Neurocytoma Research Articles

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

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. 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). 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. 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.

Diagnosis and treatment of supratentorial tumors with small round cell malignancies diagnosed by intraoperative rapid pathology

Objective To explore the diagnosis and treatment of supratentorial tumors with small round cell malignancies diagnosed by intraoperative rapid pathology. Methods A retrospective analysis was conducted on the clinical data of 36 patients with supratentorial tumors diagnosed as small round cell malignant tumors by intraoperative frozen pathology at Department of Neurosurgery, Nanfang Hospital, Southern Medical University from January 2011 to December 2017. All patients were treated with complete tumor resection according to the principle of glioma operation. After the operation, the MRI of the head was reexamined, and the volume quantitative analysis was used to determine the degree of tumor resection, which was divided into complete resection, subtotal resection and partial resection. All patients were followed up regularly in outpatient department, and MRI was reexamined to explore the recurrence of tumor. Results MRI of 36 patients after operation showed that 31 patients (86.1%) had complete resection and 5 patients (13.9%) had subtotal resection. The results of pathological diagnosis were as follows: primary central nervous system lymphoma (10 cases), glioblastoma (6 cases), central neurocytoma (5 cases), primitive neuroectodermal tumors (3 cases), brain metastasis tumor (2 cases), pineal cell tumors(2 cases), ependymoblastoma (2 cases), World Health Organization (WHO) grade Ⅱ glioma (2 cases), pituitary adenoma (1 case), periangioblastoma (1 case), olfactory neuroblastoma(1 case) and desmoplastic small round cell tumor (1 case). Among the 33 patients diagnosed by MRI before operation, 8 (24.2%) had the same pathological results with preoperative diagnosis, including 4 cases of lymphoma, 1 case of central neurocytoma, 1 case of primitive neuroectodermal tumor, 1 case of hemangioblastoma and 1 case of metastasis. The follow-up duration of 36 patients was 28.9±8.1 months and ranged from 6 to 70 months . During the follow-up period, 26 out of 36 patients (72.2%) relapsed. Conclusions The supratentorial tumors with small round cell malignant tumor diagnosed by rapid pathology during operation should be resected in the maximal range under the premise of safety, the pathological results are various and its therapeutic effect seems relatively good. Key words: Supratentorial tumor; Pathology; Treatment outcome; In operation; Small round cell tumor

Neurocitoma extraventricular: reporte de caso y revisión de la literatura

El neurocitoma extraventricular (NEV) es una neoplasia del SNC con diferenciación neuronal, infrecuente, cuya incidencia hasta ahora ha sido reportada como 0,02-0,13% de los tumores intracraneales. Fue descrita por primera vez en el año 1992 por Nishio et al. y ha sido introducida recientemente por la Organización Mundial de la Salud (OMS) en su cuarta edición de la clasificación de los tumores del sistema nervioso central (SNC). Esta entidad presenta características y comportamiento similares al neurocitoma central (NC), pero se localiza en tejido parenquimatoso extraventricular. Se presenta el caso de un paciente varón de 28 años quien acude al servicio de urgencias del Hospital Universitario del Caribe por cefalea intensa, de predomino en la madrugada y la mañana, asociada a náusea y emesis, marcada somnolencia, lenguaje confuso, no control de esfínteres; y pérdida de fuerza en hemicuerpo izquierdo. Las imágenes diagnósticas (tomografía computarizada y resonancia magnética) identifican una lesión cortico-subcortical a nivel parietooccipital izquierdo. La evaluación de esta por hematoxilina y eosina permitió un diagnóstico preliminar de Tumor de Bajo Grado, de posible origen glioneuronal. Posteriormente la inmunomarcación reportó positividad en las células tumorales para Sinaptofisina, y negatividad para GFAP (Proteína Ácida Fibrilar Glial) e Inhibina. Teniendo en cuenta dichos resultados en la tinción para inmunohistoquímica, se reporta la lesión como un Neurocitoma Extraventricular Parietooccipital Izquierdo WHO grado II.

Diagnostic value of magnetic resonance imaging and hydrogen ion magnetic resonance spectroscopy for central neurocytoma

Objective To analyze the clinical value of magnetic resonance imaging (MRI) and hydrogen proton magnetic resonance spectroscopy (~1H-MRS) in the diagnosis of central neurocytoma (CNC). Methods From February 2010 to February 2018, the clinical data of 12 patients with CNC confirmed by surgery and pathology in Quzhou People's Hospital were retrospectively analyzed.All patients underwent MRI diagnosis before operation.Among them, 9 patients received ~1H-MRS check. Results In this group of 12 patients, 9 patients occurred in the 2/3 area of the lateral ventricle, 2 cases occurred in the posterior third of the lateral ventricle, 1 case occurred in the frontal lobe; 11 cases were solid tumors, and 1 case was cystic tumors.MRI showed plaque calcification in the tumor, distortion of vascular shadow, T1WI was equal low signal, T2WI was contoured mixed signal; 8 cases showed uneven light and moderate enhancement, 3 cases showed uneven enhancement, 1 case showed ring enhancement; 8 cases of ~1H-MRS showed a significant increase in choline peak (Cho), N-acetylaspartate (NAA) peak decreased, and Cho/NAA increased.Meanwhile, 1 case of glycine peak (Gly) increased. Conclusion CNC has specific MRI performance, and plays an important role in preoperative diagnosis and differential diagnosis.Combined with ~1H-MRS examination, it can grasp the tumor biochemical index and metabolism from the molecular level and improve the accuracy of preoperative diagnosis. Key words: magnetic resonance imaging; hydrogen proton magnetic resonance spectroscopy; central neurocytoma; differential diagnosis

Atypical central neurocytoma with leptomeningeal dissemination

Atypical central neurocytoma with leptomeningeal dissemination

Sellar/suprasellar extraventricular neurocytoma

Objective To explore the clinicopathological features of extraventricular neurocytoma located in the sellar/suprasellar region. Methods The clinical manifestations, neuroimaging, histopathological, immunohistochemical and molecular genetic features were retrospectively analyzed in one case of sellar/suprasellar extraventricular neurocytoma, and the related literatures were reviewed. Results A 27-year-old female presented with intermittent headache, accompanied by blurred vision for 5 months. Head MRI demonstrated a mass with a well-defined margin measuring 3.80 cm × 2.50 cm × 3.40 cm located in the sellar/suprasellar region. The tumor showed isointense to hyperintense signals on T 1 WI and hyper-hypointense mixed signals on T 2 WI, and slightly hyperintense signal on diffusion-weighted imaging (DWI). The pituitary was not shown. A transsphenoidal sellar tumor resection, cerebrospinal fluid (CSF) rhinorrhea repairing and optic decompression were performed. The mass was lightly yellow and tough with abundant blood supply and filled with old hemorrhage. The pituitary tissue was pushed to the left rear. Microscopy examination showed a diffuse invasive growth pattern with neuropil background in some area. The tumor cells were uniform on size and shape with round to oval, exquisite and hyperchromatic nuclei. No mitosis was found. Immunohistochemical staining showed the tumor cells were positive for neuronal nuclei (NeuN) and thyroid transcription factor-1 (TTF-1) in nuclei, calretinin (CR) in nuclei and cytoplasm, synaptophysin (Syn), chromogranin A (CgA), E-cadherin, matrix metalloproteinase-9 (MMP-9) in cytoplasm, and focally positive for S-100 protein (S-100) in nuclei, and neurofilament protein (NF), cytokeratin 8 (CK8) and vimentin (Vim) in cytoplasm. The Ki-67 labeling index was about 3%. The tumor tissue was negative for reticular fiber staining. Molecular genetic analysis showed that isocitrate dehydrogenasel (IDH) gene was not mutated, and 1p/19q was intact in tumor cells. The final pathological diagnosis was extraventricular neurocytoma, WHO grade Ⅱ. Conclusions Extraventricular neurocytoma located in the sellar/suprasellar region is very rare. The histological features are similar to central neurocytoma in ventricle. Tumor cells were in diffusely invasive growth and were uniform in size and shape, with round nuclei. Fibrillary areas mimicking neurophil and branching thin-walled capillaries can be seen. The differential diagnosis includes pituitary adenoma, oligodendroglioma, clear cell ependymoma, and so on. DOI: 10.3969/j.issn.1672-6731.2017.12.009

The clinical application of the telovelar approach in tumor resection of the fourth ventricle

Objective To investigate the safety of telovelar approach for removing the fourth ventricle tumors. Methods The clinical data of 30 patients with fourth ventricle tumors was analyzed from July, 2012 to July, 2017 in our hospital. All patients were treated through telovelar approach. Results 25 cases had total tumor resection and 5 cases had subtotal resection. 24 cases recovered well after operation [Glasgow treatment outcome classification (GOS): 5 points]. 4 cases suffered from facial numbness and deviated mouth (GOS: 4 points), limb hemiparesis occurred in one case (GOS: 3 points). One patient died from pulmonary infection (GOS: 1 points). None of the survived patients underwent reticence, and the follow-up duration was 2 to 40 months. No obvious tumor recurrence occurred. Pathology results confirmed: 12 cases of ependymoma, 5 cases of astrocytoma, 2 cases of arachnoid cyst, 2 cases of medulloblastoma, 2 cases of choroid plexus papilloma, 2 cases of metastatic carcinoma, 2 cases of central neurocytoma, 2 cases of hemangioblastoma and 1 case of cavernous hemangioma. Conclusion Resection of the fourth ventricle tumors is very difficult. The tumors can fully be exposed through telovelar approach which avoiding the incision of the vermis and reducing the complications. Key words: Telovelar approach; Fourth ventricle tumors; Total tumor resection; Safety

Gamma knife radiosurgery for central neurocytoma

Objectives To evaluate the efficacy of gamma knife radiosurgery for the treatment of central neurocytoma and to discuss the treatment strategies. Methods Nine patients with central neurocytoma treated with gamma knife radiosurgery were analyzed retrospectively. They were confirmed by surgical pathology. A total of 12 tumor foci were treated. The tumor volume ranged from 0.04 to 14.3 cm3 (mean volume 3.75 cm3). The mean radiation dose of tumor margin was 13.7Gy (range, 11 to 15 Gy). The mean central dose was 26.8 Gy (range, 18 to 33.3 Gy). The follow up was assessed according to the MRI findings before and after gamma knife treatment and long-term quality of life (karnofsky performance scale, KPS). Results The mean follow-up time was 46 months. Magnetic resonance imaging showed the tumor reduction in 5 cases, tumor disappearance in 1 case, no change in 1 case, and tumor recurrence in 2 cases. Four patients had 10-point improvement in the KPS score, and 3 did not have any change in the KPS score. The KPS score decreased in 1 case. One patient had intraventricular dissemination and died of tumor recurrence at 42.5 months after gamma knife radiosurgery. The total effective rate of gamma knife treatment was 78%. One patient had enlarged cerebral ventricles and recovered at 6 months after gamma knife radiosurgery. No radiation-related complications occurred. Conclusions Residual or recurrent central neurocytoma after gamma knife radiosurgery is safe and effective. In addition to assessing tumor local control, clinical follow-up also needs to pay attention to the problems of possible ventricle changes and tumor dissemination. Key words: Gamma knife radiosurgery; Central neurocytoma; Radiosurgery

Role of adjuvant radiation in the management of central neurocytoma: Experience from a tertiary cancer care center of India.

Neurocytoma (NC) is a rare benign neuronal tumor. A complete excision remains curative for most of these tumors, but atypical histology and extra-ventricular location often necessitates adjuvant therapy. We intended to explore the clinico-pathological features and treatment outcome in patients of NC in our institute. Medical records were reviewed and data collected on NC over a 6-year period (2006-2012) from the departmental archives. Disease free survival (DFS) was analyzed by Kaplan-Meier method. A total of 18 patients met the study criteria. Fourteen patients had intra-ventricular neurocytoma (IVNC), right lateral ventricle being the most common site of origin. Gross total resection and near total resection were achieved in eight cases each whereas tumor decompression and biopsy could be done in two cases. On post-operative histopathological examination, eight patients were found to have atypical NC while 10 patients had typical NC. All patients underwent adjuvant radiation. The median dose of post-operative radiation was 56 Gy. All patients were alive at their final follow-up. One patient had both clinical and radiological evidence of local relapse. In the evaluable patients (n = 18), after a median follow-up of 35 months the DFS rate at 2 years and 3 years are 100% and 83% respectively. Use of adjuvant radiation to a total dose of 56 Gy enhances the local control and achieves superior survival in patients of NC. Use of 3D conformal planning techniques may help us to achieve better therapeutic ratio in patients with NC.

Clinicopathological analysis of unusual rosette-forming glioneuronal tumor in brain parenchyma

Background Rosette-forming glioneuronal tumor (RGNT) is a rare and novel brain tumor. It affects mainly young adults and arises in the midline, primarily involving the cerebellum, and the walls or floor of the fourth ventricle. The tumor is composed of distinctive histological components, uniform neurocytes forming rosettes and (or) perivascular pseudorosettes, as well as astrocytic component resembling pilocytic astrocytoma. To our best knowledge, no more than 50 cases of RGNT have been described in the literatures to date and found commonly in association with the ventricular system. Only a few cases have been known to occur at sites outside of its usual location. Herein, we present a rare case of RGNT of brain parenchyma. Due to its rarity and non-specific appearance in radiological examination, it is a diagnostic challenge for radiologists and histopathologists to differentiate RGNT in unusual sites from other intracranial lesions because of its similarities in radiological and histological findings. The aim of this study is to summarize the clinicopathological features of RGNT and discuss the differential diagnosis of histologically similar tumors in brain. Methods The clinical manifestation of a patient with RGNT occurring in left frontal lobe was presented retrospectively. Resected mass was routinely paraffin-embedded and stained with Hematoxylin and Eosin. Dako EnVision immunohistochemical staining system was used to detect the tumor antigen expressions, including glial fibrillary acidic protein (GFAP), S-100 protein (S-100), cytokeratin (CK), neuronal nuclear antigen (NeuN), synaptophysin (Syn), neuron-specific enolase (NSE), chromogranin A (CgA), oligodendrocytes transcription factor-2 (Olig-2), epithelial membrane antigen (EMA) and Ki-67 (MIB-1). Results A 12-year-old girl presented with 2-year history of twitches and mild headache. MRI revealed a solid well-circumscribed lesion in left frontal lobe with mild heterogeneous enhancement. The lesion was observed to locate in brain parenchyma and there was no evidence of tumor infiltrating in ventricular system. Craniotomy was performed and the tumor was removed totally. Histological examination revealed that the tumor was distinctive in its juxtaposition of patterned neurocytic and pilocytic astroglial components. The neurocytic component showed the tumor cells had small, uniform round nuclei with scant cytoplasm and formed narrow perivascular pseudorosettes or Homer-Wright-like rosettes arrays of neurocytic nuclei around delicate eosinophilic neuropil cores. The glial component tended to exhibit pilocytic astrocytoma-like morphology with long, hair-like processes and Rosenthal fibers. Immunohistochemical staining showed that the tumor cells in glial component were diffusely positive for GFAP and S-100, but negative for NeuN, Syn and NSE. However, perivascular pseudorosettes or Homer-Wright-like rosettes were positive for Syn and Olig-2, and negative for GFAP. Ki-67 index was low and less than 1%. Based on clinical presentation and histological findings, a final histological diagnosis of RGNT in brain parenchyma, WHO grade Ⅰ, was made according to the criteria of WHO classification. The patient has not received chemotherapy and attended follow-up for 12 months, without any neurological deficit or signs of recurrence. Conclusions RGNT is a rare tumor and classified as mixed neuronal-glial tumor. RGNT probably derives from a common progenitor cell originated from subependymal plate or brain parenchyma, able to differentiate toward both glial and neuronal phenotype. RGNT in brain parenchyma is also observed to have the similar biological behaviors and histopathological characteristics with its intra-ventricular counterpart. With similarities in histological findings, it may be difficult to differentiate RGNT from extraventricular neurocytoma, dysembry oplastic neuroepithelial tumor (DNT), and ependymoma with neuronal differentiation or neuropil-like islands. The presence of distinct low-grade glial component in the tumor and appropriate immunohistochemical profile are necessary for correct diagnosis. doi: 10.3969/j.issn.1672-6731.2014.03.014

Detection on clinical pathology features of central neurocytoma by using Syn, GFAP, S-100 and NSE

Objective To investigate the clinical and pathological features of central neurocytoma (CNC),diagnosis and differential diagnosis,tissue and clinical prognosis.Methods 37 cases of brain glial cell tumors,immunohistochemistry synaptophysin (Syn),neuron-specific enolase (NSE),glial fibrillary acidic protein (GFAP),S-100 detection,detection of 9 cases of CNC.7 cases were aecompanied by differentiation characteristics of CNC.Results 6 cases were male,3 female,mean age 38.Clinical manifestations of headache,dizziness,vomiting,convulsions,coma symptoms,limb weakness.Location:2 cases occurred in the lateral ventricles,7 cases occurred in the external ventricles.The size of the tumor diameter was 3-7.5 cm.Light microscopy of tumor cells with uniform rouud,less cytoplasm,transparent,nucleus was small and round,chromatin was uniform,stippling,nuclear center,nuclear Zhou Kong halo,resembling the oligodendrocyte,tumor cells diffusely arranged densely,visible with a chrysanthemum shape,cell free zone neuropil like structure,interstitial,abundant blood vessels,slender,branched,a few appeared hyperplasia,calcifications and cystic degeneration.A few cases had nuclear atypia,mitotic and focal necrosis visible.Immunohistochemistry:Syn (strong positive 7/9,positive 2/9),S-100(strong positive 4/9,positive 4/9),NSE (weak positive 6/9),GFAP (positive 3/9,weak positive 4/9),Vimentin (strong positive 2/9,positive 1/9,weak positive 1/9),Ki67(weak positive 9/9),NF (weak positive 3/9).Follow-up of 8 cases,6 cases died,2 cases survived.Conclusions CNC morphologic features have multiple differentiation,which can occur within the ventricles,are also found in the brain of outdoor,misdiagnosed as ependymoma,oligodendroglioma and medulloblastoma tumors.Immune phenotype with differentiation,ventricle external CNC may be a glial cell differentiation gene type of the tumor,but for a prompt accompanied by glial cell differentiation of CNC,with biological behavior instability,poor clinical prognosis.Immunohistochemical Syn,GFAP are helpful to the diagnosis and differential diagnosis.High expression of GFAP and Ki67,suggests poor clinical prognosis. Key words: Brain tumor; Neurocytoma; Glial cell differentiation; Central; Immunohistochemistry; Diagnosis; Prognosis

Central Neurocytomas

Objective:Neurocytomas (NCs) comprise a rare but distinct clinicopathologic entity, often with a benign course. Our operative series of 33 patients with NCs attempts to review the clinical characteristics of this rare entity.Method:Retrospective analysis of the case records of all the patients with

Anterior interhemispheric transcallosal approach for removal of central neurocytoma in lateral ventricle

Objective To investigate the effect of anterior interhemispheric transcallosal approach for removal of central neurocytoma in lateral ventricle.Methods From Apt 1996 to Jun 2010,central neurocytomas in lateral ventricles were removed through anterior interhemispheric transcallosal approaches in 16 cases,and the clinical data were retrospective analyzed.Results The diameters of the tumors were between 2 centimetres and 4 centimetres in 7 cases,more than 4 centimetre in 9 cases.The bottom of the tumor was located in septum pellucidum in 9 cases,in lateral wall of the ventricle in 7 cases.7 cases had hydrocephalus.Total removal was achieved in 12 cases.4 patients of subtotal resection of tumors received radiotherapy after operation.There was no perioperative death.14 patients were followed - up from 1year to 7 years and there was no recurrence.Conclusions The anterior interhemispheric transcallosal approach is a valid choice for removal of central neurocytoma in lateral ventricle.Through this approach,protection of normal brain tissue by using of natural brain cavity and prevention of postoperative complications could be achieved.The relationship between the tumor and lateral wall or bottom of ventricle is the main factor that influences the total removal of tumor. Key words: Anterior interhemispheric transcallosal approach; Microsurgery; Central neurocytoma; Lateral ventricle

Angiographic characteristics and clinical analysis of central neurocytomas

Objective To analyze the angiographic characteristics of central neurocytomas and discuss their significance to surgical strategies.Method The angiographic characteristics of 27 cases of central neurocytoma were retrospectively analyzed.Their surgical procedures were reviewed and cerebral angiographies were thoroughly examined in arterial,capillary and venous phases.Results The degree of tumor - staining was related to the size of tumor.These tumors commonly received their blood supply from the medial and lateral posterior choroidal arteries.The enlarged ipsilateral thalamostriate veins were elevated upward and the ipsilateral internal cerebral veins were depressed downward in the lateral projection of the carotid angiography.The venous angle were irregularly widened.Conclusions Surgery should be the best strategy for central neurocytomas.The angiography should be listed as the essential examination so that the eloquent structure could be protected and the GTR rate is improved. Key words: Central nervous system neoplasms; Cerebral angiography; Microsurgery

Central neurocytomas:clinical analysis of 94 cases

Objective To analyze the clinical characteristics of central neurocytomas,and discuss the therapeutic strategies.Methods 94 cases of central neurocytomas were studied retrospectively.All patients underwent operation with removal of the tumor through either transcallosal or transcortical approach.Total resection was achieved in 66 patients,subtotal resection in 26 patients and partial resection in 2 patients.The clinical,radiological,histologic and immunohistochemical features of these patients were reviewed and analyzed.Methods Amongthe 94 cases of central neurocytomas,two died after surgery.Among the 84 followed- up cases,56 cases underwent postoperative radiotherapy,4 cases had recurrence.Most patients have favorable prognosis.Conclusion Central neurocytomas occur mostly in the lateral ventricle near the Monro's foramen.Total resection is the best treatment.Postoperative radiotherapy for partially removed tumors may reduce the possibility of recurrence. Key words: Central neurocytoma; Intraventricular tumor; Microsurgery; Prognosis

Central neurocytoma of cervical spinal coed: a case report

患者男,27岁.因右侧上肢乏力、右侧上下肢体感觉异常2年于2009年7月29日就诊.体检:一般情况良好.神经系统检查示右上肢肌力Ⅳ级,肌肉轻度萎缩,右上肢及右小腿痛觉过敏,右侧霍夫曼征阳性,右侧腱反射亢进. MRI表现:颈髓明显增粗,T1WT以等信号为主,夹杂小片状低信号,境界模糊,蛛网膜下腔明显变窄,局部闭塞;T2WI呈混杂信号影,内见多发片状及结节样高信号,注射Gd-DTPA后病灶明显不均匀强化。

Central neurocytoma in the fourth ventricle:a case report

患者男,27岁,于5个月前无明显诱因出现呕吐,3～4次/日,呕吐物为胃内容物,非喷射性,伴有头晕,时有轻微头胀痛感,按胃炎治疗无明显改善.1个月前出现行走不稳,足间距增宽,并渐出现记忆力下降、健忘,无肢体麻木、无力及口角歪斜等症状.体检:神志清楚,双眼视乳头水肿,站立不稳,闭目难立征阳性,余神经体征检查均阴性。

Radiation therapy for residual neurocytoma

Objective To investigate the role of radiation therapy in the treatment of postoperative residual or recurrent central neurocytoma. Methods The outcome of radiation therapy for 6 patients with residual neurocytom was analyzed and the related literatures were reviewed. Results In five patients treated by radiotherapy, with the dose ranged from 55 to 60 Gy and the follow-up period from 24 to 36 months, the tumor disappearance was found in 1 patient and reduction in tumor size in 4 cases. One patient experienced delayed radiative-necrosis in the parenchyma of thalamus adjacent to the tumor covered by the field of radiation therapy. One patient was treated by Gamma Knife surgery with the prescribed dose of 15 Gy. Eighteen months later, the tumor had decreased in size and the patient remained neurologically intact. Conclusion It is suggested that the central neurocytoma is sensitive to radiation and the radiation therapy appears to have an effect on tumor control. A radiation dose of 54 Gy appeared sufficient. However, it should be carefully decided whether to use radiation therapy for a residual tumor, taking into account the benign biologic behavior and potential radiation toxicity. Gamma knife surgery may be an effective and safe treatment choice for patient. Key words: Central neurocytoma; Radiotherapy; Stereotactic radiosurgery; Prognosis

Single-port endoscopic removal of intraventricular hemorrhage or tumor of the brain: A technical note

Background/Purpose. Although endoscopic surgery is a minimally invasive procedure, it is a relatively inefficient method for removing intraventricular hemorrhage (IVH) and intraventricular tumors. To improve the efficiency of endoscopic surgery, we used either a polypropylene tube or a 3-mL syringe tube combined with a working channel endoscope to remove IVH and an intraventricular tumor. Methods. From January 2005 to October 2005, four patients with IVH and one patient with intraventricular tumor were treated by endoscopic surgery in our hospital. During surgery, we experimented with two different endoscopic sheaths to approach the intra ventricular lesions. The indications for surgery in patients with IVH were consciousness disturbance with acute hydrocephalus and a hematoma volume＞20 mL. Results. In the patient with neurocytoma, the tumor was totally resected. In the four patients with IVH, the preoperative Graeb scores ranged from 8 to 12 (median 10 ) and the postoperative Graeb scores ranged from 3 to 5 (median 4), representing a reduction of 67%. The preoperative IVH ventriculocranial ratio ranged from 0.35 to 0.43 and the postoperative ventriculocranial ratio ranged from 0.14 to 0.29, representing a reduction of 36%. At 6-month follow-up, the mortality rate was 25%; none of the patients showed evidence of shunt-dependent hydrocephalus. The mean Glascow outcome scale at 6-month follow-up was 3.5±1.9. Conclusion. A working channel endoscope combined with either a polypropylene endoscopic sheath or a 3-mL syringe endoscopic sheath facilitates the removal of IVH and intraventricular tumors in endoscopic surgery.

Intraventricular neurocytoma: a clinicopathological study of 20 cases with review of the literature.

The clinicopathological features of 20 cases of central neurocytomas are described. They accounted for 0.28% of all intracranial tumours diagnosed during a 16 year period (1980-1995). Lower mean age of the patients at diagnosis (23.1 years), male preponderance (M:F=1.8:1) and higher incidence of involvement of the right lateral ventricle (10/20 cases) were noted in this series, in contrast to reports from Western literature. Total removal of the tumour was done in 14 cases while the remaining six underwent partial resection. Morphogically, the tumours had a striking resemblance to oligodendrogliomas (11/20 had been earlier diagnosed as oligodendrogliomas) and an interesting finding was the presence of dilated vascular channels in 12/20 tumours. The diagnosis was confirmed in all cases by immunohistochemistry and/or electron microscopy. While 18 cases were histologically benign, two had features of atypical neurocytoma. Five patients died due to postoperative complications. The remaining patients received postoperative radiation and their follow-up revealed that all of them were doing well at 12 to 72 months after surgery. These neoplasms should be suspected in any young patient with radiological evidence of an intraventricular lesion; for their differentiation from gliomas, immunohistochemistry and electron microscopy should be done. This is important because, unlike gliomas, these tumours have a relatively favourable prognosis and their current treatment of choice is complete surgical removal without adjuvant chemo- or radiotherapy. Copyright 1999 Harcourt Publishers Ltd.