Supratentorial Intraventricular Tumors: Experience from a Tertiary Neurosurgical Center in Nepal

To evaluate the outcome of management of intraventricular lesions operated by an open transcortical (nonendoscopic) approach. Case series. Department of Neurosurgery, Dow University of Health Sciences and Civil Hospital, Karachi, from January 2009 to December 2011, with six months follow-up. All cases with lesions in the lateral ventricle and anterior third ventricle operated by open transcortical approach, were included after informed consent. Total excision of the lesion was attempted in all cases. Patients were analyzed for outcome in terms of establishment of diagnosis, completeness of resection, morbidity and mortality. A total of 33 patients were operated. Twenty three were males and 10 were females. Colloid cyst was diagnosed in 14 patients (42%), giant cell astrocytoma and choroid plexus papilloma in 4 cases each and subependymoma and central neurocytoma in 2 cases each, epidermoid, choroid plexus carcinoma, oligodendroglioma, metastasis, meningioma, ependymoma and cavernous hemangioma in one patient each. The overall outcome could be labeled as good in 24 out of 33 cases (73%) and in 8 cases (24%) it was fair. One patient died due to ventriculitis and was categorized as poor outcome (3%). Postoperative complications included meningitis, seizures, intraventicular haemorrhage, subdural collection and transient hemiparesis. Nine patients had persistent hydrocephalus requiring ventriculoperitoneal shunt. Conventional open transcortical approach is still effective in getting conclusive biopsy or near total excision of the lesion with minimum morbidity.

In an effort to shed light upon the nature of the colloid cyst, the immunohistochemical properties of 21 examples of this lesion were compared with those of other neuraxial cysts and choroid plexus epithelium. The neuraxial cysts included the following: eight Rathke's cleft cysts, 25 pituitaries containing follicular cysts of the pars intermedia, and four enterogenous cysts. Fifteen examples of normal choroid plexus and 12 choroid plexus papillomas were studied as well. These lesions were examined for localization of the following antigens: cytokeratins, epithelial membrane antigen, secretory component, carcinoembryonic antigen, prealbumin, vimentin, glial fibrillary acidic protein (GFAP), S-100 protein, neuron-specific enolase, 68-kD neurofilament protein, chromogranin, serotonin, and lysozyme, and with Leu-7 monoclonal antibodies. Five colloid cysts were immunostained with monoclonal antibodies that were specific for Clara-cell antigens and surfactant, respectively. Sugar moieties were localized using Ulex europaeus I, and Ricinus communis agglutinin I lectins. All Rathke's cleft cysts and follicular cysts of the pars intermedia as well as three selected colloid cysts were examined for pituitary hormones. The epithelial cells of colloid and enterogenous cysts, as well as those lining follicular and Rathke's cleft cyst, showed uniformly strong reactivity for cytokeratins, epithelial membrane antigen, secretory component, and vimentin, and bound Ulex europaeus lectin. Occasional cells in colloid cysts were positive for Clara cell-specific antigens. Reaction for carcinoembryonic antigen was present on the apical surface of scattered cells of colloid, follicular, and Rathke's cleft cysts. Many cells of follicles in the pars intermedia as well as individual cells of five Rathke's cleft cysts were also immunoreactive for chromogranin, S-100 protein, GFAP, and pituitary hormones. Colloid and enterogenous cysts were negative for prealbumin, S-100 protein, GFAP, and neuron-specific enolase; in all but a few instances, they failed to bind Ricinus communis agglutinin. In contrast, normal choroid plexus and choroid plexus papillomas were positive for prealbumin, S-100 protein, neuron-specific enolase, cytokeratin, vimentin, and Ricinus communis agglutinin receptors; they lacked Ulex europaeus lectin, 56/66-kD cytokeratins, and epithelial membrane antigen. Unlike normal choroid plexus, choroid plexus papillomas were often GFAP-positive. All tissues studied were nonreactive for lysosome, serotonin, and neurofilament, and with Leu-7 antibodies. This study indicates that the immunophenotype of epithelium lining colloid cysts is similar to that of other cysts showing endodermal or ectodermal differentiation and to respiratory tract mucosa. Epithelium of colloid cysts is immunohistochemically different from that of normal or neoplastic choroid plexus. These findings indicate an endodermal rather than neuroepithelial nature for colloid cysts.

This study aims to identify the prevalence of and risk factors for seizure development after supratentorial brain tumor resection in pediatric patients. This could be used to guide the postoperative management and usage of anti-epileptic drugs (AEDs). Retrospective study was conducted for patients between 0 and 21 years with supratentorial tumor resection between 2005 and 2015 at a single institution. Two hundred patients (114 males/86 females) were identified. Median age at resection (±SD) was 9.025 ± 5.720 years and mean follow-up was 4 ± 2 years. Resection was gross total in 82 patients (41%) and partial in 118 patients (59%); 66 patients (33%) experienced preoperative seizures, and 67 patients (34%) experienced postoperative seizures; 18 patients (27%) had early seizures, and 49 patients (73%) had late seizures. Univariate analysis identified risk factors for postoperative seizures as: preoperative seizures (P < 0.001), age less than 2 years (P = 0.003), temporal location (P < 0.001), thalamic location (P = 0.017), preoperative hyponatremia (P = 0.017), World Health Organization grade (P = 0.008), and pathology (P = 0.005). Multivariate regression identified 5 robust risk factors: temporal location (odds ratio [OR] 4.7, 95% CI: 1.7-13.3, P = 0.003), age <2 years (OR 3.9, 95% CI: 1.0-15.4; P = 0.049), preoperative hydrocephalus (OR 3.8, 95% CI: 1.5-9.4; P = 0.005), preoperative seizure (OR 2.8, 95% CI: 1.2-6.5; P = 0.016) and parietal location (OR 0.25, 95% CI: 0.06-0.99; P = 0.049). Extent of resection did not correlate with seizure development (P > 0.05). This study reveals 5 risk factors for postoperative seizures after resection of supratentorial tumors. These factors should be considered in postoperative management of these patients.

Neoplasms of the Nervous System and Related Topics

Reliability of Imaging-Based Diagnosis of Lateral Ventricular Masses in Children

Introduction Glioblastoma is the most common and the most malignant type of gliomas. Cerebral hemispheres are usual locations for gliomas. Isolated third ventricular presentation is very rare for glioblastomas. A new case of isolated third ventricular glioblastoma has been presented in this report.Case descriptionA 36-year-old woman was admitted to outpatient clinic with headache, blurred vision and confusion. A head CT scan and MRI had showed third ventricular mass lesion with obstructive hydrocephalus. Previous to her admission to our clinic, a ventriculo-peritoneal shunt had been inserted and her hydrocephalus had been relieved to some extent in acute settings. In our clinic, stereotactic biopsy was performed and a second ventriculoperitoneal shunt was inserted from the opposite site. Histopathological diagnosis was glioblastoma. Radiotherapy and chemotherapy were started immediately after the surgery. Patient’s hydrocephalus has resolved and she was well at post-operative 6th month.Discussion and evaluationIn differential diagnosis list of the tumors presenting in the third ventricle, there are plenty of tumors such as colloid cyst, meningioma, germinoma, craniopharyngioma, lymphoma, choroid plexus papilloma, subependymal giant cell astrocytoma, chiasmatic and hypothalamic benign astrocytoma. Ring enhancement of this region pathology is a peculiar sign for glioblastoma, yet not pathognomonic. Tumor histology is crucial to yield the final diagnosis.ConclusionManagement of obstructive hydrocephalus, making histopathological diagnosis, starting adjuvant radiotherapy and chemotherapy in isolated third ventricular glioblastomas is a safe and effective approach when we consider malignant nature and intractable progress of glioblastomas.

The differential diagnosis of lateral ventricular tumors was explored by retrospective analysis of 47 pathologically proved cases identified by CT and/or MR imaging. Third ventricular tumors adjacent to the foramen of Monro (e.g., colloid cysts) were excluded. Forty-six patients underwent CT, and eight had MR imaging. The most common neoplasms were choroid plexus papilloma (10 cases) and meningioma (nine cases). Other common neoplasms included subependymoma (six cases), subependymal giant cell astrocytoma (five cases), and metastasis/lymphoma (four cases). Important features for differential diagnosis included age of the patient, the tumor's location within the lateral ventricle, and density on CT before IV administration of contrast material. Fifty percent of the tumors were in the ventricular atrium. All intraventricular tumor types (except subependymoma) showed contrast enhancement. MR was most useful in evaluating tumor location, size, and extent, but it did not help in eliminating alternative diagnoses. On the basis of patients' ages, specific tumor location, and the appearance on CT scans or MR images, an algorithm for differential diagnosis of lateral ventricular tumors was developed.

Children suffering from epilepsy with suspected low-grade tumors may benefit from a surgical approach that considers the epileptogenic zone, which can be more extensive than the tumor region. This study aimed to determine the prevalence of epilepsy in children undergoing supratentorial tumor resection and the factors predictive of postoperative seizure freedom in children with low-grade tumors. Subjects 3 months to 21 years undergoing supratentorial brain tumor resection between 2007 and 2011 were included in this retrospective study. Children with supratentorial, cortically based tumors and a preoperative diagnosis of epilepsy were considered epilepsy surgery candidates. Pre- and postoperative MRI were reviewed and scored for extent of resection, adjacent dysplasia, and remaining abnormal cortex postoperatively. The prevalence of seizures in all cases of supratentorial tumors was 46/87 (53 %). Eighteen were epilepsy surgery candidates. Eight of 18 (44 %) were seizure-free postoperatively with a mean follow-up of 39 months. Children who were seizure free postoperatively had tried fewer anticonvulsants than those with continued seizures (1.7 v. 2.9, p = 0.01). Presurgical evaluation was nonstandardized, and a more extensive workup and resection were performed in children who continued to have seizures postoperatively. All epilepsy surgery candidates had low-grade tumors on histological evaluation, indicating that a surgical approach that takes into consideration the epileptogenic zone is reasonable in this population. Gross total resection should be the goal, with additional attention to resection of the epileptogenic zone when located in the noneloquent cortex.

Surgical management of colloid cyst of the third ventricle—a study of 105 cases

Subependymal giant cell astrocytomas (SEGAs) are benign tumors, most commonly associated with tuberous sclerosis complex (TSC). Arising from the lateral ependymal surface, these tumors may obstruct one or both foramina of Monro, resulting in hydrocephalus and often requiring treatment. Although interhemispheric-transcallosal and transcortical-transventricular approaches have been the standard methods for resecting these tumors, advances in neuroendoscopic techniques have expanded SEGAs as a potential target for endoscopic resection. The authors present a case of an endoscopically resected SEGA with stereotactic guidance in a 4-year-old girl with TSC. A gross-total resection of an enlarging SEGA was achieved. This represents one of the early case reports of endoscopically resected SEGAs. Although recent advances in medical treatment for SEGAs with mammalian target of rapamycin (mTOR) pathway inhibitors have shown promising initial results, the long-term safety and efficacy of mTOR inhibitors has yet to be determined. The propensity of these tumors to cause obstructive hydrocephalus requires that a surgical option remain. Neuroendoscopic approaches may allow a safe and effective technique.

INTRODUCTION: Intraventricular brain tumors account for approximately 10% of all tumors of the central nervous system. Despite the fact that some of these tumors are aggressive high grade lesions, most of them are histologically benign and potentially curable after surgical resection. Considering the slow growth of this tumors, for a long time this lesions remain clinically asymptomatic, until they reach significant size, which makes surgical removal technically difficult. The aim of the current study was to identify patterns of results of surgical treatment of patients with intraventricular tumors, depending on the lesion location and its degree of differentiation. MATERIAL AND METHODS: A retrospective analysis of 54 consecutive surgical interventions on intraventricular brain tumors that were treated at the Uzhhorod Regional Clinical Center of Neurosurgery and Neurology in the period from January 2004 to February 2014. Localization of tumors was as follows: the fourth ventricle - 32 (59%), third ventricle - 13 (24%), lateral ventricles - 9 (17%). Neurological symptoms due to increased intracranial pressure was a common clinical manifestation of tumors for all patients. We used the following surgical approaches: suboccipital craniotomy for tumors of the fourth ventricle, transcallosal or transcortical-transventricular approach to the third ventricle and transcortical approach to lesions of lateral ventricles. Histological structure of the tumors: ependymoma - 13 (24%), astrocytoma - 12 (22%), medulloblastoma - 8 (14.0%), colloid cyst - 7 (13.0%), choroid plexus papilloma - 6 (11%), meningioma - 3 (6%), choroid plexus carcinoma - 1 (2%), teratoma - 1 (2%), central neurocytoma - 1 (2%), dermoid cyst - 1 (2%), chordoid glioma of the third ventricle - 1 (2%). The degree of tumors differentiation: grade I-II - 37 (69%), grade III-IV - 17 (31%). RESULTS: According to the data, among intraventricular tumors prevail the tumors of the fourth ventricle (59%), the most frequent histological type - ependymoma (24%). The total tumor removal was achieved in 38 patients (70%), of whom in 33 (87%) the degree of tumor differentiation was grade I-II. Postoperative mortality was 9% (5 patients), of which 4 (80%) - with the localization of the tumor in the third and fourth ventricles. The results of treatment were assessed according to Karnofsky scale: more than 60 points - 46 (85%) patients. CONCLUSIONS Acceptable results of surgical treatment of intraventricular brain tumours are in direct dependence from the tumor localisation and its histological structure. The most unfavorable factor is the localization of tumors in close proximity to the brainstem and the low degree of tumor differentiation.

We report on a case of subependymal giant cell astrocytoma(SEGA)in a patient with tuberous sclerosis(TSC)that presented with intratumoral hemorrhage and acute hydrocephalus. Initial treatment was external ventricular drainage to control the intracranial pressure;however, the tumor increased in size due to recurrent hemorrhage. Subsequently, the tumor was successfully removed via the transcortical-transventricular approach without neurological deterioration. Although intratumoral hemorrhage is extremely rare in patients with SEGA, subsequent acute hydrocephalus resulting from obstruction of the foramen of Monro will be fatal if prompt surgical treatment is not available. Careful and periodical radiographic examination of the central nervous system will be mandatory in patients with TSC, especially in those who have subependymal nodules(SEN)or SEGA around the foramen of Monro. Radical surgical removal should be considered before they become symptomatic.

The outcome of surgical management of subependymal giant cell astrocytoma in tuberous sclerosis complex

The aim of this study was to describe the clinical and procedural risk factors associated with the unplanned neurosurgical intensive care unit (NICU) readmission of patients after elective supratentorial brain tumor resection and serves as an exploratory analysis toward the development of a risk stratification tool that may be prospectively applied to this patient population. This was a retrospective observational cohort study. The electronic medical records of patients admitted to an institutional NICU between September 2018 and November 2021 after elective supratentorial brain tumor resection were reviewed. Demographic and perioperative clinical factors were recorded. A prognostic model was derived from the data of 4892 patients recruited between September 2018 and May 2021 (development cohort). A nomogram was created to display these predictor variables and their corresponding points and risks of readmission. External validation was evaluated using a series of 1118 patients recruited between June 2021 and November 2021 (validation cohort). Finally, a decision curve analysis was performed to determine the clinical usefulness of the prognostic model. Of the 4892 patients in the development cohort, 220 (4.5%) had an unplanned NICU readmission. Older age, lesion type, Karnofsky Performance Status (KPS) < 70 at admission, longer duration of surgery, retention of endotracheal intubation on NICU entry, and longer NICU length of stay (LOS) after surgery were independently associated with an unplanned NICU readmission. A total of 1118 patients recruited between June 2021 and November 2021 were included for external validation, and the model's discrimination remained acceptable (C-statistic = 0.744, 95% CI 0.675-0.814). The decision curve analysis for the prognostic model in the development and validation cohorts showed that at a threshold probability between 0.05 and 0.8, the prognostic model showed a positive net benefit. A predictive model that included age, lesion type, KPS < 70 at admission, duration of surgery, retention of endotracheal intubation on NICU entry, and NICU LOS after surgery had an acceptable ability to identify elective supratentorial brain tumor resection patients at high risk for an unplanned NICU readmission. These risk factors and this prediction model may facilitate better resource allocation in the NICU and improve patient outcomes.

Pediatric III ventricular tumors constitute an heterogeneous group of neoplasms which share some specific characteristic from the surgical point of view. All these tumors are rare and tend to remain clinically silent until they produce hydrocephalus. By excepting colloid cyst, which may induce an abrupt deterioration in clinical condition because of their critical location in the CSF pathways, most of these tumors reach a considerable large size before being recognized. Macrocrania in infants, false localizing signs, namely sixth cranial nerve palsy, and, more rarely, hemiparesis secondary to transtentorial herniation are the most common clinical manifestations of the associated chronic hydrocephalus. Although the ventricular dilation is often a favorable feature for the surgical removal, the unfolding of the thinned cerebral cortex and the consequent hemispheric subdural fluid collections represent the most typical surgical complication of the surgical treatment often requiring additional surgical procedures. The most typical tumors are the choroid plexus and the colloid cysts, the first of them relatively common, the second exceedingly rare in the pediatric population. Subependymal giant cell astrocytomas and subependymal are also quite known intraventricular tumors arising from the subependymal glia. Finally, midline gliomas from the adjacent parenchima and craniopharyngiomas from the suprachiasmatic cistern may rarely invade the ventricular system. Finally in children, meningiomas and teratomas may expand within the III ventricle, often without any recognizable origin from the ventricular wall. Choroids plexus papillomas represent only 0.4 to 0.6% of intracranial tumors, but up to 90% to these tumors occur within the first 2 years of life. The young age, the commonly large size and hypervascularity of these tumors may require tailored surgical strategies, including staged surgical excision, preoperative embolization or chemotherapy. The control of impaired CSF dynamics still remains one difficult to solve postoperative complication. Bilateral forms carry the worst prognosis in terms of postoperative hydrocephalus and neurodevelopmental outcome. Colloid cysts are extremely rare in children. The endoscopic excision is nowadays preferred in the young patients due to the large experience generally available with the endoscopic technique in the pediatric department. Subpendymal giant cell astrocytomas, the typical neoplasms of tuberous sclerosis is characterized by an unpredictable clinical evolution. In growing tumors causing obstructive hydrocephalus surgical excision is regarded as the most reliable surgical option due to the multiple failures of bilateral CSF shunting therapy observed in the subjects undergoing the extrathecal CSF shunt placement. In summary, most of the III ventricle tumors in children are benign in nature. Consequently, the surgical treatment remains the most effective therapeutic options. However, the deep location of these tumors, the strict anatomical relationships with important functional structures, the frequently associated hydrocephalus, their large size together with their relatively rare occurrence which may prevent reaching a sufficient experience, suggest that these tumors should be dealt with in specialized pediatric neurosurgical centres.