Suprasellar masquerader: Chordoid glioma.

BACKGROUND: Chordoid glioma is a rare well-circumscribed glial neoplasm arising in adults and predominantly affects females. Tanycytes of the third ventricle have been proposed as the cell of origin owing to its location. It is characterized by chordoid features with myxoid and inflammatory stroma and recurrent PRKCA p.D463H missense mutation. CASE REPORT: We present two cases (30-year-old female and 45-year-old male) with similar complaints of behavioral change and headache. Midline suprasellar homogeneously enhancing mass was seen on contrast-enhanced magnetic resonance imaging. Histopathology and immunohistochemistry was characteristic of chordoid glioma with cords and clusters of epithelioid cells arranged in a solid pattern. There were variable amounts of myxoid stroma and lymphoplasmacytic infiltrate. No mitosis, necrosis, or brain invasion was noted. The cells expressed strong diffuse positivity for glial fibrillary acid protein (GFAP) and weak nuclear thyroid transcription factor (TTF-1). Epithelial membrane antigen (EMA)and brachyury were negative. Subsequently, the lady underwent gross total excision and died soon after the operation. The male patient received radiotherapy and is currently doing well after 6 months of follow-up. CONCLUSION: The rare occurrence as well as the radiological and morphological overlaps in chordoid gliomas make them a true masquerader. Combination of GFAP and TTF-1 in the immunohistochemical panel can be useful in differential diagnosis. Mainstay of treatment is complete surgical excision, with adjuvant radiotherapy becoming increasingly important.

Endoscopic Cylinder Surgery for Ventricular Lesions.

Cylinder retractors have been developed to reduce the risk of brain retraction injury during surgery by dispersing retraction pressure on the brain. In recent years, various types of cylinder retractors have been developed and widely used in neurosurgery. The ventricles, being deep structures within the brain, present an effective area for cylinder retractor utilization. Endoscopy provides a bright, wide field of view in the deep surgical field, even through narrow corridors.This chapter introduces surgical techniques using an endoscope through a cylinder. Given the deep and complex shapes of the ventricles, preoperative planning is paramount. Two main surgical techniques are employed in endoscopic cylinder surgery. The wet-field technique involves the continuous irrigation of artificial cerebrospinal fluid (CSF) during the procedure, maintaining ventricle shape with natural water pressure, facilitating tumor border identification, and achieving spontaneous hemostasis. Conversely, the dry-field technique involves CSF drainage, providing a clear visual field even during hemorrhage encounters. In intraventricular surgery, both techniques are used and switched as needed.Specific approaches for lateral, third, and fourth ventricular tumors are discussed, considering their locations and surrounding anatomical structures. Detailed intraoperative findings and strategies for tumor removal and hemostasis are presented.Endoscopic cylinder surgery offers a versatile and minimally invasive option for intraventricular tumors, leading to improved surgical outcomes. Overall, this technique enhances surgical precision and patient outcomes in intraventricular tumor cases.

Intraventricular Tumors: Surgical Considerations in Lateral and Third Ventricular Tumors.

Management of lateral and third ventricular tumors has been a challenge for neurosurgeons. Advances in imaging and pathology have helped in a better understanding of the treatment options. Technical refinement of microsurgical technique and addition of endoscopy has enabled more radical excision of tumors, when indicated, and added more safety.A proper understanding of the pathology at various ages and treatment options is continuously evolving. Many pediatric tumors are amenable to conservative surgical methods with effective complementary treatments. However, radical surgery is required in many adults as the main treatment and for many benign tumors. Various intraventricular lesions encountered and their surgical management is reviewed here for their efficacy, safety, and outcome, encompassing changes in our practice over the last 20 years.

The Anterior Interhemispheric Transcallosal Approach to the Ventricles: How We Do It.

Intraventricular tumors of the lateral and third ventricles are relatively rare, accounting for 1-2% of all primary brain tumors in most large series [1-4]. They can be uniquely challenging to approach due to their deep location, propensity to become large before they are discovered, and association with hydrocephalus [5, 6]. The surgeon's goal is to develop a route to these deep lesions that will cause the least morbidity, provide adequate working space, and achieve a complete resection. This must be performed with minimal manipulation of the neural structures encircling the ventricles, avoiding functional cortical areas, and acquiring early control of feeding vessels [7, 8].

Endoneurosurgical Resection of Parenchymal and Intraventricular Lesions Using Tubular Retraction System.

OBJECTIVE: Endoscopic surgery has emerged in the recent years as an alternative to the conventional microsurgical approaches for removal of the deep-seated brain and intraventricular tumors. Endoport has enhanced the tumor access and visualization without any significant brain retraction. In this chapter, we describe the surgical technique of the endoscopic excision of the deep-seated intra-axial brain tumors using tubular retraction system with review of the literature. METHODS: The endoscopic endoport technique that we use at our institution for the surgical management of intraventricular and intraparenchymal brain tumors has been described in details with illustrations. RESULTS: Results from the literature review of brain parenchymal and intraventricular port surgery were analyzed, and the feasibility and safety of this technique were discussed. Surgical complication avoidance and management were highlighted. The port technique offers numerous potential advantages, including: (1) reducing focal brain injury by distributing retraction forces homogenously; (2) minimizing white matter disruption and the risk of fascicles injury during cannulation; (3) ensuring stability of the surgical corridor during the procedure; (4) preventing inadvertent expansion of the corticectomy and white fiber tract dissection throughout surgery; (5) protecting the surrounding tissues against iatrogenic injuries caused by instrument entry and reentry. CONCLUSION: The endoport-assisted endoscopic technique is a safe and minimally invasive method that offers an effective alternative option for resection of intraventricular and parenchymal brain lesions. Excellent outcome comparable to other surgical approaches can be achieved with acceptable complications.

Endoscopic Supraorbital Translaminar Approach.

Surgical selection for third ventricle tumors demands meticulous planning, given the complex anatomic milieu. Traditional open microsurgical approaches may be limited in their access to certain tumors, prompting the exploration of alternative techniques. The endoscopic supraorbital translaminar approach (ESOTLA) has emerged as a promising alternative for managing these tumors. By combining a minimally invasive keyhole approach with endoscopic visualization, the ESOTLA provides enhanced illumination and a wider angle of view within the third ventricle. This unique advantage allows for improved access to retrochiasmatic tumors and reduces the need for frontal lobe and optic chiasm retraction required of microscopic techniques, decreasing the risk of neurocognitive and visual deficits. Complications related to the ESOTLA are rare and primarily pertain to cosmetic issues and potential compromise of the hypothalamus or optic apparatus, which can be minimized through careful subarachnoid dissection. This chapter offers a comprehensive description of the technical aspects of the ESOTLA, providing insights into its application, advantages, and potential limitations. Additionally, a case description highlights the successful surgical extirpation of an intraventricular papillary craniopharyngioma via the ESOTLA followed by targeted therapy. To better illustrate the stepwise dissection through this novel approach, a series of cadaveric and intraoperative photographs are included.

The telovelar approach for fourth ventricular tumors in children: is removal of the posterior arch of C1 necessary?

PURPOSE: Various surgical nuances of the telovelar approach have been suggested. The necessity of removing the posterior arch of C1 to accomplish optimal tumor exposure is still debated. Therefore, we report on our experience and technical details of the fourth ventricular tumor resection in a modified prone position without systematic removal of the posterior arch of C1. METHODS: A retrospective analysis of all pediatric patients, who underwent a fourth ventricular tumor resection in the modified prone position between 2012 and 2021, was performed. RESULTS: We identified 40 patients with a median age of 6 years and a M:F ratio of 25:15. A telovelar approach was performed in all cases. In 39/40 patients, the posterior arch of C1 was not removed. In the remaining patient, the reason for removing C1 was tumor extension below the level of C2 with ventral extension. Gross or near total resection could be achieved in 34/39 patients, and subtotal resection in 5/39 patients. In none of the patients, a limited exposure, sight of view, or range of motion caused by the posterior arch of C1 was encountered, necessitating an unplanned removal of the posterior arch of C1. Importantly, in none of the cases, the surgeon had the impression of a limited sight of view to the most rostral parts of the fourth ventricle, which necessitated a vermian incision. CONCLUSION: A telovelar approach without the removal of the posterior arch of C1 allows for an optimal exposure of the fourth ventricle provided that critical nuances in patient positioning are considered.

Anatomy of the posterior Fossa: a comprehensive description for pediatric brain tumors.

Posterior fossa tumors are the most common pediatric brain tumors, and present unique challenges in terms of their location and surgical management. The posterior fossa comprehends complex anatomy and represents the smallest and deepest of the three cranial base fossae. An in-depth understanding of posterior fossa anatomy is crucial when it comes to the surgical resection of pediatric brain tumors. Mastering the knowledge of posterior fossa anatomy helps the neurosurgeon in achieving a maximal and safe volumetric resection, that impacts in both overall and progression free survival. With the advancements in microsurgery, the telovelar approach has emerged as the workhorse technique for the resection of posterior fossa tumors in pediatric patients. This approach involves meticulously dissecting of the natural clefts present in the cerebellomedullary fissure, making a comprehensive understanding of the underlying anatomy key for its success.

A comprehensive approach to lateral ventricular tumor resection: techniques, technologies, and outcomes.

This study reviews lateral ventricular tumors (LVTs), which are rare brain lesions accounting for 0.64-3.5% of brain tumors, and the unique challenges they present due to their location and growth patterns. Once deemed inoperable, advancements in microneurosurgery, imaging, and tumor pathobiology have significantly improved treatment outcomes. This letter summarizes recent studies and key findings in the management of LVTs. Research by S.A. Maryashev et al. identified risk factors for early hemorrhagic complications following the surgical resection of lateral ventricular neoplasms, highlighting the significance of patient characteristics, tumor location, and surgical approach. The study found that factors such as gender, hydrocephalus, tumor blood flow, and Evans index correlate with a higher risk of hemorrhage, with the transcallosal approach having a greater risk compared to the transcortical approach. The utilization of navigation technologies, including fMRI, neuronavigation, and intraoperative brain mapping, has been shown to reduce surgical complications and enhance patient outcomes in the treatment of lateral ventricular meningiomas. Moreover, endoscopic and endoport-assisted endoscopic techniques have proven to be valuable in intraventricular tumor surgery, enabling minimally invasive procedures with better visualization and fewer complications. The integration of advanced surgical techniques, neuroimaging, and neurophysiological monitoring emphasizes the necessity of a multidisciplinary approach to optimize patient outcomes. To improve the study's validity and applicability, further research with larger sample sizes and advanced statistical analyses is needed. This letter advocates for the continued exploration of innovative surgical techniques and technologies to enhance the management of lateral ventricular tumors.

Risk factors for early intraventricular hemorrhagic complications following lateral ventricular tumor surgery.

The complications anticipated in the postoperative period after surgical resection of lateral ventricular neoplasms (LVN) are hemorrhage, hydrocephalus. At the N.N. Burdenko Neurosurgery Center, 48 patients with LVN underwent surgical resection. We focused on the correlation between approaches to the ventricular system on one hand and the extent of resection and incidence of complications on the other based on anatomical and perfusion characteristics estimated via preoperative magnetic resonance imaging (MRI) with arterial spin labeling perfusion. By eliminating the surgical approach as a potential risk factor, we were able to demonstrate the correlation between the frequency of postoperative hemorrhage, the Evans index value, patient's gender, tumor blood flow (nTBF) and the location of the mass in the anterior horn of the lateral ventricle. The risk of hemorrhage depends on the patient's gender, presence of hydrocephalus, location of the mass and tumor blood flow values. The risk increases along with increase in Evance index and nTBF values.

Surgical outcomes of trigonal intraventricular meningiomas: a single-centre study.

Trigonal meningiomas are rare intraventricular tumours that present a surgical challenge. There is no consensus on the optimal surgical approach to these lesions, though the transtemporal and transparietal approaches are most frequently employed. We aimed to examine the approach-related morbidity and surgical nuances in treating trigonal meningiomas. This retrospective review assimilated data from 64 trigonal meningiomas operated over 15 years. Details of clinicoradiological presentation, surgical approach and intraoperative impression, pathology and incidence of various postoperative deficits were recorded. In our study, Trigonal meningiomas most frequently presented with headache and visual deterioration. The median volume of tumours was 63.6cc. Thirty-one meningiomas each (48.4%) were WHO Grade 1 and WHO Grade 2, while 2 were WHO Grade 3. The most frequent approach employed was transtemporal (38 patients, 59.4%), followed by transparietal (22 patients, 34.4%). After surgery features of raised ICP and altered mental status resolved in all patients, while contralateral limb weakness resolved in 80%, aphasia in 60%, seizures in 70%, and vision loss in 46.2%. Eighteen patients (28.13%) developed transient postoperative neurological deficits, with one patient (1.5%) developing permanent morbidity. Surgery for IVMs results in rapid improvement of neurological status, though visual outcomes are poorer in patients with low vision prior to surgery, longer duration of complaints and optic atrophy. The new postoperative deficits in some patients tend to improve on follow up. Transtemporal and transparietal approaches may be employed, based on multiple factors like tumour extension, loculation of temporal horn, size of lesion with no significant difference in their safety profile.

Technical aspects of fourth ventricle ependymomas in adults: how I do it.

BACKGROUND: Ependymomas in the fourth ventricle in adults are rare entity. Surgical treatment of adult ependymomas is the only treatment modality since no other effective alternative is available. Radical resection often means cure but it is hindered by the nature and location of the lesion. METHODS: Technical aspects of the fourth ventricle ependymoma surgery in adults are discussed. Anatomy of the area is provided with the step-by-step surgical algorithm. CONCLUSION: Radical resection of low-grade ependymoma with a detailed understanding of the anatomy in this area is vital considering the high effectiveness of the treatment and its excellent prognosis.

How I do it? Resection of left ventricular central neurocytoma via trans-sulcal approach.

BACKGROUND: The management of lateral ventricle tumors requires a balance between maximizing safe resection and preserving neurological function. METHOD: The authors present a successful case of a left lateral ventricular central neurocytoma resection. The trans-superior frontal sulcus approach was employed, providing a safe corridor while minimizing damage to the surrounding neuroanatomy. The use of an endoscope further facilitated the procedure, enabling the confirmation of complete tumor removal and the preservation of deep venous drainage and periventricular structures. CONCLUSION: This case highlights the utility of the trans-sulcal approach and the benefits of endoscopic assistance in the management of lateral ventricle tumors.

Stereotactic radiosurgery for intraventricular meningioma: a systematic review and meta-analysis.

BACKGROUND: Intraventricular meningioma (IVM) is a rare subtype of intracranial meningioma, accounting for 9.8 to 14% of all intraventricular tumors. Currently, there is no clear consensus on which patients with IVM should receive conservative treatment, surgery, or stereotactic radiosurgery (SRS). This research aims to analyze the outcomes, including survival and recurrence rates of patients who undergo SRS for IVM as a primary or adjuvant treatment. METHODS: A systematic search was conducted in Scopus, Web of Science, PubMed, and Embase till June 5th 2023. Screening and data extraction were performed by two independent authors. Random-effect meta-analysis was performed to determine the tumor control proportion of IVM cases treated with SRS. Individual patient data (IPD) meta-analysis was performed for the progression-free survival (PFS) of the patients in the follow-up time. All analyses were performed using the R programming language. RESULTS: Out of the overall 132 records, 14 were included in our study, of which only 7 had enough data for the meta-analysis. The tumor control proportion was 0.92 (95% CI, 0.69-0.98) in patients who underwent SRS for primary IVM. The overall tumor control in both primary and adjuvant cases was 0.87 (95% CI, 0.34-0.99). the heterogeneity was not significant in both meta-analyses (P = 0.73 and P = 0.92, respectively). Post-SRS perifocal edema occurred in 16 out of 71 cases (0.16; 95% CI, 0.03-0.56), with no significant heterogeneity (P = 0.32). IPD meta-analysis showed a PFS of 94.70% in a 2-year follow-up. Log-rank test showed better PFS in primary SRS compared to adjuvant SRS (P < 0.01). CONCLUSIONS: According to this study, patients with IVM can achieve high rates of tumor control with a low risk of complications when treated with SRS, regardless of whether they have received prior treatment. Although SRS could be a promising first-line treatment option for asymptomatic IVM, its efficacy in symptomatic patients and its comparison with resection require further investigation.

Consensus guidelines for the management of primary supra-tentorial intraventricular tumour for low- and middle-income countries.

Almost any primary or metastatic brain tumour can manifest in intraventricular (IV) locations. These tumours may either originate within the ventricular system or extend into the IV space through growth. Such neoplasms represent a broad spectrum, with supratentorial IV tumours forming a heterogeneous group. This group includes primary ependymal tumours, central neurocytomas, choroid plexus tumours, and notably, meningiomas, as well as a variety of non-neoplastic, benign, glial, and metastatic lesions that can secondarily invade the IV compartment. Often presenting with nonspecific symptoms, these tumours can lead to delayed medical attention. The diversity in potential diagnoses, combined with their deep and complex locations, poses significant management challenges. This paper aims to delineate optimal management strategies, underscoring the importance of multidisciplinary care, especially in settings with limited resources, to effectively navigate the complexities associated with treating intraventricular brain tumours.

Successful Surgical Treatment of a Giant Intraventricular Meningioma: A Case Report and Literature Review.

In our study, we document the case of a 48-year-old patient who presented at our clinic with various neurological disturbances. Magnetic Resonance Imaging revealed the presence of an intraventricular meningioma located in the body of the left lateral ventricle measuring 60 mm in diameter. This tumor was classified as a giant meningioma, accompanied by a significant amount of digitiform-type edema. A surgical procedure was conducted, resulting in a gross total resection of the tumor. Histopathological analysis identified the tumor as a fibrous meningioma. Postoperative assessments, as well as follow-ups conducted at 3 months and 1 year post-surgery, indicated considerable neurological improvement. The patient exhibited a remission of hemiparesis and gait disturbances along with a marginal improvement in the status of expressive aphasia. This case report underscores the significance of achieving total and safe resection of the tumor and includes an analysis of various cases from the literature, particularly focusing on those that describe minimally invasive surgical approaches and highlight the benefits of radiosurgery in the treatment of giant intraventricular meningiomas.

[Disseminated ependymal dysembryoplastic neuroepithelial tumor: a case report and literature review]. / Disseminirovannaya vnutrizheludochkovaya dizembrioplasticheskaya neiroepitelial'naya opukhol': klinicheskii sluchai i obzor literatury.

Dysembryoplastic neuroepithelial tumor (DNET) is a benign mixed neuronal-glial neoplasm (WHO grade 1). DNET is most often localized in temporal lobes and found in children and young people with epilepsy. There a few cases of DNET in ventricular system with dissemination along the ependyma in the world literature. MATERIAL AND METHODS: We present a rare case of T1- and T2-negative ventricular system tumor. Only FIESTA imaging revealed dissemination with multiple focal lesions of the third ventricle, its bottom and lateral walls, anterior horns of lateral ventricles, cerebellar vermis, cervical and lumbar spinal cord. RESULTS: The patient underwent transcortical endoscopic biopsy of the third ventricle tumor with simultaneous ventriculoperitoneal shunting. DNET was diagnosed, and radiotherapy was subsequently performed. Literature data on this issue were analyzed. CONCLUSION: To date, disseminated forms of DNET are extremely rare. X-ray features and morphological results allow us to establish the correct diagnosis and determine further treatment strategy.

Incidentally exploring natural course of a rare entity: representative case for rosette-forming glioneuronal tumors.

Rosette-forming glioneuronal tumors (RGNT) are extremely rare mostly benign tumors of the central nervous system, which are often studied for its histological aspects despite relatively small numbers of clinical especially radiological knowledge.Despite the increasing number of publications on different localizations and treatment protocols, the morphologic and temporal development process of this rare tumor entity is not clear. We were able to coincidentally observe the entire course of the tumor growth of a RGNT on subsequent MRI examinations in a typical case with mild clinical symptoms and no other neurological illnesses, thus possible clinical complications were prevented.

Granular cell tumor of the neurohypophysis presenting as a third ventricle mass.

Granular cell tumors of the neurohypophysis (GCT) are rare benign neoplasms belonging, along with pituicytoma and spindle cell oncocytoma, to the family of TTF1-positive low-grade neoplasms of the posterior pituitary gland. GCT usually present as a solid sellar mass, slowly growing and causing compressive symptoms over time, occasionally with suprasellar extension. They comprise polygonal monomorphous cells with abundant granular cytoplasm, which is ultrastructurally filled with lysosomes. Here we report the case of a GCT presenting as a third ventricle mass, radiologically mimicking chordoid glioma, with aberrant expression of GFAP and Annexin-A, which lends itself as an example of an integrated diagnostic approach to sellar/suprasellar and third ventricle masses.

Transcallosal and endoscopic hybrid approach to a rare entity of pediatric intraventricular tumors-cribriform neuroepithelial tumor: a case report and literature review.

PURPOSE: Cribriform neuroepithelial tumor (CRINET) is a provisional category of intraventricular tumors, sharing similarities with AT/RTs, and there is a lack of data about its pathology, prognosis, and surgical approaches in the literature. We have been challenged to describe the surgical approach to a rare case of CRINET and describe the intraoperative features since none has been described before. Surgical resection and chemotherapy hold a great importance of favorable prognosis. METHODS: Twenty-month-old male with intraventricular tumor underwent transcallosal intraventricular tumor resection and endoscopic intraventricular second look stages. The tumor was initially considered choroid plexus carcinoma and histopathological results pointed CRINET. The patient also received Ommaya reservoir for intrathecal chemotherapy employment. The patient's preoperative and postoperative MRI scans and tumor's pathological features are described with a brief history of the disease in the literature. RESULTS: Lack of SMARCB1 gene immunoreactivity and presence of cribriform non-rhabdoid trabecular neuroepithelial cells led to the CRINET diagnosis. The surgical technique helped us to approach directly into the third ventricle and perform total resection and intraventricular lavage. The patient recovered without any perioperative complications and is consulted pediatric oncology for further treatment planning. CONCLUSION: With our limited knowledge on the matter, our presentation may provide an inside to the course and progress of the CRINET as a very rare tumor and may help to set a basis for future investigations focused on its clinical and pathological features. Long courses of follow-up periods are required for establishing treatment modules and assessing the responses to surgical resection techniques and chemotherapy protocols.