniopharyngioma is a benign tumour, which progresses slowly and compresses the pituitary gland and nearby structures. First line of treatment is surgery followed by adjuvant radiotherapy as complete resection is usually not feasible. Here, we are reporting a case of recurrent craniopharyngioma treated with LINAC based SRS.Case Presentation: A 43 years old man diagnosed case of craniopharyngioma in May 2019. He underwent left pterional craniotomy and subtotal resection of tumour and kept on observation. He developed symptomatic as well as radiological recurrence in July 2020. Second debulking was not possible, so we did SRS on 23rd August 2020; 14Gy Dose was delivered to gross tumour volume. Six months after SRS, Patient is doing well.Conclusions: LINAC based SRS is a frameless, non-invasive and safe procedure with excellent clinical outcomes for recurrent or residual craniopharyngioma.

Changes in the filtration function of the spleen after surgery following traumatic organ injuries

The incidence of splenic injuries among all closed injuries of the abdominal cavity is from 15.5 to 30.0 %, and a mortality rate is between 7 % and 26 %. The tactics in the treatment of splenic injuries is determined by the degree of traumatic injury, the patient's condition during the operation, and a concomitant pathology. Studies of tissue regeneration of the operated spleen allow identifying two main options for its regeneration, associated with the nature of blood circulation in the preserved part. Objective — to study the regeneration and filtration function of the spleen after various types of surgery for the management of traumatic spleen injuries. Materials and methods. The results of treatment of 85 patients in the period 2015 — 2020 were analyzed. To investigate the changes in the filtration function of the spleen, a study of the peripheral blood (general blood test) was performed to assess the shape of erythrocytes. In order to determine the size of the residual splenic parenchyma, its structure and regenerative processes were evaluated and sonographic examination was carried out. Results. The average increase in the size of the residual splenic tissue after subtotal resection of the organ with the formation of couplings of the parenchyma averages 40.2 ± 3.4 % one year after surgery, and in patients who underwent subtotal resection of the spleen with covering the cut plane with adhesive hemostatic plate 70 % — 49.14 ± 6.77 %. The study of changes in the filtration function of the spleen in patients, who underwent subtotal resection of the spleen, showed the appearance of target cells, acanthocytes and halocytes in the peripheral blood, but their number was insignificant and did not exceed normal (not more than 3 %). Any destroyed and pathologically altered erythrocytes were not visualized in patients, who underwent atypical resection of the spleen (mass deficit less than 30 %). Conclusions. Spleen regeneration is determined both by the nature of blood supply to the residual splenic tissue and its size, and does not depend on the nature of the pathological process. Organ‑preserving surgical interventions on the spleen allow maintaining the filtration function of the spleen.

An Evidence-Based Vestibular Schwannoma Surgical Outcome Grading Scale for Large-to-Giant Vestibular Schwannomas

Objectives: The objective of this study was to determine the relationship between facial nerve function and extent of resection (EOR) as outcomes in the surgical management of large vestibular schwannoma (VS) (≥ 2.5 cm maximal) and evaluate use of a new grading system that incorporates both outcomes.Methods: We conducted a systematic review of the electronic databases using different MeSH terms from 1990 to 2021. 5,623 patients from 56 studies were found appropriate for inclusion in the study. Surgical approach was reported in 5,144 patients, including translabyrinthine approach (TL) in 43.3% (n=2,225), retrosigmoid (RS) approach in 56.3% (n=2,899), retrolabyrinthine (RL) approach in 0.3% (n=16), and extended translabyrinthine (EX) approach in 0.1% (n=4). The proposed VS Grading System defines outcomes of gross total resection (GTR), near total resection (NTR) and subtotal resection (STR) with good facial nerve function [House-Brackmann (HB) Grade I-II] as Classes A, B and C respectively. Those with poor facial nerve outcome (HB III-VI) are graded as Classes D, E and F, respectively. Results:. As expected, patients with STR had a higher likelihood of better facial nerve outcomes (HB I-II) compared to NTR [Odds Ratio (OR): 7.30, 95%CI: 2.45-12.1, p=0.004] and GTR (OR: 9.61, 95%CI: 3.61-15.6, p=0.002), while NTR had better facial nerve outcome than GTR (OR: 1.5, 95%CI: 1.14-4.6, p<0.0001). A Class A result, representing the best possible outcome, was obtained in 55.8% of TL vs. 49.4% undergoing RS approach. Conclusion: Complete surgical resection with preservation of facial nerve function is the gold standard for large VS.

Endonasal Endoscopic and Transoral Approaches to the Craniovertebral Junction and the Clival Region

Introduction: The transoral approach was first described by Kanavel in 1917 to treat injuries of the craniospinal junction. In 2002, Frempong-Boaudu reported 7 adults who underwent endoscopically assisted transoral surgery, this was the first report that endoscope was used in an assisted manner for transoral surgery. In 2005, Kassam published the first report of an EEA being used to perform an odontoidectomy. Method: A descriptive, retrospective and linear study was carried out in 16 patients who underwent surgery using the microsurgical and endoscopic transoral and Extended Endoscopic Endonasal approach in the period from January 2004 to May 2021. Results: The average age of the patients was 45 years and there was a predominance of the male sex (10) with the female one (6). The tumoral pathology (9), 6 cases with histological diagnosis of clivus cordroma and 1 patient with chondrosarcoma, two cases with Meningioma and cholesterol granuloma of clival localization, achieving gross total resection in 2 of them and in the rest subtotal resection, all improved neurological symptoms. Complications, partial dehiscence of the velopalatine surgical wound 1 case, 1 lesion of the left vertebral artery and 1 case decompensated Diabetes Mellitus and hemoneumothorax during stay in the ICU, which death 7 days after surgery and 1 CSF fistula. Conclusions: Both, the transoral and endonasal endoscopic approaches have their precise indications in each particular case, having their indications well defined. To achieve this, we must have neuroimaging studies and be able to correctly define the selection of the surgical approach.

Treatment strategy for multisegmental cervicomedullary ependymoma: illustrative case

BACKGROUND Сervicomedullary ependymoma (CME) is a rare tumor of the central nervous system. The CME treatment strategy is insufficiently represented in the literature and is a complex task for neurosurgeons. OBSERVATIONS The authors describe an infrequent case of a large multisegmental CME that extended from the medulla oblongata to the cervical spinal cord at the level of the sixth cervical vertebra in a 21-year-old female. Neurological disorders presented with headache, dysphagia, hypophonia, and weakness in the limbs. Subtotal removal of the tumor was performed according to intraoperative neurophysiological monitoring (IONM) results. A wait-and-see approach with patient follow-up was chosen. LESSONS Total tumor removal of the CME is the most important favorable prognostic factor. Subtotal resection can be considered if the borders of the tumor are unclear and the result of IONM is unfavorable. The role of postoperative radiation therapy in the case of subtotal removal of the tumor remains controversial.

Skull Base Meningiomas: Is Surgical Resection Enough? Outcome Evaluation and Prognostic Factors Analysis in a Single-Center Cohort

Background Surgical resection represents the mainstay of treatment in skull base meningiomas (SBMs). Considering the high recurrence rate reported, an adjuvant radiation therapy (RT) treatment should be considered. The aim of this study was to evaluate the progression-free survival (PFS), overall survival (OS), and prognostic factors conditioning outcome. Methods Patients receiving surgical resection for grade I SBMs were included. The extent of resection (EOR) was dichotomized as gross total resection (GTR) and subtotal resection (STR). RT was administered only in patients receiving STR. Clinical outcome was evaluated by brain magnetic resonance imaging (MRI) performed every 6 months for the first year and yearly thereafter. Results From January 2000 to December 2015, 123 patients were treated. The majority were females (70.7%), with a Karnofsky Performance Score (KPS) ≥80 (95%), and symptoms at diagnosis (91%). GTR was performed in 30% of cases and STR in 70%. RT was performed in 18 (20.9%) patients at diagnosis and in 29 (33.7%) patients at progression. Improvement or stability of neurologic status was obtained in 78.9% of patients. The median follow-up time was 91 months (range: 40–230 months). Local recurrence occurred in 34 (27.6%) patients at a median time of 45 months (range: 6–214 months). The median, 2-, 5-, and 10-year PFS were 193 months, 89.3, 81.8, and 72.5%, respectively. On univariate and multivariate analyses, factors impacting on PFS were EOR, tumor location, neurologic postoperative status, and adjuvant RT in STR. Conclusions A safe surgical resection followed by RT adjuvant treatment could represent the better choice to obtain local control maintaining neurologic integrity. Our data underlined the value of adjuvant RT in incompletely resected meningiomas.

A case of mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy (MOGHE)

Recently, in the scientist community of specialists dealing with structural epilepsy, it has been noticed an increasing interest in a special form of cortical development disorder not to be included in the ILAE Classification of the epilepsies the 2017 revision. It is so-called mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy (MOGHE). There are a number of publications devoted to the neuroimaging features of MOGHE, which are possible to distinguish from other epileptogenic substrates in comparisons with clinical/anamnestic data and dynamic observation. Our paper describes the case of a patient under 6 years suffering from pharmacoresistant epilepsy with histologically confirmed MOGHE, and having undergone the procedure of epileptic surgery. MRI showed an increased intensity of the T2/FLAIR signal from the white matter in combination with signs of laminar hyperintensivity, regional sulcation disturbance, smoothness of gray-white matter demarcation in the right frontal lobe. A signal intensification from the white matter with the formation similarity of the «transmantl» sign and further pronounced smoothness of the gray-white matter demarcation was observed on dynamic MRI. These changes were estimated as focal cortical dysplasia. Pre-surgical examination revealed a correlation of epileptiform activity with MRI changes. The subtotal resection of the right frontal lobe and the morphological conclusion established the presence of MOGHE was performed.

Postoperative Adjuvant Radiotherapy in Atypical Meningioma Patients: A Meta-Analysis Study

Background and PurposeConsensus regarding the need for adjuvant radiotherapy (RT) in patients with atypical meningiomas (AMs) is lacking. We compared the effects of adjuvant RT after surgery, gross total resection (GTR), and subtotal resection (STR) on progression-free survival (PFS) and overall survival (OS) in patients with AMs, respectively.MethodsWe performed a systematic review and meta-analysis of the literature published in PubMed, Embase, and the Cochrane Library from inception to February 1, 2021, to identify articles comparing the PFS and OS of patients receiving postoperative RT after surgery, GTR and STR.ResultsWe identified 2307 unique studies; 24 articles including 3078 patients met the inclusion criteria. The sensitivity analysis results showed that for patients undergoing undifferentiated surgical resection, adjuvant RT reduced tumor recurrence (HR=0.70, p&lt;0.0001) with no significant effect on survival (HR=0.89, p=0.49). Postoperative RT significantly increased PFS (HR=0.69, p=0.01) and OS (HR=0.55, p=0.007) in patients undergoing GTR. The same improvement was observed in patients undergoing STR plus RT (PFS: HR=0.41, p&lt;0.00001; OS: HR=0.47, p=0.01). A subgroup analysis of RT in patients undergoing GTR showed no change in PFS in patients undergoing Simpson grade I and II resection (HR=1.82, p=0.22) but significant improvement in patients undergoing Simpson grade III resection (HR=0.64, p=0.02).ConclusionRegardless of whether GTR or STR was performed, postoperative RT improved PFS and OS to varying degrees. Especially for patients undergoing Simpson grade III or IV resection, postoperative RT confers the benefits for recurrence and survival.