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.

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.

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.

[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.

Neuroendoscopic transventricular transchoroidal approach for access to the posterior zone of the third ventricle or pineal region.

The endoscopic transventricular transchoroidal approach facilitates entry into the posterior part of the third ventricle, allowing a visualization field from the foramen of Monro to the pineal region through this anatomical corridor. Combined surgery to treat the target lesion and possible endoscopic third ventriculostomy (ETV) can be performed through a single burr hole. A detailed description of this surgical technique is given, and a series of cases from our center is presented. This retrospective study included patients with lesions in the pineal region or posterior zone of the third ventricle who underwent surgery between 2004 and 2022 in our center for tumor biopsy or endoscopic cyst fenestration. In nine cases, the transchoroidal approach was performed. Demographic and clinical variables were collected: sex, age at diagnosis, clinical presentation, characteristics of the lesion, pathological diagnosis, characteristics of the procedure, complications, subsequent treatments, evolution, follow-up time, and degree of success of the endoscopic procedure. The mean and range of the quantitative variables and frequency of the qualitative variables were analyzed, together with the statistical significance (p < 0.05). Surgical planning was carried out by performing a preoperative MRI, calculating the ideal entry point and trajectory for each case. The preoperative planning of the surgical technique is described in detail. Of our sample, 55.6% were women, with a mean age of 35 years (7-78). The most common clinical presentation was intracranial hypertension (55.6%), with or without a focus. Eight patients presented hydrocephalus at diagnosis. The most frequent procedure was endoscopic biopsy with ETV (66.7%). The pathological diagnosis varied widely. Procedure-related complications included one case of self-limited bleeding of the choroidal fissure at its opening and one intraventricular hemorrhage due to tumor bleeding in the postoperative period. Non-procedure-related complications comprised two ETV failures and one case of systemic infection, while late complications included one case of disease progression and one case of radionecrosis. Four patients died, one due to poor neurological evolution after post-surgical tumor bleeding and three due to causes unrelated to the procedure. The rest of the patients had a favorable evolution and were asymptomatic or stable. The transchoroidal approach through a single burr hole is a feasible and safe option for access to the posterior part of the third ventricle. Proper planning of each case is necessary to avoid complications.

Lymphoma of the central nervous system originating from the septum pellucidum region: Two case reports with literature review.

RATIONALE: Non-Hodgkin lymphoma affecting the brain, eyes, and cerebrospinal fluid without systemic spread is known as primary central nervous system lymphoma (PCNSL). While intracerebroventricular PCNSL is commonly found in the lateral ventricles and the third and fourth ventricles, the occurrence of PCNSL originating from the septum pellucidum is extremely rare. PATIENT CONCERNS: Two patients presented with recent memory loss and high cranial pressure. DIAGNOSES: Magnetic resonance imaging revealed a clear enhancing lesion in the septum pellucidum region. Pathological examination confirmed that both cases were primary large B-cell lymphoma GCB (germinal center B-cell-like) subtypes located in an "immune-privileged" area. INTERVENTIONS: Both patients underwent total tumor resection, and the procedures were successfully completed without surgical complications. OUTCOMES: Over a 1-year period, treatment included four cycles of high-dose methotrexate combined with temozolomide. During the follow-up period (19-23 months), no recurrence of the lymphoma was observed. LESSONS: In cases of PCNSL in the septum pellucidum, it is crucial to consider it as a potential differential diagnosis for intraventricular tumors. Surgical interventions should focus on maximizing tumor resection while ensuring the protection of critical structures like the fornix and peripheral neural components. The role of surgery compared to biopsy, as well as the long-term complications, necessitates extended follow-up. Additionally, an individualized treatment approach, considering factors such as age, Karnofsky performance score, and organ function assessment, can lead to positive outcomes.

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.

Clinical Results and Quality of Life After Transcortical Versus Transcallosal Resection of Adult Lateral and Third Ventricle Lesions: Long-Term Outcome.

BACKGROUND: Resection of intraventricular tumors can be achieved using 2 main operative approaches: transcallosal or transcortical. This study aims to describe preoperative and postoperative factors as well as quality of life (QoL) based on long-term results in these patients. METHODS: Patients underwent surgery of primary intraventricular lesions between 2007 and 2020 via a transcortical (group A) or transcallosal (group B) route. The main clinical parameters were completeness of resection, overall survival, surgical complications, postoperative neurologic deficits, and seizure rates. QoL was assessed using a modified questionnaire Short-Form 36 inventory. RESULTS: Forty patients (19 women and 21 men) met the inclusion criteria. Group A consisted of 26 patients (12 women and 14 men; median age 45.5 years ± 16.7 standard deviation) and had lower preoperative tumor volume (confounder) compared with group B (7 women and 7 men; age 50.0 ± 17.4 years). Gross total resection was achieved in 65% in group A and 71% in group B. Follow-up was 7.8 ± 3.9 years. New seizures/permanent neurologic deficits occurred in 27%/15% (group A) and 29%/29% (group B) and surgical complications in 23% of patients. Group B had a higher degree of memory impairment (21%) compared with group A (10%). QoL impairment was present in both groups mainly regarding physical role function and mental health index. CONCLUSIONS: Keeping in mind the limitations, transcallosal surgery was associated with a higher probability of neurologic deficits and memory impairment in our series. However, it had fewer surgical complications with similar gross total resection and seizure rates.

Stereotactic radiotherapy outcomes for intraventricular brain tumours in 11 dogs.

Published radiotherapy data for canine intraventricular tumours are limited. In this retrospective, longitudinal study (9/2011-2018), 11 dogs with intraventricular masses were treated with stereotactic radiotherapy (SRT). Pathologic diagnosis was available from surgery or necropsy in 6/11 cases, revealing choroid plexus papilloma (3) or carcinoma (2), and ependymoma (1). The remainder were magnetic resonance imaging (MRI)-diagnosed as suspected choroid tumours or ependymomas. Tumours were located in the third or lateral ventricle (8), fourth ventricle (2), and cerebellopontine angle (1). Surgery was performed in three dogs prior to radiotherapy, and all showed gross residual/recurrent disease at treatment. Dogs received 8 Gray × 3 fractions (7), or 15 Gray × 1 fraction (4). Ten dogs were deceased at analysis, and one was living. The estimated median overall survival time (OS) from first SRT treatment was 16.9 months (515 days, 95% CI 33-1593 days). The survival time for two pathology-diagnosed carcinoma dogs were 24 and 133 days, respectively, and survival time for dogs with moderate to marked ventriculomegaly (4/11) ranged from 24 to 113 days. A total of 10/11 showed clinical improvement per owner or clinician, but two had short-lived benefits and were euthanized within 6 weeks of SRT. Limited conclusions on radiation-specific complications are possible due to the small dataset and limited follow-up imaging. This study provides preliminary evidence that radiotherapy outcomes are variable with intraventricular tumours, and some long-term survivors are noted.

Neuroendoscopy: intraventricular and skull base tumor resection in children.

During the last 30 years, the neurosurgeons have witnessed a revolution in the practice of interventricular surgery. The advent of neuroendoscopy at the end of the 1980s has allowed a minimally invasive management of a very large series of pathologies in pediatric neurosurgery ranging from hydrocephalus to arachnoid cyst to intraventricular tumors. The progresses in the management of hydrocephalus, intracranial cyst, and the fluid filled collection nevertheless has been more rapid and radical due to the simpler equipment that is necessary to perform this kind of surgery. The intraventricular tumors instead have been addressed in a slower way, and for many years, the only endoscopic procedure that was allowed on interventricular tumors was a biopsy associated with the management of hydrocephalus. Only very small tumors have been considered operable for complete removal during many years due to the limitations of the neuroendoscopic equipment and to the small calibers of the working channel. More recently, the advent of new devices and new surgical techniques are offering new perspectives on the possibility of intraventricular tumor surgery in children. In this review, we describe the historical perspective of the learning curve of intraventricular tumor surgery under neuroendoscopic control and try to offer a view of the future perspective in the removal of larger intraventricular tumors, analyzing the main indications for intraventricular endoscopic tumor surgery. We offer as well an historical perspective of the evolution of skull base surgery and endonasal transsphenoidal approach for skull-based tumors in children. This kind of surgery that has acquired widespread acceptance for many pathologies in adult age has diffused more slowly in pediatric neurosurgery due to the anatomical limitation observed in these age range. Also in this field, the slow evolution of the technique and of the technology available to neurosurgeons has allowed a very significant expansion of indication for the minimally invasive removal of skull base tumors in children.

Cytological features of chordoid glioma: A case report with summary of prior published cases.

Chordoid glioma is an uncommon low-grade glioma and is a CNS WHO grade 2 tumour in the current WHO 2021 classification. Predominantly it is seen in the third ventricle and in young adults. Although the histological features of chordoid glioma are well documented, there is sparse literature describing its cytological features. Here we describe the squash cytological features of a case of chordoid glioma along with summary of prior published cases. The smears tend to be quite cellular, the cells show mild pleomorphism, anisonucleosis, and absent mitotic activity. The background shows a distinctive bluish myxoid stroma. It can be mistaken for high grade glioma on squash cytology.

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.

Hemostasis in a giant intraventricular tumor using a saline-cooled radiofrequency bipolar coagulator: technical note.

BACKGROUND: Meningiomas are relatively rare in children and tend to be intraventricular and cystic, with often malignant behavior. Complete excision is associated with the most favorable outcome; moreover, the size and extent of these lesions often make complete excision in one step impossible because of the risk of intraoperative death from uncontrollable hemorrhage. CASE PRESENTATION: A 10-year-old girl was admitted for headache in the last 3 months and was found to have a giant left intraventricular lesion with a volume of 166.63 cm3, which caused hydrocephalus and significant mass effect. Very large draining veins were evident within the tumor, draining into the thalamostriates and internal cerebral veins. Cerebral angiography showed multiple feeders originating mainly from branches of the posterior left choroidal artery with distal afferents that could not be embolized. Therefore, a left parietal transcortical approach was chosen. Given the vascularity of the tumor, saline-cooled radiofrequency coagulation (Aquamantys®) was used to reduce blood loss intraoperatively. Gross total resection (GTR) was achieved with an estimated blood loss of 640 mL. Pathology analysis was consistent with WHO grade 1 transitional meningioma. Postoperatively, the patient was neurologically intact, and MRI confirmed complete resection. CONCLUSION: Aquamantys® is a novel bipolar coagulation device that employs a new bipolar coagulation technique combining radiofrequency energy and saline to achieve hemostatic sealing by denaturing collagen fibers. This offers the possibility of achieving adequate hemostasis even in giant intraventricular tumors in infants to obtain GTR resection with minimal blood loss.

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.

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.

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.