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.

Central neurocytomas in children: Clinicopathologic features and long-term surgical outcomes.

PURPOSE: Pediatric central neurocytomas (PCNs) is an extremely rare entity, and very few reports have exclusively focused on the clinicopathologic features and surgical outcomes of PCNs. METHODS: This study was undertaken to elucidate the clinical and pathological characteristics and long-term surgical outcomes of PCNs. RESULTS: Fourteen consecutive patients with pathologically verified PCNs were identified, including 9 girls and 5 boys, with a mean age of 15.2 ± 3.9 years (range 3-18 years). Children tended to present with symptoms of increased intracranial pressure (ICP) (p = 0.003), an acute clinical course (p = 0.044), and a shorter duration of symptoms (p = 0.019) than their adult counterparts. Surgery was performed under the guidance of a neuronavigation system and intraoperative ultrasound; gross total resection (GTR) was achieved in 12 patients (85.7%), and subtotal resection (STR) was achieved in two patients (14.3%). One child received adjuvant radiotherapy (RT) owing to the atypical features of the lesion. During a mean follow-up of 90.2 ± 37.0 months (range 42-156 months), one patient (7.1%) with atypical features experienced lesion relapse 2 years after surgery and received RT, and the symptoms of all children were relieved. CONCLUSION: Surgery can benefit children with CNs and ensure relatively long-term progression-free survival. RT can be administered to patients with residual or relapsed lesions.

Pediatric atypical extraventricular neurocytoma: Clinical features and survival outcomes.

BACKGROUND: Extraventricular neurocytoma (EVN) in children is an extremely rare encountered tumor. Little information is available about the treatment and prognosis of this rare disease in children. This study was undertaken to elucidate the clinical-radiological characteristics and treatment outcomes of pediatric patients with atypical EVN. METHODS: A retrospective review of patients' demographic features, treatment modalities, and outcomes in our institution from January 2011 to December 2019 was conducted. RESULTS: Seven consecutive children harboring atypical EVN in our center were included, with a male predominance (n = 5, 71.4%) and a mean age of 11.8 ± 4.9 years (range: 2-18 years). The lesions mainly involved the frontal (n = 4, 57.1%) and temporal lobes (n = 4, 57.1%). Gross total resection (GTR) was achieved in 6 patients (85.7%), and subtotal resection (STR) was achieved in the remaining patient (14.3%). All lesions demonstrated a high Ki-67 index (≧5%) and atypical features pathologically. Five patients (71.4%) received radiotherapy and/or chemotherapy after surgery. During follow-up, 5 patients (71.4%) experienced lesion progression, and 2 (14.3%) of them died. The median progression-free survival was 48 months. CONCLUSIONS: The prognosis of pediatric patients with atypical EVN was dismal after aggressive treatment. Most tumors progressed, and this progression was positively correlated with the Ki-67 index. Surgical excision is the main treatment modality for atypical EVN, and radiation/chemotherapy should also be introduced after surgery.

Central neurocytomas: research trends, most cited papers, and scientometrics analysis to date.

Central neurocytoma is the most common primary intraventricular tumor in adults being classified by the World Health Organization (WHO) as a benign grade II tumor with a good prognosis. Given the recent advances with regard to this tumor, a bibliometric analysis was due to study the future direction of research for neurocytomas. A comprehensive Elsevier's Scopus database search was performed to capture all published and indexed studies to date relevant to neurocytoma. A discrete set of validated bibliometric parameters were extracted and analyzed on R v4.1.3. A total of 1002 documents were included in our analysis covering a period between 1910 and 2021 (111 years). Around 98.5% of the documents were multi-author publications with a collaboration index (CI) of 4.21. Acta Neuropathologica, The American Journal of Surgical Pathology, and Cancer were the journals to include the highest number of top ten cited articles (2 out of 10 most cited articles, 20%). Switzerland (4 out of 10, 40%) accounted for the country to have the highest number of top 10 most cited articles with the USA (5588 out of 16,395 citations, 34.1%) having the greatest number of citations. Lastly, our analysis reported an annual growth rate of 6.9% for the number of papers produced by year. This is the first bibliometric analysis to study the top 10 most cited articles with regard to neurocytomas. A shift from histopathologic and clinical symptoms towards the treatment and management of the tumor was observed in our analysis.

Comparison of surgery with or without adjuvant radiotherapy in treating central neurocytoma: a single-center retrospective real-world study.

PURPOSE: To investigate the efficacy and safety of adjuvant radiotherapy (RT) in patients with central neurocytoma (CN). METHODS: The study included 68 patients with CN retrospectively, was further divided into surgery + RT group (31 patients) and surgery alone group (37 patients). Progression-free survival (PFS), overall survival (OS), and adverse reactions (AEs) were compared between the two groups. RESULTS: The median follow-up duration was 82.2 (interquartile range, 64.7-104.5) months. Patients in the surgery + RT group tended to have longer PFS than those in the surgery alone group (5-year PFS rate: 92.7% vs. 86.3%; P = 0.074). There was no significant difference in OS between the two groups (5-year OS rate: 96.8% vs. 94.3%; P = 0.639). Subgroup analysis revealed a significant improvement in PFS in patients receiving RT after surgery in patients who underwent subtotal resection (STR) (P = 0.045). In the overall population, univariate multivariate analysis revealed that gross total resection (GTR) (P = 0.002), tumor location in the unilateral ventricle (P = 0.008), and MIB-1 (Ki-67) labeling index (LI) < 5% (P = 0.009) were favorable independent prognostic factors for PFS. Whereas tumor location in the unilateral ventricle (P = 0.043) was a favorable independent prognostic factor for OS. Moreover, RT patients experienced AEs (Grade 1-2, well-tolerated). CONCLUSION: Adjuvant RT in the treatment of CNs showed satisfactory safety, and postoperative RT could improve PFS in STR patients. Furthermore, GTR, tumor development in the unilateral ventricle, and MIB-1 LI < 5% were found to be favorable factors affecting the prognosis of CNs.

Gamma Knife radiosurgery for the treatment of central neurocytoma: a single-institution experience of 25 patients.

Central neurocytomas (CNs) are extremely rare tumors that account for 0.1-0.5% of all intracranial neoplasms. Recently, Gamma Knife radiosurgery (GKRS) has become a treatment option in patients with CN. We aimed to evaluate the efficacy and safety of GKRS in 25 CN patients and review the results along with relevant literature. GKRS patient database was searched, and 25 patients who underwent GKRS for CN between 2009 and 2018, were evaluated retrospectively. The study cohort included 15 female and ten male patients with a median age of 32 years (range, 5-60). The most common presenting symptom was headache (88%). The neurological examination was unremarkable in all patients, except for one patient with decreased vision. Twenty patients (80%) had a history of surgical resection. Most of the tumors (92%) were located in the ventricles, and the median tumor volume was 4.8 cm3 (range, 0.8-28.1). The median marginal dose was 14 Gy (range, 12-15) to a median isodose of 50% (range, 40-50). Following a median follow-up of 80 months (range, 36-138), local tumor control was achieved in 100% of patients. Distant recurrence was observed in one patient (4%). No adverse radiation effect was observed. Regarding non-specific post-GKRS symptoms, one patient experienced a prolonged headache, and one epileptic patient experienced a brief partial seizure. In our patient cohort, GKRS yielded favorable local tumor control (100%) during a median follow-up of 6.6 years. Our series demonstrates that GKRS is an effective and safe treatment option for patients with primary or residual CNs.

[Central neurocytomas: long-term treatment outcomes]. / Tsentral'nye neirotsitomy golovnogo mozga: otdalennye rezul'taty lecheniya.

Central neurocytoma is a rare benign brain tumor. These tumors may be giant and accompanied by compression of ventricular system and surrounding structures. Modern treatment of brain neurocytoma includes extended resection and restoration of normal CSF circulation. Surgical treatment does not often lead to total resection of these tumors. Redo resection was preferred in patients with tumor progression for a long time. In the last decade, various authors report stereotactic irradiation for continued tumor growth to ensure local growth control. This study was aimed at evaluation of postoperative outcomes in patients with brain neurocytomas, as well as treatment of tumor progression in long-term period. OBJECTIVE: To analyze recurrence-free survival in patients with brain neurocytomas, risk factors of recurrence-free survival, effectiveness of various treatments for tumor progression and delayed complications. MATERIAL AND METHODS: Long-term postoperative follow-up data of patients with brain neurocytomas are reported in the manuscript. We analyzed recurrence-free survival and risk factors of recurrence-free survival, treatment outcomes in patients with progression of brain neurocytomas, long-term complications and their prevention. RESULTS: Follow-up included 84 out of 115 patients with brain neurocytoma after surgical treatment in 2008-2017. Follow-up period ranged from 2 to 10 years (mean 6 years) after resection. Most patients had regression of neurological symptoms after surgery. Continued tumor growth within 12-96 months after surgery occurred in 26 (30.19%) out of 84 patients (19 cases after partial resection and 7 cases after total resection according to MRI data). Two-year recurrence-free survival was 94%, 5-year survival - 83%. Risk factors of continued tumor growth were resection quality and Ki-67 index. Redo resection was performed in 7 cases. Eleven patients underwent stereotactic irradiation for tumor progression. Indications for stereotactic irradiation of central neurocytoma are MR data on continued growth of lateral ventricle tumor without signs of ICH and CSF flow impairment. There were no cases of hemorrhage inside the residual tumor and CSF flow impairment in early postoperative period after redo resection. In all cases (n=11), stereotactic irradiation (mean follow-up 2.5 years) ensured satisfactory control of tumor growth with reduction of the neoplasm in 4 cases and no tumor growth in 7 cases. CONCLUSION: Resection of central neurocytoma ensures long-term recurrence-free period. The main causes of tumor recurrence are partial resection and high proliferative activity (Ki-67 index over 5%). Redo resection is advisable for tumor progression followed by CSF flow impairment. In case of continued growth of neurocytoma without signs of intracranial hypertension, stereotactic irradiation with various fractionation modes ensures effective and safe control of tumor growth.

[Central neurocytomas: surgical treatment outcomes and new trends and approaches in the treatment]. / Tsentral'nye neirotsitomy golovnogo mozga: rezul'taty khirurgicheskogo lecheniia i novye tendentsii i strategii v lechenii.

AIM: Central neurocytomas of the brain are rare benign tumors of the cerebral lateral ventricles. The main treatment for them is surgical resection. Resection provides a long-term relapse-free period, but surgical intervention is associated with a number of significant difficulties due to the location, size, and blood supply features of these tumors. The postoperative period is often accompanied by hemorrhagic complications, impaired cerebrospinal fluid circulation, and worsening of neurological symptoms. PURPOSE: The study purpose was to evaluate the effectiveness of surgical treatment in neurocytoma patients, assess the risk of complications after neurocytoma resection, and develop techniques for their prevention. MATERIAL AND METHODS: The paper presents surgical treatment outcomes in 115 patients with central neurocytomas for the period from 2008 to 2017. The choice of a surgical approach and the surgical features are described in detail, and the radicality is assessed with allowance for the location and size of tumors. The immediate clinical outcomes of treatment are evaluated, complications are analyzed, and methods for prevention and treatment of complications are described. RESULTS: Analysis of the data revealed that the tumor was resected totally in 41 (36%) patients, subtotally - in 37 (32%), and partially in 37 (32%). The most common and dangerous complications were as follows: CSF circulation occlusion with the development of hydrocephalus in 23 (20%) patients; hemorrhage into the residual tumor, which required immediate revision of the surgical wound in most cases, in 25 (22%) patients. In the early postoperative period after tumor resection, almost all patients developed deterioration of condition with transient worsening of cerebral and focal symptoms. The degree and duration of this worsening were different. In 50% of cases, the postoperative condition was characterized by reduced voluntary activity, drowsiness or jitteriness, and motor or speech anxiety. CONCLUSION: We consider resection of neurocytomas as the main treatment option, despite the potential complications and effects of the operation.

Imaging characteristics of cerebral extraventricular neurocytoma with pathological correlation.

PURPOSE: Extraventricular neurocytoma (EVN) is an exceedingly rare tumor. In this study, we sought to characterize the imaging and pathological features of this uncommon tumor. METHODS: Retrospective review of 18 patients (9 male; 9 female) with pathologically confirmed EVN treated at a single center between 2005 and 2017. RESULTS: All patients had a solitary lesion. Sixteen lesions were found in hemispheres. The greatest tumor diameter ranged from 2.6 to 8 cm. The lesions were generally solid with cystic components; the solid portion appeared isodense or hyperdense on CT, isointense to hypointense on T1WI, and slightly hyperintense on T2WI. Heterogeneous hyperintensity interspersed with isointense or hypointense areas suggestive of hemorrhage, calcification or vascular flow voids were seen on T2WI. Heterogeneous enhancement was noted in 17 lesions; no enhancement was observed in one lesion. Cystic components were observed in 13 lesions; 9 of these showed characteristic perilesional cysts (9/13). Mild to moderate peritumoral edema (15/18), calcification (4/16), intratumoral hemorrhage (11/18) and vascular flow voids (10/16) were observed in some lesions. Pathologically, tumor cells showed round nucleus and fine neuropil matrix. Foci of calcification in the solid portion of the tumor were seen in five cases. Microcystic changes were observed in almost all cases. Some lesions exhibited positive staining for synaptophysin (Syn) (15/16) and neuronal nuclei (NeuN) (7/8). MIB-1 was determined for 10 patients; seven of these had an MIB-1 ≥ 3. These six patients experienced recurrence; four of them relapsed twice. CONCLUSIONS: EVNs occur as single intracranial solid mass with cystic components (especially peripherally located cysts); solid portion exhibits slight hyperintensity or heterogeneous signal intensity. Mild to moderate peritumoral edema, calcification, intratumoral hemorrhage and vascular flow voids were characteristic features of extraventricular neurocytoma. Positive staining for synaptophysin and neuronal nuclei confirmed the diagnosis. A combination of atypical pathologic features and atypical radiologic features should be considered for prognostic assessment.

Amenorrhoea and reversible infertility due to obstructive hydrocephalus: literature review and case report.

BACKGROUND: Endocrine abnormalities are well-recognized consequences of intracranial pathology such as pituitary tumours. Less commonly, hydrocephalus may lead to dysfunction of the endocrine system, presenting as amenorrhoea or precocious puberty. We present a case report and literature review of hydrocephalus causing endocrine abnormalities including reversible infertility. CASE DESCRIPTION: A 34 year-old female presented with amenorrhoea and infertility. MRI showed a third ventricular mass and hydrocephalus. The amenorrhoea resolved within weeks of endoscopic third ventriculostomy and tumour biopsy; pregnancy ensued within 6 months. Thirty-two cases of hydrocephalus-related amenorrhoea were reported between 1915 and 2007. All patients who underwent modern hydrocephalus treatment experienced partial or complete resolution of endocrine dysfunction. Successful pregnancy was reported in three patients, as in our case presentation. While mechanisms of dysfunction have not been completely elucidated, studies point toward loss of GnRH pulsatility due to compression of the medio-basal hypothalamic structures. CONCLUSION: Hydrocephalus can cause endocrine dysfunction, including amenorrhoea, which may reverse with CSF diversion. Therefore, cranial imaging is an important component in the evaluation of such endocrine abnormalities.

The painter from Sinaloa: artistic analysis of a case of spatial agnosia and neglect of visual shapes.

Paintings produced spontaneously by patients with neurological lesions represent a fascinating opportunity to analyze some aspects of the underlying disease and involved brain mechanisms. Many cases of artists who have suffered spatial neglect following a neurological disease have been reported in the literature. However, only a few studies evaluating the different subtypes of graphic neglect and aspects related to the construction of perspective (three dimensionality) in works of art have been published. In the present article, we present the case of an artist who, after resection of a central neurocytoma that affected the right thalamo-parietal connections, suffered an impairment of the ability to create perspective in his paintings and involuntary omission of only shapes in the left side of his paintings, although colors and contours were preserved.

Ventricular Central Neurocytoma: Rate of Shunting and Outcome 2 Years After Total and Subtotal Excision.

BACKGROUND: Central neurocytoma is an intraventricular tumor that affects young adults. It has a favorable prognosis after adequate surgical intervention; however, an aggressive course may take place in some cases. OBJECTIVE: The objective of the study was to evaluate the rate of shunting and the outcome of control measures in patients with central neurocytoma submitted to total and subtotal excision. METHODS: Twelve patients were included in this study, with a follow-up of 24 months. Data collected included: age, sex, clinical presentation, early morbidity and mortality, radiological findings (tumor location, features, residual, recurrence, and hydrocephalus). All patients underwent surgery for total or subtotal excision through a transcortical approach. External Ventricular Drain (EVD) was inserted then removed or replaced by a shunt. Histopathology and the MIB index were used to confirm diagnosis and guide the follow-up; adjuvant radiotherapy or Gamma Knife radiosurgery were used for residual tumor or recurrence. RESULTS: The ages of the patients ranged from 14 to 48 years. Two patients died early, after total and subtotal excision, from sepsis and thalamic infarction, respectively. Six patients (60 %) had a total excision; two of them had a high MIB index and showed small recurrence at 12 months and 18 months, respectively, and received Gamma Knife radiosurgery. One of the six patients with total excision needed a shunt, and no shunt was needed in the four otherpatients; a subtotal excision was done for four patients (40 %). An early shunt was inserted for two of these patients, radiosurgery-controlled for one patient, while radiotherapy was used for control in the other three patients; radiotherapy control failed in one patient, who underwent a second surgery at 18 months. CONCLUSION: Central neurocytoma may have a favorable prognosis, with a lower incidence of shunt insertion throughout its course than that for other intraventricular tumors, if total removal is achieved.

Cavernoma of the septum pellucidum in the region of foramen of Monro.

A rare case of cavernoma in the region of the septum pellucidum is reported. A 35-year-old female patient presented with chronic headaches. Her neurological exam was normal. Her magnetic resonance (MR) imaging showed a lesion within the inferior aspect of the septum pellucidum extending into the anterior third ventricular region, blocking the foramen of Monro, resulting in moderate supratentorial asymmetrical hydrocephalus. A central neurocytoma or subependymoma was suspected on imaging. Complete excision of the septum pellucidum cavernoma was performed using microneurosurgical techniques through an interhemispheric transcallosal route. The patient had an excellent outcome and is cured. Although rare, septum pellucidum cavernomas should be considered in the differential diagnosis of anterior third ventricular lesions in the region of foramen of Monro. The unusual location, atypical radiological features, differential diagnosis as well as surgical nuances in the management of a cavernoma in the septum pellucidum and anterior third ventricular region are discussed in the light of current literature.

[Multiple primary liponeurocytoma of the central nervous system].

We report a unique case of primary multiple liponeurocytoma. Liponeurocytoma is a rare benign tumor (Grade II) of the posterior cranial fossa with neural- or probably astrocytic-lineage cells; the tumor stroma contains mature adipocytes. This case is the thirty-eighth case of this pathology reported in the world literature and the first case of multiple form of this disease. We have provided a sufficiently thorough neuroradiological and histological picture that allows one to differentiate between a liponeurocytoma and other histological variants. Recommendations for the tactics for managing patients with this rare disease are proposed based on the analysis of treatment of this pathology reported in the world literature.

Mesial temporal extraventricular neurocytoma: a rare cause of refractory complex partial seizure.

AIM: Mesial temporal extraventricular neurocytoma (mtEVN) is a rare cause of refractory complex focal seizures. The characteristics of this clinical entity are discussed in this article. METHODS: We report two cases of mtEVN and review the related literature, with particular emphasis on radiological characteristics, clinical features, and operative techniques. RESULTS: After successful surgery, our two cases of mtEVN achieved excellent outcome. Including the cases presented here, a total of three cases of mtEVNs and 11 of neocortical temporal extraventricular neurocytoma (ntEVNs) are reported in the literature. mtEVNs are distinct from ntEVNs with regards to demographics, aetiology, radiological features, and operative techniques. CONCLUSION: mtEVNs and ntEVNs exhibit distinguishing features. Under electrocorticographic monitoring, tailored resection of the neocortical epileptogenic focus, as well as the entire tumour and mesial temporal structures, can yield excellent outcome and satisfactory seizure control.

Primary central neurocytoma of the mesencephalic tectum in a pediatric patient.

PURPOSE: Neurocytomas are tumors or neuronal differentiation, typically located within the supratentorial ventricular system. The extraventricular location is uncommon. A limited number of cases involving the brainstem have been reported and may be misdiagnosed as brainstem gliomas. Furthermore, midbrain neurocytomas are extremely rare, and no similar cases in pediatric patients have been reported in the literature to date. Brainstem location of neurocytomas often precludes gross total removal of the lesion, and in these cases, adjuvant therapies may be helpful. METHODS: We report a case of a 16-year-old child who presented with signs and symptoms of increased intracranial pressure. The magnetic resonance imaging study demonstrated the presence of a primary mesencephalic tectum lesion causing obstructive hydrocephalus. The patient underwent emergent ventriculoperitoneal shunt implantation, resolving the hydrocephalus and the clinical symptoms. The lesion was partially removed through a suboccipital craniotomy and supracerebellar infratentorial approach to the mesencephalic tectum, without intraoperative complications. RESULTS: Histological examination of the lesion was consistent with the diagnosis of extraventricular neurocytoma. The patient was referred to the oncology department for additional treatment with Gamma Knife radiosurgery. CONCLUSIONS: Although brainstem neurocytoma is rare, this case demonstrates that it should be included in the differential diagnosis of brainstem gliomas. Because of brainstem tumor location, complete surgical removal may be challenging or not possible, with a high risk of postoperative neurological deficits. Adjuvant therapies may prevent local tumor growth in cases of tumor remnants or recurrences following microsurgery in selected cases.

Cerebellar liponeurocytoma with extracranial extension: case report.

Cerebellar liponeurocytoma is a newly recognized, rare clinicopathological entity commonly described in the cerebellar hemispheres or the vermis. We present a rare case of cerebellar liponeurocytoma arising from the left cerebellar amygdala with extracranial extension. Such a condition has never been previously reported.