Case report: A rare case of neurocytoma of the Vth cranial nerve.

We report a case of neurocytoma originating from cranial nerve V. A 53-year-old female patient presented with a 20-day history of right frontotemporal facial paresthesia and pain. Magnetic resonance imaging (MRI) showed a 2.5-cm × 1.4-cm "dumbbell" enhancing lesion located in the cisternal segment of cranial nerve V with extension into Meckel's cave, and the signal characteristics were suggestive of trigeminal neurinoma. The lesion was resected through a subtemporal middle cranial fossa approach. Intraoperative findings revealed that the tumor originated from the cisternal segment of cranial nerve V and extended into Meckel's cave through the trigeminal foramen. No dural attachment was found. The tumor was debulked using sharp dissection and bipolar cautery under the microscope. Extraventricular neurocytomas (EVNs) are extremely rare tumors of the central nervous system. To date, only two cases of neurocytomas arising from cranial nerve VIII have been described. This paper summarizes the clinicopathological features of a case of neurocytoma originating from the cisternal segment of cranial nerve V with extension into Meckel's cave and expounds the relevant diagnoses and treatments, which may provide a practical clinical basis and experience for the diagnosis and treatment of EVN in the future.

Consensus guidelines for the management of primary supra-tentorial intraventricular tumour for low- and middle-income countries.

Almost any primary or metastatic brain tumour can manifest in intraventricular (IV) locations. These tumours may either originate within the ventricular system or extend into the IV space through growth. Such neoplasms represent a broad spectrum, with supratentorial IV tumours forming a heterogeneous group. This group includes primary ependymal tumours, central neurocytomas, choroid plexus tumours, and notably, meningiomas, as well as a variety of non-neoplastic, benign, glial, and metastatic lesions that can secondarily invade the IV compartment. Often presenting with nonspecific symptoms, these tumours can lead to delayed medical attention. The diversity in potential diagnoses, combined with their deep and complex locations, poses significant management challenges. This paper aims to delineate optimal management strategies, underscoring the importance of multidisciplinary care, especially in settings with limited resources, to effectively navigate the complexities associated with treating intraventricular brain tumours.

Radiotherapy for central neurocytoma: A multicentric retrospective study in France.

PURPOSE: Neurocytomas represent 0.25 to 0.5% of primary brain tumours and are mainly found in young adults. These tumours have neuronal differentiation. The cornerstone treatment is neurosurgery. The efficacy of other therapies, including radiotherapy, is still unclear. The objective of this study was to evaluate the management of central neurocytomas and the role of radiotherapy. MATERIALS AND METHODS: All adult patients (age 18 years or older) newly diagnosed with a histologically confirmed neurocytoma between 2006 and 2015 in France were included. RESULTS: One hundred and sixteen patients were diagnosed with a central neurocytoma during the study period. All patients underwent surgical resection, and six received adjuvant radiotherapy. Eleven patients received radiotherapy due to progression. After a median follow-up of 68.7 months, local failure occurred in 29 patients. The 5-year local control rate was 73.4%. According to univariate analysis, marker of proliferation Ki67 index greater than 2% (hazard ratio [HR]: 1.48; confidence interval [CI]: 1.40-1.57; P=0.027) and subtotal resection (HR: 8.48; CI: 8.01-8.99; P<0.001) were associated with an increase in local failure. Gross total resection was associated with a higher risk of sequelae epilepsy (HR: 3.62; CI: 3.42-3.83; P<0.01) and memory disorders (HR: 1.35; CI: 1.07-1.20; P<0.01). Ten patients (8.6%) died during the follow-up. The 10-year overall survival rate was 89.0%. No prognostic factors for overall survival were found. CONCLUSION: The analysis showed that patients who underwent subtotal surgical resection, particularly when the tumour had a Ki67 index greater than 2%, had an increased risk of local recurrence. These patients could benefit from adjuvant radiotherapy.

How I do it? Resection of left ventricular central neurocytoma via trans-sulcal approach.

BACKGROUND: The management of lateral ventricle tumors requires a balance between maximizing safe resection and preserving neurological function. METHOD: The authors present a successful case of a left lateral ventricular central neurocytoma resection. The trans-superior frontal sulcus approach was employed, providing a safe corridor while minimizing damage to the surrounding neuroanatomy. The use of an endoscope further facilitated the procedure, enabling the confirmation of complete tumor removal and the preservation of deep venous drainage and periventricular structures. CONCLUSION: This case highlights the utility of the trans-sulcal approach and the benefits of endoscopic assistance in the management of lateral ventricle tumors.

Extraventricular Neurocytoma of the Sellar Region Presenting With Syndrome of Inappropriate Antidiuresis.

Neurocytomas are neuronal tumors that are usually intraventricular. Rare cases can arise from extraventricular sites. To our knowledge, only 29 cases of extraventricular neurocytoma of the sellar region (EVNSR) have been reported in the literature. We describe a case of a 39-year-old woman who presented with a one-month history of refractory headache, nausea and vomiting. Magnetic resonance imaging (MRI) showed a 5.1 × 3.1 × 2.2 cm sellar and suprasellar mass, suggestive of a pituitary adenoma (PA). She had hyponatremia, obstructive hydrocephalus, and panhypopituitarism at presentation (hypogonadism, adrenal insufficiency). After glucocorticoid replacement therapy and ventriculoperitoneal shunt, the vomiting and headache resolved, but she remained with nausea and hyponatremia. She was submitted to surgery, and histopathological analysis revealed a neurocytoma with positive immunostaining for arginine vasopressin. Syndrome of inappropriate antidiuresis (SIAD) was diagnosed but did not resolve after surgery due to residual tumor, despite fluid restriction and saline replacement. SIAD later resolved with empagliflozin. In conclusion, EVNSR is extremely rare and can be misdiagnosed as PA on MRI. In the context of SIAD and extraventricular neurocytoma, a secreting arginine vasopressin tumor must be considered. SIAD can be challenging to treat, with excision of the EVNSR the treatment choice and, alternatively, empagliflozin associated with fluid restriction.

Intraventricular neurocytoma: A diagnostic challenge with prognostic value.

Intraventricular neurocytoma is a low incidence central nervous system tumor. It predominantly affects young adults with no apparent gender predilection. The main symptoms include headache, nausea and vomiting. These result from hydrocephalus due to the obstruction of cerebrospinal fluid flow. On diagnostic imaging, neurocytoma can be suspected by some features, such as peripheral cysts, lobulated contours and septa that bridge the ventricular wall, giving a "scalloped" appearance. There are other characteristics, but they are less specific for the diagnosis. The atypical variant of neurocytoma is even rarer and leads to a worst prognosis. Atypical neurocytomas develop higher proliferative potential identified by the Ki-67 biomarker and higher recurrence rate. There are few studies about the imaging characteristics of atypical neurocytomas. At this point, there are no reliable distinctive features to differentiate atypical neurocytomas, especially due to their low incidence. We present the case of a 20-year-old female patient with symptoms of intracraneal hypertension. CT and MRI of the brain revealed a mass occupying the body of the left lateral ventricle, adjacent to the foramen of Monro. The mass was primarily solid with discrete peripheral cyst and a few scalloped areas. It also showed signs of supratentorial obstructive hydrocephalus. The tumor was partially removed because of bleeding and compromise of vascular structures. Immunohistochemistry revealed positive synaptophysin, elevated Ki-67 (7%), increased number of blood vessels and moderate nuclear atypia. After surgery, the patient persisted with signs of intracranial hypertension, not improving with clinical management and requiring aggressive surgical procedures. While rare, atypical neurocytoma requires a better characterization, especially through imaging, to optimize immediate management and explore new therapeutic options.

Molecular and clinical characterization of atypical central neurocytomas: implications for diagnosis and treatment strategies.

OBJECTIVES: This study aimed to investigate the histological and molecular characteristics of atypical central neurocytomas (CNs) and evaluate their clinical treatment outcomes, with the aim of identifying reliable biomarkers for differentiation and optimal treatment strategies. METHODS: We conducted a retrospective study including 61 patients diagnosed with CNs. Clinical data, neuroimaging, and pathological findings were analyzed. RNA sequencing was performed on tumor tissues to identify differentially expressed genes. RESULTS: Histological atypia and the Ki-67 index showed no significant impact on progression-free survival (PFS) or overall survival (OS). RNA sequencing identified significant genetic alterations in pathways such as neuroactive ligand-receptor interaction, cAMP, MAPK, and Ras signaling. Differently expressed genes included AMOTL1, PIK3R3, TGFBR1, SMO, COL4A6, MGP, SOX4, IGF2, SLIT1, and CKS2. The five-year OS rate (p = 0.015) and PFS rate (p = 2.00 × 10-6) were significantly higher in the complete resection (CR) group compared to the incomplete resection (IR) group. Postoperative radiotherapy did not affect OS or PFS in the CR group. The five-year PFS rate (p = 3.80 × 10-5) was significantly longer in patients in the CR group who did not receive radiotherapy compared to those in the IR group who did receive radiotherapy. The extent of surgical resection and operative approaches were found to be irrelevant to perioperative complications and dysfunctions at the last follow-up. CONCLUSION: CR is crucial for a better prognosis in patients with atypical CNs. Additional radiotherapy after CR offers little benefit. Histological atypia and the Ki-67 index are not effective in distinguishing between atypical and typical CNs. Identified genetic alterations provide insights into the aggressive behavior of atypical CNs, suggesting potential therapeutic targets and underscoring the need for further research to optimize treatment strategies.

Revealing subependymal giant cell astrocytoma with multimodal positron emission tomography: illustrative cases.

BACKGROUND: There is limited literature on the use of positron emission tomography (PET) for benign tumors originating in the brain ventricles, and the use of multiple tracers for subependymal giant cell astrocytoma (SEGA) has not been reported. The authors compared the PET findings in two SEGA cases with past reports and literature, exploring the distinctive characteristics of SEGA on PET. OBSERVATIONS: In a 21-year-old female with SEGA, the authors utilized 18F-fluorodeoxyglucose (18F-FDG), 11C-methionine (11C-MET), 18F-fluorothymidine (18F-FLT), 18F-fluoromisonidazole, and 18F-THK5351 tracers. Additionally, in a 6-year-old girl, the authors performed 11C-MET PET. LESSONS: The results indicated the accumulation of all tracers except 18F-FDG, with particularly intense accumulation noted with 18F-FLT. In particular, 18F-FLT demonstrated accumulation comparable to that observed in malignant tumors. This study suggests that multiple PET tracers can provide valuable insights into the characterization of SEGA, with 18F-FLT showing particular promise as a distinctive marker of blood-brain barrier disruption. Further research in larger cohorts may enhance our understanding of metabolic patterns in SEGA and aid in its diagnosis and treatment. https://thejns.org/doi/10.3171/CASE24111.

Navigating the Diagnostic and Therapeutic Challenges of Central Neurocytoma: A Case Report.

Central neurocytoma, a rare intracranial tumor predominantly located in the lateral and third ventricles, presents a diagnostic and therapeutic challenge due to its varied clinical manifestations. We report the case of a 53-year-old male presenting with right upper and lower limb weakness, headaches, blurred vision, and tingling sensations, leading to the diagnosis of central neurocytoma with associated hydrocephalus. Initial evaluation, including magnetic resonance imaging (MRI) and subsequent computed tomography (CT) scans, revealed characteristic features of the tumor. The patient underwent a two-stage surgical intervention, including tumor excision and ventriculoperitoneal shunting, followed by a tracheostomy due to respiratory complications post-surgery. Histopathological examination confirmed the diagnosis of central neurocytoma, prompting multidisciplinary management and further referral for long-term follow-up. This case underscores the importance of comprehensive evaluation, multidisciplinary collaboration, and continued research in optimizing the diagnosis and management of central neurocytomas.

Central Neurocytoma: A Case Report With Literature Review.

Central neurocytoma (CN) is a rare, low-grade, neuronal tumor frequently encountered in young adults. Complete surgical resection is the treatment of choice; however, it is associated with grave postoperative complications in a quarter of patients, including neurological (motor weakness, memory deficit, aphasia, and seizure) as well as regional (hydrocephalus, hematoma, infection, and subcutaneous hydrops) complications. Herein, we present a case of a 35-year-old female who presented with decreased vision for the last 7-8 days and headache over the last 1-1.5 years. An ophthalmologic examination suggested papilledema. Magnetic resonance imaging (MRI) of the brain illustrated a well-circumscribed, large, lobulated, altered signal intensity midline intraventricular lesion (72 × 68 mm) attached to the septum pellucidum near the foramen of Monro (FoM) most likely to be CN. The patient underwent complete surgical resection but required re-exploration the next day for hematoma removal due to intraventricular hemorrhage. Over the next 40 days, the patient developed hydrocephalus with transtentorial herniation and succumbed. Histopathological examination (HPE) was suggestive of CN and immunohistochemistry (IHC) was strongly positive for synaptophysin, thus confirming the diagnosis of CN.

Long-term outcomes of central neurocytoma - an institutional experience.

INTRODUCTION: Central Neurocytoma (CN) is a rare, WHO grade 2 brain tumor that predominantly affects young adults. Gross total resection (GTR) is often curative for CNs, but the optimal treatment paradigm including incorporation of RT, following subtotal resection (STR) and for scarcer pediatric cases has yet to be established. METHODS: Patients between 2001 and 2021 with a pathologic diagnosis of CN were reviewed. Demographic, treatment, and tumor characteristics were recorded. Recurrence free survival (RFS) and overall survival (OS) were calculated according to the Kaplan Meier-method. Post-RT tumor volumetric regression analysis was performed. RESULTS: Seventeen adults (≥ 18 years old) and 5 children (< 18 years old) met the criteria for data analysis (n = 22). With a median follow-up of 6.9 years, there was no tumor-related mortality. Patients who received STR and/or had atypical tumors (using a cut-off of Ki-67 > 4%) experienced decreased RFS compared to those who received GTR and/or were without atypical tumors. RFS at 5 years for typical CNs was 67% compared to 22% for atypical CNs. Every pediatric tumor was atypical and 3/5 recurred within 5 years. Salvage RT following tumor recurrence led to no further recurrences within the timeframe of continued follow-up; volumetric analysis for 3 recurrent tumors revealed an approximately 80% reduction in tumor size. CONCLUSION: We provide encouraging evidence that CNs treated with GTR or with RT after tumor recurrence demonstrate good long-term tumor control.

Central Neurocytoma Treated Using Supratentorial Ventricle Radiotherapy: A Single-Institution Analysis of Five Cases in Adjuvant or Salvage Settings After Surgery.

INTRODUCTION: Central neurocytoma (CN) is an extremely rare tumor primarily located in the supratentorial ventricular system, categorized as a glioneuronal or neuronal tumor. METHODS: This study presented a retrospective analysis of five CN patients who received adjuvant or salvage radiotherapy. Patients, aged 31-59 years, underwent radiation doses ranging from 60 Gy to 50.4 Gy over 27-30 fractions. RESULTS: All patients achieved effective local tumor control without severe complications. The median follow-up period was 51.7 months, demonstrating 100% overall and progression-free survival rates. DISCUSSION: Our study's clinical outcomes align with previous research, despite the limitation of a small sample size. Emphasizing the necessity for additional research, our findings added to the potential evidence of radiotherapy in managing CN. Larger, long-term studies were needed to confirm these promising results.

Clinicopathological and Molecular Profile of Sellar Neurocytoma.

OBJECTIVE: To investigate the clinical features, imaging characteristics and molecular profile of Sellar neurocytoma (SN). METHODS: Clinical, imaging, and pathological features of eleven cases of sellar neurocytoma were retrospectively analyzed. Electron microscopy was performed in five cases. Molecular features were detected in tumor tissue by RNA sequencing, qPCR and IHC. RESULTS: The clinical features of SN patients showed high incidence of hyponatremia (73%,8/11) and the tumors tended to invaded lateral side of saddle area from preoperative imaging analysis. The tumors had positive NeuN, SYN, NF, SSTR2 immunohistochemistry staining. Tumor transcriptomic analysis suggested a new LMCD1-AS1:GRM7-AS1 fusion gene event and increased expression of 10 hypothalamus-secreted hormones in SN. 15 differentially expressed genes were verified for qPCR verification. SSTR2 has been verified by immunohistochemistry. CONCLUSIONS: Hyponatremia is the dominant clinical features of SN. Preoperative imaging suggests that growth toward the dorsal region is the imaging feature of SN. SSTR2 expression and LMCD1-AS1:GRM7-AS1 fusion gene event expected to become a new molecular marker for SN. Somatostatin receptor ligand therapy may be a potential therapy for SN.

Adult Central Neurocytomas: Clinical Features and Long-Term Treatment Outcomes in Different Age Groups.

BACKGROUND: Central neurocytomas (CNs) usually occur in young adults, and the clinical characteristics and surgical outcomes of patients in different age groups may be different. METHODS: This study was undertaken to compare the clinical and long-term treatment outcomes of patients with CNs in younger and older adult age groups. RESULTS: Eighty consecutive adults with CNs were included, with a mean presentation age of 28.4±7.6 years (range: 19-66 years). Thirty (37.5%) patients were <27 years old, and they tended to manifest with multiple symptoms (P = 0.002), increased intracranial pressure (ICP) symptoms (P = 0.036), an acute clinical course (P = 0.037), worse preoperative neurologic function (P = 0.023), and a larger lesion size and volume (P = 0.004 and 0.007, respectively) than their older age counterparts (≧27 years). An older onset age (P = 0.005) or age ≧27 years (P = 0.014) and worsened Karnofsky Performance Status (KPS) scale (P = 0.040) immediately after microsurgery were associated with unimproved long-term outcomes. CONCLUSIONS: CNs in younger adult patients behave differently from those in the older age group. Surgery can halt neurologic deterioration and ensure satisfactory outcomes.

Whole-exome sequencing reveals genetic variants that may play a role in neurocytomas.

OBJECTIVES: Neurocytomas (NCs) are rare intracranial tumors that can often be surgically resected. However, disease course is unpredictable in many patients and medical therapies are lacking. We have used whole exome sequencing to explore the molecular etiology for neurocytoma and assist in target identification to develop novel therapeutic interventions. METHODS: We used whole exome sequencing (WES) to compare the molecular landscape of 21 primary & recurrent NCs to five normal cerebellar control samples. WES data was analyzed using the Qiagen Clinical Insight program, variants of interest (VOI) were interrogated using ConSurf, ScoreCons, & Ingenuity Pathway Analysis Software to predict their potential functional effects, and Copy number variations (CNVs) in the genes of interest were analyzed by Genewiz (Azenta Life Sciences). RESULTS: Of 40 VOI involving thirty-six genes, 7 were pathogenic, 17 likely-pathogenic, and 16 of uncertain-significance. Of seven pathogenic NC associated variants, Glucosylceramidase beta 1 [GBA1 c.703T > C (p.S235P)] was mutated in 5/21 (24%), Coagulation factor VIII [F8 c.3637dupA (p.I1213fs*28)] in 4/21 (19%), Phenylalanine hydroxylase [PAH c.975C > A (p.Y325*)] in 3/21 (14%), and Fanconi anemia complementation group C [FANCC c.1162G > T (p.G388*)], Chromodomain helicase DNA binding protein 7 [CHD7 c.2839C > T (p.R947*)], Myosin VIIA [MYO7A c.940G > T (p.E314*)] and Dynein axonemal heavy chain 11 [DNAH11 c.3544C > T (p.R1182*)] in 2/21 (9.5%) NCs respectively. CNVs were noted in 85% of these latter 7 genes. Interestingly, a Carboxy-terminal domain RNA polymerase II polypeptide A small phosphatase 2 [CTDSP2 c.472G > A (p.E158K)] of uncertain significance was also found in > 70% of NC cases. INTERPRETATION: The variants of interest we identified in the NCs regulate a variety of neurological processes including cilia motility, cell metabolism, immune responses, and DNA damage repair and provide novel insights into the molecular pathogenesis of these extremely rare tumors.

Gamma Knife radiosurgery for central neurocytoma: a quantitative systematic review and metanalysis.

Central neurocytomas (CN) are rare tumors within the central nervous system. Originating from the septum pellucidum and subependymal cells, they are typically found in the third and lateral ventricles. For this reason, they may lead to hydrocephalus and increased intracranial pressure. CNs are generally benign lesions that exhibit locally aggressive behavior and a high recurrence rate. Complete surgical resection is the preferred treatment; however, due to their anatomical location, this is often not feasible. Based on these findings, Gamma Knife radiosurgery (GKRS) has been introduced for managing both residual and recurrent tumors and as an initial therapy in selected cases. This study aimed to systematically review the available knowledge regarding GKRS for CN. A systematic investigation of the scientific literature was undertaken through an exhaustive search across prominent databases, including PubMed, Web of Science, and Google Scholar, by employing precise MeSH terms such as "Central neurocytoma," "Radiosurgery," "Gamma Knife," and "Stereotactic Radiosurgery." A comprehensive quantitative systematic review and meta-analysis were meticulously conducted, focusing on cases of CN treated with GKRS for a thorough evaluation of outcomes and efficacy. Seventeen articles, including 289 patients, met the inclusion criteria. Random effects meta-analysis estimates for disease control and local tumor control were 90% (95% CI 87-93%; I2 = 0%, p < 0.74) and 94% (95% CI 92-97%; I2 = 0%, p < 0.98), respectively. When considering only studies with at least 5 years of follow-up, progression-free survival was 89% (95% CI 85-94%; I2 = 0.03%, p < 0.74). The mean clinical control rate was 96%. This systematic review and meta-analysis confirmed the safety and efficacy of GKRS in managing CN.

Survival outcomes of intracranial extraventricular neurocytomas: a systematic review and individual patient data meta-analysis.

OBJECTIVE: The rarity of intracranial extraventricular neurocytomas (EVNs) has precluded accurate definition of its surgical characteristics to date. The authors present the first survival analysis of this unique entity that aims to clarify tumor characteristics, surgical outcomes, and efficacy of postoperative adjuvant therapy. METHODS: A systematic review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Searches of the MEDLINE, Embase, Scopus, Google Scholar, and Cochrane Database of Systematic Reviews databases were performed from inception to date. Primary outcomes were progression-free survival (PFS) and overall survival (OS). Prognostic variables were age, sex, tumor consistency, extent of resection, and receipt of postoperative adjuvant therapy. Survival data were analyzed using Kaplan-Meier survival curves and the log-rank test to compare dichotomized cohorts. Multivariate Cox regression models were constructed, interrogated with Schoenfeld residuals, and subsequently utilized to identify independent prognostic factors. Risk of bias was assessed with the Mayo Clinic instrument. RESULTS: Five hundred fourteen articles were initially retrieved, which was distilled to 10 included articles consisting of 101 cases of intracranial EVNs. The 5-year OS rate was 90.4% (95% CI 81.8%-99.8%) and the PFS rate was 48.6% (95% CI 34.46%-68.8%). The median PFS was 60 months. Patients younger than 50 years of age experienced superior OS (p = 0.03) and PFS (p < 0.01). Gross-total resection (GTR) was superior to subtotal resection (STR) in reducing mortality (p < 0.01). Adjuvant therapy following either STR or GTR did not significantly improve survival. CONCLUSIONS: Intracranial EVNs are rare tumors that portend a poorer prognosis than central neurocytomas, despite both being WHO grade 2 tumors. Complete surgical extirpation is the cornerstone of management. There is no clearly established role for adjuvant postoperative therapy, but each case should be managed on an individual basis.