Third Ventricle Tumors in Children

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.

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.

Neoplasms of the Nervous System and Related Topics

Colloid cysts are uncommon lesions in the pediatric age group, which most commonly occur in the fourth through fifth decades. The authors hereby report a series of 36 patients with colloid cysts in the pediatric age group. A retrospective chart review was conducted on all patients with colloid cyst who underwent surgery in our institute between November 2003 and December 2016 (13years). Patients above the age of 18 were excluded from the study. They were analyzed based on clinical presentation, radiological findings, surgical approaches, and outcome. There were 36 pediatric patients selected for the study. Age ranged from 8 to 18years. The male-to-female ratio was 3.5:1. Headache and vomiting were the most common symptoms, and papilledema is the most common clinical sign. The mean duration of symptoms was 9months (range 1day to 5years), but 27 (75%) of the children had precipitous symptoms just before presentation. Preoperative CT showed a hyperdense non-enhancing lesion in the majority of cases. Endoscopic excision was done in 13; 5 patients underwent transcortical transventricular excision, while transcallosal approach was opted for in 17 patients. In one patient, the colloid cyst could not be removed endoscopically and had to be converted to transcortical transventricular approach. Postoperatively, five patients developed CSF leak. They were successfully managed conservatively. One patient had operative site extra dural hematoma and underwent re-exploration, and two patients had transient hemiparesis which improved spontaneously. The median follow-up period was 9months in the 30 available patients. Though no formal neuropsychological testing was done at follow-up, all children appeared to be doing well without memory disturbances. Pediatric colloid cysts are less common and may show rapid deterioration. Timely surgery results in a permanent cure with minimum morbidity. The results of either micro neurosurgical or endoscopic operative excision of colloid cysts in children are excellent. All children who are symptomatic with raised intracranial pressure due to a third ventricular colloid cyst should undergo definitive surgery. There were no major permanent deficits in memory or disconnection syndromes observed with the limited anterior colostomy.

Subependymal giant cell astrocytoma (SEGA) is a WHO grade I pediatric glioma arising in 5-15% of patients with tuberous sclerosis (TSC). Rare cases of isolated SEGA without TSC have been described. The etiology, genetic mechanisms, natural history, and response to treatment of these lesions are currently unknown. We describe two such cases of isolated SEGA with follow-up. Retrospective review was performed at a single institution to describe the clinical course of pathology-confirmed SEGA in patients with germline testing negative for TSC mutations. Two cases of isolated SEGA were identified. Genetic analysis of the tumor specimen was available for one, which revealed an 18 base pair deletion in TSC1. Both cases were managed with surgical resection, one with preoperative embolization. In spite of a gross total resection, one patient experienced recurrence after three years. Treatment with an mTOR inhibitor led to a significant interval reduction of the mass on follow-up MRI. The patient tolerated the medication well for 6years and is now off of treatment for 2years with a stable lesion. Cases of SEGA outside of the context of TSC are exceedingly rare, with only 48 cases previously described. The genetic mechanisms and treatment response of these lesions are poorly understood. To date, these lesions appear to respond well to mTOR inhibitors and may behave similarly to SEGAs associated with TSC. However, given that experience is extremely limited, these cases should be followed long term to better understand their natural history and treatment response.

Monoclonal and polyclonal antisera were used against 12 cases of neuroepithelial (colloid) cysts to determine the specific antigenic profile of the cyst epithelium. Intermediate filament markers (cytokeratin, vimentin, neurofilament, and glial fibrillary acidic protein) and epithelial markers (epithelial membrane antigen and monoclonal antibody lu-5) demonstrated that colloid cyst epithelium has a unique antigenic profile in contrast to that of choroid plexus or glial tissue. Theories raised to explain the etiology of colloid cysts have included derivation from the embryonic paraphysis, detachments of developing neuroepithelium from the tela choroidea, and remnants of respiratory epithelium; a more recent theory suggests that these cysts are products of developing choroid plexus or ependyma. The present study shows that colloid cyst epithelium is distinct from that of choroid plexus or ependyma and therefore does not represent a product of their formation, nor does it represent a form of immature glia. This finding supports the conclusion that colloid cysts in any ventricular location represent a developmental anomaly of primitive neuroectoderm in the embryo, which remains unique from other products of neuroectodermal derivation.

Thirteen colloid cysts (CC), four Rathke cleft cysts (RCC), three follicular cysts of normal pituitary gland (FCP), four enterogenous cysts (EC), three normal choroid plexi (CP), three choroid plexus papillomas (CPP), and several samples of normal bronchial mucosa and ependyma were studied by electron microscopy. The ultrastructure of most of the CC was indistinguishable from that of RCC, FCP, EC, and normal bronchial mucosa in demonstrating nonciliated and ciliated epithelial cells, goblet cells, basal cells, and intermediate forms, some showing evidence of early squamous differentiation. Two CC displayed mostly squamous differentiation, and one contained basally situated cells resembling myoepithelial cells. Although the RCC and FCP displayed features similar to those of CC, they also contained cells with electron-dense granules indicating pituitary hormone production and scattered cells showing oncocytic change. EC were lined by either squamous cells or mucin-producing columnar cells. Every CC, RCC, FCP, and EC contained cells covered by glycocalyx. In contrast to aforementioned lesions, ependymal lining, CP, and CPP were composed of a single cell type, one with numerous villi, few cilia, and no signs of keratinization, mucin production, or glycocalyx coating. It is concluded that the ultrastructural properties of CC are the same as those of cysts of endodermal or ectodermal origin and of respiratory mucosa. No ultrastructural similarities were observed between CC and normal or neoplastic neuroepithelium. These ultrastructural findings support the concept of an enterogenous origin of colloid cysts and highlight their similarity to RCC.

More than meets the eye; A case report of colloid cyst in a young female with neurofibromatosis type 1

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.

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.

Introduction: Supratentorial intraventricular tumors are rare central nervous system neoplasms located deep within the ventricular system. Their proximity to eloquent neural pathways and critical vascular structures makes surgical management technically challenging. Data from low and middle income regions remain limited. This study evaluates the clinical features, histopathological patterns, surgical approaches, and outcomes of patients treated at a tertiary neurosurgical center in Nepal. Material and Method: A retrospective observational study was conducted including 27 consecutive patients who underwent surgical resection of supratentorial intraventricular tumors between September 2020 and August 2025. Demographic, clinical, radiological, histopathological, operative, and postoperative data were collected and analyzed. Statistical analysis included descriptive statistics, chi-square testing, logistic regression modeling, and calculation of confidence intervals. Institutional Review Committee approval was obtained prior to study initiation. Results: The mean age was 24.9 ± 13.2 years, with a male predominance of 21 (77.8%). Headache was the most common presenting symptom, occurring in 14 (51.9%) patients. Colloid cyst was the most frequent histopathological diagnosis, identified in 7 (25.9%) patients, followed by subependymal giant cell astrocytoma in 5 (18.5%) and choroid plexus tumors in 5 (18.5%). The transcortical-transventricular approach was most frequently utilized in 14 (51.8%) cases. Postoperative hydrocephalus requiring cerebrospinal fluid diversion occurred in 5 (18.5%) patients. Overall mortality was observed in 3 (11.1%) patients. No statistically significant association was found between tumor type or surgical approach and postoperative complications. Conclusion: Microsurgical resection remains the cornerstone of management for supratentorial intraventricular tumors in resource-limited settings. Although acceptable surgical outcomes can be achieved, postoperative hydrocephalus continues to be a significant challenge. Larger prospective multicenter studies are needed to optimize surgical strategies and improve patient outcomes.

Endoscopic versus Open Microsurgical Excision of Colloid Cysts: A Comparative Analysis and State-of-the-Art Review of Neurosurgical Techniques

Colloid cysts (CCs) are rare at all ages, and particularly among children. The current literature on pediatric CC is limited, and often included in mixed adult/pediatric series. The goal of this multinational, multicenter study was to combine forces among centers and investigate the clinical course of pediatric CCs. A multinational, multicenter retrospective study was performed to attain a large sample size, focusing on CC diagnosis in patients younger than 18 years of age. Collected data included clinical presentation, radiological characteristics, treatment, and outcome. One hundred thirty-four children with CCs were included. Patient age at diagnosis ranged from 2.4 to 18 years (mean 12.8 ± 3.4 years, median 13.2 years, interquartile range 10.3-15.4 years; 22% were < 10 years of age). Twenty-two cases (16%) were diagnosed incidentally, including 48% of those younger than 10 years of age. Most of the other patients had symptoms related to increased intracranial pressure and hydrocephalus. The average follow-up duration for the entire group was 49.5 ± 45.8 months. Fifty-nine patients were initially followed, of whom 28 were eventually operated on at a mean of 19 ± 32 months later due to cyst growth, increasing hydrocephalus, and/or new symptoms. There was a clear correlation between larger cysts and symptomatology, acuteness of symptoms, hydrocephalus, and need for surgery. Older age was also associated with the need for surgery. One hundred three children (77%) underwent cyst resection, 60% using a purely endoscopic approach. There was 1 death related to acute hydrocephalus at presentation. Ten percent of operated patients had some form of complication, and 7.7% of operated cases required a shunt at some point during follow-up. Functional outcome was good; however, the need for immediate surgery was associated with educational limitations. Twenty operated cases (20%) experienced a recurrence of their CC at a mean of 38 ± 46 months after the primary surgery. The CC recurrence rate was 24% following endoscopic resection and 15% following open resections (p = 0.28). CCs may present in all pediatric age groups, although most that are symptomatic present after the age of 10 years. Incidentally discovered cysts should be closely followed, as many may grow, leading to hydrocephalus and other new symptoms. Presentation of CC may be acute and may cause life-threatening conditions related to hydrocephalus, necessitating urgent treatment. The outcome of treated children with CCs is favorable.

Colloid cysts are benign slow-growing cystic lesions located on the roof of the third ventricle that usually present with symptoms related to gradual rise of intracranial pressure. They mostly remain asymptomatic and sometimes grow progressively and cause diverse symptoms associated with increased intracranial pressure such as headache, diplopia, and sixth cranial nerve palsy. Here we report a 47-year-old female who presented to the emergency department with acute severe headache and nausea/vomiting. On MRI examination acute hydrocephaly due to hemorrhagic colloid cyst was detected. Acute hemorrhage in colloid cysts is extremely rare and may present with symptoms of acute increase in the intracranial pressure. Intracystic hemorrhage is very rarely reported as a complication of colloid cyst presenting with paroxysmal symptoms of acute hydrocephaly.

Endoscopic management of third ventricle colloid cysts, analysis of the technical nuances and the neuroendoscopic anatomy: A preliminary experience - IJN- Print ISSN No: - 2581-8236 Online ISSN No:- 2581-916X Article DOI No:- 10.18231/j.ijn.2019.019, IP Indian Journal of Neurosciences-IP Indian J Neurosci