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.

[Chordoid gliomas of the third ventricle]. / Opyt lecheniya khordoidnykh gliom III zheludochka.

BACKGROUND: Chordoid glioma is a rare slow-growing tumor of the central nervous system. Available world experience includes no more than 200 cases (lesion of the third ventricle in absolute majority of cases). Recognition and treatment of chordoid glioma are currently difficult problems due to small incidence of this disease. OBJECTIVE: To describe clinical manifestations and surgical treatment of chordoid glioma of the third ventricle considering literature data and own experience. MATERIAL AND METHODS: There were 12 patients (6 men and 6 women) with chordoid glioma between 2004 and 2023 (10 patients with lesion of the third ventricle, 1 - lateral ventricle, 1 - pineal region). Only patients with tumors of the third ventricle were analyzed. RESULTS: Total and subtotal resection was performed in 1 and 3 cases, respectively. Five patients underwent partial resection, 1 patient underwent biopsy. The follow-up data were available in 7 out of 10 patients (mean 25 months). Radiotherapy was performed in 4 patients (continued tumor growth in 2 cases). One patient died. CONCLUSION: Chordoid glioma is a benign tumor predominantly localized in the third ventricle. Preoperative MRI and CT in some cases make it possible to suspect chordoid glioma and differentiate this tumor from craniopharyngioma, meningioma and pituitary adenoma by such signs as isointense signal in T1WI, hyper- or isointense signal in T2WI, homogeneous contrast enhancement and edema of basal ganglia in T2 FLAIR images. The only effective treatment for chordoid glioma is surgery. Total resection is often impossible or extremely dangerous due to location of tumor, large size and invasion of the third ventricle. Postoperative mental disorders and diabetes insipidus, including severe hypernatremia, are common that requires mandatory monitoring of water and electrolyte balance.

Subependymomas of the fourth ventricle: To operate or not to operate?

BACKGROUND: There is a paucity of literature regarding the clinical characteristics and management of subependymomas of the fourth ventricle due to their rarity. Here, we describe the operative and non-operative management and outcomes of patients with such tumors. METHODS: This retrospective single-institution case series was gathered after Institutional Review Board (IRB) approval. Patients diagnosed with a subependymoma of the fourth ventricle between 1993 and 2021 were identified. Clinical, radiology and pathology reports along with magnetic resonance imaging (MRI) images were reviewed. RESULTS: Patients identified (n = 20), showed a male predominance (n = 14). They underwent surgery (n = 9) with resection and histopathological confirmation of subependymoma or were followed with imaging surveillance (n = 11). The median age at diagnosis was 51.5 years. Median tumor volume for the operative cohort was 8.64 cm3 and median length of follow-up was 65.8 months. Median tumor volume for the non-operative cohort was 0.96 cm3 and median length of follow-up was 78 months. No tumor recurrence post-resection was noted in the operative group, and no tumor growth from baseline was noted in the non-operative group. Most patients (89 %) in the operative group had symptoms at diagnosis, all of which improved post-resection. No patients were symptomatic in the non-operative group. CONCLUSIONS: Surgical resection is safe and is associated with alleviation of presenting symptoms in patients with large tumors. Observation and routine surveillance are warranted for smaller, asymptomatic tumors.

Resection of an Intraventricular Metastatic Tumor with Minimally Invasive Port Technique: 2-Dimensional Operative Video.

The use of minimally invasive port technology has been proposed as a safe method to reduce retractor-induced parenchymal injury, particularly for the resection of deep-seated lesions.1-6 A 69-year-old woman with a history of previous colon cancer surgery presented with gait disturbances and progressive headaches. Magnetic resonance imaging revealed a tumor involving the right ventricular atrium that appeared consistent with metastasis. A parieto-occipital craniotomy was performed on the basis of the preoperatively planned surgical trajectory (Video 1). After the dural incision, the arachnoid was opened down to the sulcus under visualization with microscope. Next, the ViewSite Brain Access system tubular retractor (VBAS; Vycor Medical Inc., Boca Raton, Florida, USA) was introduced toward the lesion under navigation guidance. Once the ventricular atrium was entered, the surface of the tumor came into view. It was coagulated and progressively debulked with ultrasonic aspirator. After the mass was adequately decompressed, a plane of dissection between the ependyma and the tumor could be developed with dynamic angulation of the port in order to allow better visualization. Finally, the tumor could be gently rolled away from the choroid plexus and removed. Meticulous hemostasis was achieved, and the tubular retractor was slowly removed. The patient recovered uneventfully without neurologic deficits on follow-up, and the postoperative magnetic resonance imaging evidenced a complete resection of the tumor. The video illustrates technical nuances and demonstrates the feasibility of minimal access port surgery for the resection of intraventricular lesions with low morbidity and mortality using microsurgical techniques. The patient consented to the publication of her image.

Staged Gamma Knife radiosurgery for a rosette-forming glioneuronal tumor of the fourth ventricle: a case report.

BACKGROUND: Rosette-forming glioneuronal tumor (RGNT) is a rare slow-growing neoplasm with mixed glial and neurocytic components. Surgical resection is the mainstay of treatment, whereas the role of adjuvant radiation therapies for residual or recurrent tumors has been poorly investigated. CASE PRESENTATION: We describe the case of a patient with a recurrent fourth ventricular RGNT who was treated with two-staged Gamma Knife radiosurgery (GKRS). GKRS was effective in controlling tumor growth and safe up to seven years from treatment. CONCLUSIONS: This case suggests that GKRS may be a safe and effective treatment for patients with recurrent or residual RGNT.

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.

Endoscopic endonasal approach to remove pediatric intraventricular tumors of the third ventricle.

PURPOSE: Pediatric intraventricular tumors of the third ventricle are among the most difficult-to-treat brain tumors. Recently, endonasal endoscopic surgery for suprasellar, third ventricle, and median clivus lesions has become the common procedure, and its indications are expanding to pediatric cases. We describe our strategy for endonasal endoscopic surgery for pediatric third ventricle tumors. METHOD: We report on surgical anatomies and our surgical procedures in detail, including case presentations. RESULT: Endoscopic endonasal surgery has the advantage of providing a wider view of the tumor site, hypothalamus, optic chiasm, and other critical structures. Good indications for the endoscopic endonasal approach for intra-third ventricular tumors are those arising from the floor of the third ventricle. In particular, craniopharyngioma, a typical pediatric suprasellar tumor, sometimes extends into the third ventricle, causing great operative difficulty. However, aggressive removal for long-term control while preserving memory and visual function is important. We perform surgery with a strategy of radically removing tumors without causing damage to visual or brain function, and we adopt the "4-hands technique by two neurosurgeons" in full endoscopic surgery to remove tumors safely and aggressively. CONCLUSION: We describe our strategy for endonasal endoscopic surgery for pediatric third ventricle tumors, especially those extending from the suprasellar region into the third ventricle, and present a representative case.

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.

Third Ventricular Subependymomas: Clinical Features and Outcomes Over Two Decades.

BACKGROUND: Subependymomas are uncommon, benign slow-growing neoplasms of the central nervous system preferentially arising within the fourth and lateral ventricles. Third ventricle involvement has been described rarely. The aim of this study is to provide the first systematic review of third ventricular subependymomas (TVSE) by analyzing all reported cases over 2 decades and describing a case example. METHODS: MEDLINE and Embase databases were searched for the 20 years ending January 1, 2022, using relevant MeSH and non-MeSH terms, including "subependymoma" and "third ventricle." Methodology followed PRISMA guidelines. RESULTS: Of 804 identified studies, 131 met inclusion eligibility. The literature yielded 17 patients with TVSE plus our example (18 total). Of these patients, 83% (15/18) presented in adulthood (average age, 42 ± 19 years), of whom 73% were women. The pediatric cohort age was 5 ± 1 years, 67% (4/6) of whom were girls. The most common presenting symptom in both cohorts was headache (80%), followed by memory disturbances and vomitus. In adults, symptomatic tumors were approached by open craniotomy in all but 1 case, most using a transcallosal approach. Gross total resection was obtained in 73%. A ventriculoperitoneal shunt was inserted in 2/15 adult and 4/6 pediatric patients. Overall, both cohorts showed symptomatic improvement without disease recurrence. One patient died perioperatively. CONCLUSIONS: Subependymomas should be considered in the differential diagnosis of third ventricular tumors. The clinical presentation of TVSE mainly parallels hydrocephalus symptoms and, hence, awareness is of vital importance for timely treatment. The surgical goal should be gross total resection, which can be curative and offers greatest clinical improvement across the population.

Role of intra-operative squash cytology in rosette-forming glioneuronal tumor of the fourth ventricle: a case report.

Rosette-forming glioneuronal tumor (RGNT) of the 4th ventricle is a newly described WHO grade I brain tumor included in recent WHO classification of CNS tumors. It is a biphasic tumor thought to originate from pluripotent progenitor cells of subependymal plate. Intra-operative diagnosis plays an important role, as complete surgical excision is the treatment of choice. We are reporting a case of RGNT in a 19 years-old young male emphasizing the intra-operative pathological pointers and their role in accurate diagnosis for the suitable surgical intervention.

[Neuroendoscopic Surgery for Pediatric Intraventricular Tumors].

Various tumors are found in ventricles and tissues surrounding the ventricles in children, adolescents, and young-adult patients. They cause cerebrospinal fluid pathway obstruction, resulting in hydrocephalus and isolated ventricles. In this paper we describe a neoplasm arising from the lateral and third ventricle. Intraventricular tumors are good candidates for neuroendoscopic surgery. Firstly, minimally invasive and established procedures, neuroendoscopic biopsy with flexible endoscopy or rigid endoscopy, are performed. These endoscopic biopsies can be performed concurrently with other procedures, such as third ventriculostomy or septostomy, to improve cerebrospinal fluid circulation. Neuroendoscopic biopsy for intraventricular tumor is associated with high diagnostic yield and relatively low incidence of morbidity and mortality, compared with open surgery. Endoscopic diagnostic procedure also can be applied in patients without ventricular dilatation, assisted by neuronavigation or echo-guided equipment. Secondly, neuroendoscopic cylinder surgery also makes it possible to remove intraventricular tumors with less damage. The two hands technique with rigid endoscopy helps debulking and hemostasis during tumor removal. Further developments in endoscopic equipment and training systems aimed at enhancing cooperation between the operator and assistant should be expected in the future. Neuroendoscopic resection appears to be a safe and reliable treatment option for patients with intraventricular tumors.

High-Grade Supratentorial Intraventricular Tumours in Adults.

High-grade Intraventricular tumours are rarely encountered. Many of these tumours will have similar patterns on imaging of signal intensity and contrast enhancement. As IVT are regularly not targetable by radiation or systemic therapy, until now, surgical resection presents the treatment of choice.

Minimally Invasive Resection of Intraventricular Pilocytic Astrocytoma Using the Aurora Surgiscope in an Adult Patient: Technical Note.

BACKGROUND: Pilocytic astrocytomas account for approximately 5%-6% of all gliomas and are most commonly diagnosed between the ages of 8 and 13 years. Although they may occur throughout the neuraxis, approximately two thirds arise from the cerebellum and optic pathway. Other locations of origin include midline structures such as thalamus, hypothalamus, and periventricular regions. Surgical approaches to lateral or third ventricular tumors include anterior transcallosal, subfrontal translamina terminalis, and anterior transcortical approaches. The Aurora Surgiscope is a single-use, disposable minimally invasive neurological endoscope designed for intraparenchymal hemorrhage evacuation. We present the successful use of this system to aid resection of a large intraventricular pilocytic astrocytoma. METHODS: A 29-year-old man presented with signs of developing hydrocephalus and was found to have a large intraventricular tumor, which was later identified to be a rare intraventricular pilocytic astrocytoma. A ventriculostomy was performed as a temporizing measure, and he was transferred to our tertiary care facility for surgical management. Sulcal dissection was performed, and the endoscope was inserted to create a minimally invasive corridor to the lateral ventricle. Using the endoscope, bimanual surgery using multiple instruments simultaneously was possible and enabled gross total resection of the tumor. RESULTS: The patient tolerated the procedure well and was discharged at his neurological baseline level. CONCLUSIONS: Extensive sulcal dissection preceding placement of the endoscope allowed access to the intraventricular space with minimal passage of parenchymal tissue. High-definition visualization was provided and allowed the operating surgeon to freely use both hands during surgery.

Non-enhancing intraventricular tumours in adults.

Intraventricular tumours are relatively uncommon among all brain tumours, and non-enhancing lesions, mostly subependymoma, are even less frequently reported. Select cases of subependymoma can show variable contrast enhancement as well. Gross total surgical resection is recommended for treating these lesions, with no significant role of adjuvant chemotherapy or radiotherapy.

Grading Trigone Meningiomas Using Conventional Magnetic Resonance Imaging With Susceptibility-Weighted Imaging and Perfusion-Weighted Imaging.

OBJECTIVE: To compare conventional magnetic resonance imaging (MRI), susceptibility-weighted imaging (SWI), and perfusion-weighted imaging (PWI) characteristics in different grades of trigone meningiomas. METHODS: Thirty patients with trigone meningiomas were enrolled in this retrospective study. Conventional MRI was performed in all patients; SWI (17 cases), dynamic contrast-enhanced PWI (10 cases), and dynamic susceptibility contrast PWI (6 cases) were performed. Demographics, conventional MRI features, SWI- and PWI-derived parameters were compared between different grades of trigone meningiomas. RESULTS: On conventional MRI, the irregularity of tumor shape (ρ = 0.497, P = 0.005) and the extent of peritumoral edema (ρ = 0.187, P = 0.022) might help distinguish low-grade and high-grade trigone meningiomas. On multiparametric functional MRI, rTTPmax (1.17 ± 0.06 vs 1.30 ± 0.05, P = 0.048), Kep, Ve, and iAUC demonstrated their potentiality to predict World Health Organization grades I, II, and III trigone meningiomas. CONCLUSIONS: Conventional MRI combined with dynamic susceptibility contrast and dynamic contrast-enhanced can help predict the World Health Organization grade of trigone meningiomas.

Primary intraventricular tumors - Imaging characteristics, post-treatment changes and relapses.

Primary intraventricular neoplasms are rare tumors that originate from the ependymal or subependymal, septum pellucidum, choroid plexus and the supporting arachnoid tissue. Knowledge of the common locations of these tumors within the ventricular system, together with key imaging characteristics and presentation age, can significantly narrow the differential diagnosis. In 2016, the WHO reorganized the classification of several primary CNS tumors by combining histopathological and molecular data. This study highlights the imaging characteristics, histopathological and molecular data, treatment strategies and post-treatment changes of primary intraventricular tumors. Molecular-based diagnosis can not only aid in patient stratification and personalized treatment, but it can also provide prognostic and predictive value independent of WHO classification.

Radiologic and Clinical Outcomes of Stereotactic Radiosurgery for Intraventricular Metastases.

OBJECTIVE: The optimal management of intraventricular metastases remains debatable. The aim of this study is to define the safety and efficacy of Gamma-Knife radiosurgery in the treatment of intraventricular metastases. METHODS: This retrospective, single-center study involved patients that were treated with stereotactic radiosurgery (SRS) for intraventricular metastases. The study end points included SRS-related toxicity, local and distal intracranial tumor control, as well as the incidence of post-treatment hydrocephalus and leptomeningeal dissemination. Factors associated with radiologic and clinical outcomes were also analyzed. RESULTS: The cohort included 17 consecutive patients who underwent stereotactic radiosurgery for treatment of 41 intracranial metastases, of which 23 were primary intraventricular (intraventricular metastasis). Median overall survival from primary tumor diagnosis and from SRS treatment were 28 and 5 months, respectively. With a median radiological follow-up of 3 (interquartile range 3) months, 7 patients (41.18%) experienced overall intracranial disease progression, whereas 7 (27.27%) intraventricular metastases progressed radiologically. Four (23.53%) and 3 (17.65%) patients developed hydrocephalus and leptomeningeal dissemination post-SRS, respectively. Four patients (23.53%) died due to intracranial disease progression. CONCLUSIONS: SRS offers a reasonable chance of local tumor control for patients with intraventricular brain metastasis. However, the risk of hydrocephalus and leptomeningeal spread of disease is not inconsequential and merits close follow-up for patients with brain metastasis involving the ventricular system.