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.

Spontaneous Regression of a Symptomatic Cyst of the Cavum Septi Pellucidi and Vergae.

Spontaneous regression of cysts of the cavum septi pellucidi (CSP) and cavum vergae (CV) is rare and little discussed. The authors present their case report of this phenomenon following a severe headache in a 23-year-old woman, in whom magnetic resonance imaging (MRI) had previously confirmed significant thinning of the left lateral cyst wall. We consider this finding to be a possible predisposing factor to rupture and the spontaneous regression of such cysts. In addition to the mechanism of cyst regression, the interrelated causes of their expansion and formation will be discussed.

Idiopathic membranous occlusion of the foramen of Monro: an unusual cause of hydrocephalus and headache in adults.

Occlusion of foramen of Monro is an uncommon clinical entity that usually presents in children. Common causes are obstructing mass, infectious etiologies or vascular malformation. Rarely, it may be an idiopathic stricture or membrane. We report a case of idiopathic membranous obstruction of the foramen of Monro in a 45-year-old male with no past medical or surgical history. He presented with new intermittent dull and burning bifrontal severe headache for 2 d, which was alleviated slightly by non-steroidal anti-inflammatory medication. Imaging showed marked dilation of the lateral ventricles with normal third and fourth ventricles. The patient was discharged initially with conservative medical management and close follows up; however, the headache continued to progress and neurosurgical intervention was offered. The patient underwent endoscopic exploration, fenestration of the septum pellucidum, and right ventriculoperitoneal shunt placement. Bilateral membranous obstruction of foramina of Monro and an auto-fenestrated cavum septum pellucidum were identified intraoperatively. The patient reported resolution of headache post-operatively without recurrence on 1-month follow up. This case is unusual in that the patient presented without any known neurologic history or prior intracranial infections. It became apparent at the time of surgery that chronic obstruction of the bilateral foramina with collapse of the third ventricle had developed, and the safest durable treatment for him was septostomy and cerebrospinal fluid (CSF) shunt placement.

Neuroendoscopic treatment of symptomatic cyst of the septum pellucidum in children: A case series.

BACKGROUND: Symptomatic cysts of the septum pellucidum (CSP) are extremely rare in children and surgical indications are not well defined. A very careful clinical and neuroradiologic evaluation is necessary to consider a patient for surgical indication. METHODS: We present a surgical series of 7 pediatric patients. Clinical and radiological features of the patients, including clinical presentation, previous treatment, pre, and post-operative MRI, immediate postoperative, neuropsychiatric assessment, and outcomes were reviewed. RESULTS: There were 5 males and 2 females (mean age 8 yrs). Five patients presented a history of severe intermittent headaches, two of them were admitted with acute symptoms of raised intracranial pressure. One patient presented Epilepsy and ADHD and one patient had severe psychosis. Overall, psychiatric disorders were diagnosed in six patients, three patients had Intellectual Disability (ID). In all cases, the cyst presented a ballooning feature, with a mean volume of 18,36 cm3 (range 10,62-28,5) and significant lateral bulging of both layers. All were operated on endoscopically without complications. After surgery, a very significant decrease in cyst volume was observed (mean volume 5,68 cm3; range 3,18-10,1) with complete disappearance of the ballooning aspect. Headaches resolved in all patients. In two patients operated in emergency papilloedema and vision improved in the first week after surgery. No recurrence of the cysts was noted during follow-up in all patients. CONCLUSIONS: CSP may be associated with behavioral or psychiatric problems also in children. Neuroendoscopic surgery is a safe and effective therapeutic modality to treat CSP presenting with symptoms and signs of intracranial hypertension with good clinical results.

Surgical management of symptomatic cavum septum pellucidum cysts: systematic review of the literature.

Cavum septum pellucidum (CSP) and cavum vergae (CV) cysts are commonly found incidentally. They are usually asymptomatic but may present with symptoms related to obstructive hydrocephalus. There is no consensus about the management of symptomatic CSP and CV cysts. We present, to the best of our knowledge, the first systematic review of the different treatment options for symptomatic CSP and CV cysts. We conducted a literature review using PubMed database, searching for cases of symptomatic CSP and CV cysts managed surgically, and published until April 2019. Preoperative characteristics, surgical procedure, and postoperative outcome were analyzed using SPSS® software (Statistical Package for Social Sciences, IBM®). We found 54 cases of symptomatic CSP and CV cysts managed surgically (34 males, 20 females, 1.7/1 male to female ratio). Mean age was 24.3 ± 20.1 years. The most common presentation was headaches (34 patients, 62%), followed by psychiatric symptoms (27 patients, 49.1%). Preoperative radiological hydrocephalus was present in 30 patients (54.5%). The most common surgical procedure was endoscopic fenestration (39 patients, 70.9%), followed by shunting (10 patients, 18.2%), open surgery (3 patients, 5.5%), and stereotactic fenestration (1 patient, 1.8%). Complete resolution of symptoms was achieved in 36 patients (65.5%) and partial resolution in 7 patients (12.7%), and symptoms were unchanged in 2 patients. The present review suggests that surgical treatment could provide resolution of the symptoms in most of the cases, regardless of the procedure performed. Although mean follow-up was short among the studies, recurrence rate was low.

Third Ventricle Cavernous Malformation and Obstructive Hydrocephalus Thought to Be a Colloid Cyst.

BACKGROUND: Third ventricle cavernous malformations (CMs) associated with ventriculomegaly and obstructive hydrocephalus are quite rare in patients. Preoperative surgical planning can be challenging due to the lesion's non-specific appearance on CT and magnetic resonance imaging that can mimic other intraventricular pathologies, such as a colloid cyst. Management of these lesions can be varied in the setting of obstructive hydrocephalus. CASE DESCRIPTION: The patient is a 78-year-old woman who first presented to her primary care provider with balance difficulties and inability to ambulate on her own. She also had bladder incontinence and progressive, severe headaches. Imaging of the brain demonstrated entrapment of the right lateral ventricle and obstructive hydrocephalus due to a lesion in the third ventricle obstructing the right foramen of Monro, thought to be a colloid cyst. A right frontal neuroendoscopic approach with direct visualization, however, confirmed a third ventricle CM. A septal pellucidum fenestration was performed to restore cerebrospinal fluid communication and no resection of the lesion was performed. The patient recovered well after the operation and at clinical follow-up reported no headaches and was walking well without a walker and with no neurological deficits. CONCLUSIONS: A third ventricle CM was discovered after a neuroendoscopic approach for resection of a presumed colloid cyst in a patient with obstructive hydrocephalus. A neuroendoscopic septostomy was performed to treat the obstructive hydrocephalus and no resection was attempted. The patient suffered no complications and is at her neurologic baseline with no deficits.

The importance of staged surgery for giant atypical central neurocytoma.

The aim of this article is to discuss the importance of staged surgeries when approaching atypical central neurocytoma in children. Also, we show the preoperative embolization of the lesion as a maneuver to reduce the intraoperative bleeding. Central neurocytomas represent less than 0.5% of all intracranial tumors, and atypical central neurocytomas usually have unfavorable outcome, with high recurrence rate. The intraventricular location is frequent, with a predilection for the lateral ventricles. When completely resected, these lesions have a good prognosis. We report a case of a 12-year old male patient that presented with a history of headache for about 6 months, which worsened for 1 week prior to admission. Magnetic resonance imaging (MRI) brain showed a massive lesion occupying both lateral ventricles. He underwent a microsurgical treatment of a highly vascularized lesion, but the perioperative bleeding required interruption of the surgery. Thus, a preoperative embolization was able to occlude most arterial feeders and allowed subtotal resection in a second surgery. The patient had complete neurological recovery despite immediate post-operative deficits, and the histopathology was suggestive of atypical neurocytoma. Two-stage surgery with preoperative adjuvant embolization is a feasible strategy for treatment of large central neurocytomas in children.

Giant colloid cyst occupying a cavum septum pellucidum et vergae.

Colloid cysts are uncommon, intracranial lesions frequently arising from the anterior aspect of the third ventricle. Rarely a cyst presents greater than 30 mm diameter as a giant colloid cyst. This case reports a patient with a giant colloid cyst occupying a cavum septum pellucidum et vergae. The clinical and operative significance of this anatomical variation is discussed and the giant colloid cyst literature reviewed.

[Cysts of septum pellucidum, cavum vergae and cavum veli interpositi. Meta-analysis of 368 cases]. / Kisty prozrachnoi peregorodki, polosti Verge i promezhutochnogo parusa. Metaanaliz 368 klinicheskikh sluchaev.

OBJECTIVE: To review and systematize literature data on the incidence of cysts of septum pellucidum, cavum vergae and cavum veli interpositi, their clinical manifestations, indications for surgical treatment and optimal surgical approach. MATERIAL AND METHODS: An analysis included 72 manuscripts devoted to epidemiology, pathophysiology, clinical symptoms and results of surgical treatment of brain cysts. Case reports, series of cases, reviews and original studies were analyzed. RESULTS: Septum pellucidum cavity is always formed throughout an embryogenesis and persists in 20.34% of adults. Cavum vergae is observed in 2.32% of adults. Cyst of septum pellucidum is detected in 0.04% of adults. Analysis of 368 cases of cysts of septum pellucidum, cavum vergae and cavum veli interpositi has shown that clinical picture consists of headache (50% of cases), convulsive syndrome (23.6%), reduced intelligence (20.1%), behavioural disorders (15.8%), dizziness, nausea and vomiting (10.9%). Hydrocephalus occurs in 16.6% of cases. Endoscopic wall fenestration is preferred for cyst management. CONCLUSION: Brain cysts are rare and characterized by non-specific clinical manifestations. Symptomatic cyst is an indication for surgical treatment. Surgical treatment usually ensures regression of symptoms and low risk of complications.

Endoscopic Fenestration of Cavum Septum Pellucidum Cysts to the Third Ventricle: Technical Note.

BACKGROUND AND IMPORTANCE: Cavum septum pellucidum (CSP) and cavum vergae (CV) cysts are common incidental findings on imaging studies. However, they may rarely present with symptoms related to the obstruction of the foramen of Monro by the cyst leaflets. There is no consensus regarding the management of symptomatic CSP and CV cysts. We present an original transcavum interforniceal endoscopic fenestration technique. The step-by-step surgical procedure and two illustrative cases are presented. CLINICAL PRESENTATION: A 31-yr-old male and a 24-yr-old woman presented with symptomatic CSP and CV cysts. For both patients, neuronavigation was used to plan the procedure. An endoscope was introduced into the cyst through a right frontal burr-hole. After an examination of the endoscopic anatomy, a communication between the cyst and the third ventricle was performed using an endoscopic forceps. In both cases, directly after the fenestration, cerebrospinal fluid (CSF) passed through the communication, and the collapse of the cyst was appreciated. Symptoms were relieved in both patients, and neuropsychological assessment improved. Postoperative imaging showed a reduction in the cyst bulge, and patent foramen of Monro. CONCLUSION: Endoscopic fenestration of CSP and CV cysts to the third ventricle through an interforniceal navigated approach is a feasible and efficient surgical procedure. Theoretical advantages include a single tract through noneloquent brain, a perpendicular trajectory to the membrane for fenestration, and a large CSF space beyond the fenestration point.

Expanding Cyst of the Septum Pellucidum - Endoscopic Observations on the Mechanism of Development and Results of Treatment.

Cysts of the septum pellucidum (CSP) are usually asymptomatic; however, in some cases they can begin expanding and cause neurological deterioration. The mechanism leading to the formation of an expanding cyst of the septum pellucidum (ECSP) is not known. Based on observations made during endoscopic treatment of ECSP we analyzed intraoperative findings in respect to cyst formation mechanism and treatment prognosis. A group of 31 patients was studied. Only cases with bulging cyst walls occupying the frontal horns observed on imaging studies were included. The main symptom was a severe, intermittent headache. In three cases short term memory deficits were diagnosed. In one case papilloedema was observed. All patients underwent endoscopic fenestration of the ECSP. There were no cases of cyst reocclusion during a follow-up period of 1-14 years (mean 6.2 years). In 30 cases headaches resolved completely and in one case its intensity was significantly smaller. There was one case of postoperative hemiparesis. In all but two cases the thin, translucent region in the anterior part of the cyst floor was found. In the region small fissures and in three cases choroid plexus were observed. Endoscopic fenestration is the efficient treatment for ECSP. ECSP is formed on the basis of not completely closed, developmental communication of the cyst with other fluid spaces. The communication is opened by transient elevation of intraventricular pressure, and acts as a valve leading to fluid accumulation among the walls of the previously asymptomatic cavum septum pellucidum.

Pediatric rosette-forming glioneuronal tumor of the septum pellucidum.

Rosette-forming glioneuronal tumor (RGNT) is a rare tumor entity which has been reported mainly occurring in the fourth ventricle. It has been described as a benign lesion with limited extension into surrounding structures, including the cerebellar vermis, midbrain, and cerebral aqueduct. More recently, few cases involving also other midline structures have been documented as well. Here, we report about diagnosis and treatment of RGNT in the septum pellucidum in a pediatric patient which has not been described previously. A 7-year-old boy had a 3-week history of headache. Magnetic resonance imaging showed a solid mass in the septum pellucidum accompanied by hydrocephalus. The tumor was resected via a transcortical approach. Histological examination revealed the typical findings of a RGNT. At 2-year follow-up, there was no tumor recurrence, and clinical outcome was unremarkable. RGNT has to be considered in the differential diagnosis of pediatric midline tumors also outside of the fourth ventricle. Surgical resection is the first-line therapy which may result in beneficial outcome in the long term. The role of adjuvant therapy needs further definition since due to the rarity of this tumor entity, available data is very limited.

A Multifocal Glioneuronal Tumor with RGNT-Like Morphology Occupying the Supratentorial Ventricular System and Infiltrating the Brain Parenchyma.

BACKGROUND: Rosette-forming glioneuronal tumors (RGNTs) with multifocal growth throughout the ventricular system are extremely rare, and only 1 case of RGNT with dissemination limited to supratentorial ventricles has previously been reported. Recent evidence based on molecular data suggest that low-grade glioneuronal tumors (GNT) involving the septum pellucidum and the lateral ventricles, with either dysembryoplastic neuroepithelial tumor-like or RGNT-like features, may belong to a neuropathologic entity distinct from cortical dysembryoplastic neuroepithelial tumor and "typical" fourth ventricle RGNT, respectively. Given their rarity, the classification of these neoplasms is still uncertain and their clinicopathological and radiological aspects are only partially known. CASE DESCRIPTION: A 24-year-old male presented a GNT with RGNT-like morphological features centered in the septum pellucidum with multifocal masses occupying the lateral ventricles and the third ventricle with extraventricular infiltration of the frontal lobe. The patient underwent subtotal resection and 4 years follow-up. The clinicopathological and radiological features of the neoplasm are discussed. CONCLUSIONS: Advanced magnetic resonance imaging (magnetic resonance spectroscopy and perfusion-weighted imaging) may provide valuable information in the differential diagnosis between rare GNTs and other more frequent intraventricular neoplasms. In the present case, the enhancing remnant portion of the tumor showed remarkable contrast enhancement variability during the follow-up with slow in situ progression. However, available data suggest that spontaneous contrast enhancement "fluctuations" over time in RGNT may not represent a reliable indicator of tumor behavior.

Cavum Septum Pellucidum Causing Obstructive Hydrocephalus in a Toddler.

Cavum septi pellucidi (CSP) are benign developmental cystic midline cavities that are located between the lateral ventricles through the foramina of Monro. CSP are usually asymptomatic and have no cerebrospinal fluid (CSF) flow. Although their incidence is increased in association with head trauma, as well as psychiatric and behavioural disorders, this increase seldom causes disease. Herein, we discuss the case of a toddler who presented with episodic headaches for 6 weeks with associated vomiting triggered by strenuous activity. His neurological examination was normal. His MRI brain scan revealed large cavum septum pellucidum with obstructive hydrocephalus. He underwent endoscopic fenestration of the cyst with resolution of both hydrocephalus and the symptoms. Although CSP are generally asymptomatic, in rare situations, as in our illustrative case, they may cause obstructive hydrocephalus that requires urgent attention. CSF flow studies are helpful in confirming the diagnosis.

Symptomatic cysts of the cavum septi pellucidi, cavum vergae and cavum veli interpositi: A retrospective duocentric study of 10 patients.

OBJECTIVE: Cysts of the Cavum septi pellucidi (CSP), cavum vergae (CV) and cavum veli interpositi (CVI) are anterior midline intracranial findings which are typically incidental - only rarely do we encounter symptomatic cysts of this type. Only a quite small number of these cysts series have been published, controversies regarding optimal management still exist. PATIENTS AND METHODS: This is a retrospective study of 10 patients treated at 2 clinics between 2002-2018. 9 patients underwent surgery and 1 is under long-term monitoring. Apart from demographic data, the study analyzed symptoms, cyst size and progression over time, ventricle size, complications, and treatment modality. RESULTS: CSP with CV was found in 8 cases with 1 case each of CSP and CVI. The study comprised 6 men and 4 women, including 4 children. The mean follow-up time was 43.4 months. The average cyst size was 20.4 mm in CSP and 19.8 mm in CV; the CVI was 33 mm. Headache was most commonly reported (70%) followed by behavioral disturbance (30%). Disturbance in memory, psychomotor development, school performance, visual acuity, and vomiting was variously noted in 20%. The prevailing symptom was headache in adults and behavioral and autonomic disturbance in children. Postoperatively, cysts had reduced by an average of 44.3% while the ventricles remained unchanged. Symptoms resolved in all cases with residual problems in patients presenting with memory loss. No complications were noted. CONCLUSION: Endoscopic fenestration is the method of choice in the treatment of symptomatic midline cysts. We recommend that any further research focuses on precisely establishing their clinical presentation, particularly neuropsychological symptoms.

An Analysis of Outcome of Endoscopic Fenestration of Cavum Septum Pellucidum Cyst - More Grey than Black and White?

BACKGROUND: Cavum septum pellucidum (CSP) and cavum vergae are actually fluid-filled, generally communicating midline cavities located between the third ventricle and corpus callosum. There have been various reports of their association with many behavioral and psychiatric disorders. Infrequently, they have been associated with an obstructive hydrocephalus-like picture. Although the structure and management of CSP has long been known, it has been an enigma as far as functional significance and management indications are concerned. The authors of this article try to analyze the significance of a persistent cavum and involvement of the same entity in varied presentations ranging from an incidental imaging finding to acute hydrocephalus, and propose a possible implication on the present surgical intervention paradigm. PURPOSE: To assess the surgical outcome of fenestration of a CSP cyst. METHODS: Retrospective analysis of 3 patients who underwent endoscopic fenestration for CSP with obstructive hydrocephalus between 2012 and 2014 was done, and data were analyzed for symptomatic clinical improvement in particular behavior. RESULTS: Pre- and postoperative brain MRI showed a significant decrease in the size of the cyst as well as the ventricles. There were no recurrences during follow-up. All of the patients improved. CONCLUSIONS: (1) Endoscopic fenestration of symptomatic CSP cysts is a safe treatment option. (2) Neurocognitive assessment is essential in the evaluation and outcome assessment of CSP.

Imaging of spontaneous intraventricular rupture of a septum pellucidum colloid cyst: case report.

Intraventricular rupture of a colloid cyst is a rare phenomenon and has been proposed as a mechanism for sudden death in patients with colloid cysts. Imaging of a colloid cyst during rupture has been described in only one other instance. The authors report a highly unusual case of a 53-year-old man who presented with acute onset headaches and imaging findings of hydrocephalus caused by a colloid cyst originating from the septum pellucidum and superior surface of the roof of the third ventricle. Interestingly, the colloid cyst revealed imaging signs of intraventricular rupture characterized by a tail-like drainage of cystic contents into the occipital horn of the lateral ventricle. The patient was surgically treated with a craniotomy and transcallosal approach to the colloid cyst, where it was noted that the cyst wall was spontaneously open. This rare case highlights unique imaging findings of a rare event in an infrequent pathology confirmed with intraoperative microscopy. The authors further document the process of cyst rupture and speculate on its pathomechanisms.

Cavum septi pellucidi cysts: a survey about clinical indications and surgical management strategies.

INTRODUCTION: Cavum septi pellucidi (CSP) cysts have a very low incidence (0.04%). Symptomatic patients usually present aspecific symptoms. For this reason, the management of these patients is still debated. MATERIALS AND METHODS: We selected the case of a ten year old patient, with a clinical history of frontal morning headaches and difficulty in concentration. Brain MRI documented a septum pellucidum cyst and a moderate biventricular dilation. We submitted the case, and a questionnaire concerning indications to surgery and management options to an international group of 54 pediatric neurosurgeons, analyzing the results and comparing them with the current literature. RESULTS: The majority of the participants (50%) indicated as appropriate at the early stage only a clinical observation. In case of persistence of clinical symptoms, 58% opted for intracranial pressure (ICP) monitoring, which, if raised, was considered by 91% as an adequate indication to proceed with surgical treatment. A total of 98% of the participants indicated endoscopic fenestration of the cyst as the preferred surgical strategy. CONCLUSIONS: The management of symptomatic patients with CSP cyst is controversial. Our results suggest that in most of the patients with aspecific symptoms, clinical observation and eventually ICP monitoring are adequate to identify patients for surgery.