Supratentorial high-grade astrocytoma with leptomeningeal spread to the fourth ventricle: a lethal dissemination with dismal prognosis.

PURPOSE: Leptomeningeal spread to the fourth ventricle (LSFV) from supratentorial high-grade astrocytoma (HGA) is rarely investigated. The incidence and prognostic merit of LSFV were analyzed in this study. METHODS: A consecutive cohort of 175 patients with pathologically diagnosed HGA according to the 2016 WHO classification of brain tumors was enrolled. LSFV was defined as radiological occupation in the fourth ventricle at the moment of initial progression. Clinical, radiological, and pathological data were analyzed to explore the difference between HGA patients with and without LSFV. RESULTS: There were 18 of 175 (10.3%) HGAs confirmed with LSFV. The difference of survival rate between patients with LSFV or not was significant in both overall survival (OS) (14.5 vs. 24 months, P = 0.0007) and post progression survival (PPS) (6.0 vs. 11.5 months, P = 0.0004), while no significant difference was observed in time to progression (TTP) (8.5 months vs. 9.5 months P = 0.6795). In the Cox multivariate analysis, LSFV was confirmed as an independent prognostic risk factor for OS (HR 2.06, P = 0.010). LSFV was correlated with younger age (P = 0.044), ventricle infringement of primary tumor (P < 0.001) and higher Ki-67 index (P = 0.013) in further analysis, and the latter two have been validated in the Logistic regression analysis (OR 18.16, P = 0.006; OR 4.04, P = 0.012, respectively). CONCLUSION: LSFV was indicative of end-stage for supratentorial HGA patients, which shortened patients' PPS and OS instead of TTP. It's never too cautious to alert this lethal event when tumor harbored ventricle infringement and higher Ki-67 index in routine clinical course.

Epidemiología y caracterización general de los tumores cerebrales primarios en el adulto / Epidemiology and General Characterization of Primary Brain Tumors in Adults

Los tumores primarios del sistema nervioso representaron aproximadamente el 1,4% de los nuevos diagnósticos de cáncer en 2015 y causan el 2,6% de las muertes por cáncer. Estos tienen una mayor implicación clínica en la población infantil y en adultos jóvenes; y su incidencia disminuye con la edad. Los tumores más frecuentes en los adultos incluyen meningiomas, gliomas y tumores pituitarios. En este artículo se hace una revisión actualizada sobre la epidemiología de los tumores primarios del sistema nervioso, así como las principales características y actualizaciones en el manejo de los tumores más prevalentes en la población adulta.

Primará' nervous system tumors represented approximately 1,4% of new cáncer diagnoses in 2015 and cause 2,6% of deaths secondary' to cáncer. Their major clinical impact is seen in pediatric population and young adults; and their incidence decreases with age. The most frequent tumors in adults inelude meningiomas, gliomas and pituitary tumors. In this arricie, an updated review is made on the epidemiology of primar? tumors of the nervous system, as well as the main features and updates in the management of most prevalent tumors in the adult population.

Anorexia: an early sign of fourth ventricle astrocytoma in children.

INTRODUCTION: Paediatric low-grade astrocytomas of the fourth ventricle are rare tumours, generally revealed by hydrocephalus. However, some patients present with a history of severe anorexia. It might be a harbinger, which if recognized, could lead to earlier diagnosis. We decided to examine our database in order to evaluate the incidence and signification of anorexia in this context. METHODS: Retrospective monocentric study of cases of low-grade astrocytomas of the fourth ventricle operated between 1991 and 2012 in our paediatric neurosurgery department. We particularly observed the clinical presentation and long-term clinical, oncological and radiological evolution. Non-parametrical tests were used (Mann-Whitney, Fisher). RESULTS: We reviewed 34 cases, 31 pilocytic astrocytomas and 3 diffuse astrocytomas, 16 boys and 18 girls, (M/F ratio 0.89). Mean age at diagnosis was 8 years old. Seven presented with notable anorexia, the average BMI in this group was ≤2 standard deviation (SD); with clinical signs evolving for 11.5 months. Twenty-seven children had no anorexia; average BMI in this group was +1 SD, with clinical evolution for 6 months on an average of p < 0.05. We found no significant difference regarding hydrocephalus or tumour location. In all children with anorexia, body mass index improved markedly in the postoperative follow-up, which lasted, on average, for 6 years. CONCLUSION: Anorexia with stunted body weight curve is a non-exceptional presentation in children with low-grade astrocytomas of the fourth ventricle. Unexplained or atypical anorexia with negative etiologic assessment should prompt cerebral imaging. Clinical improvement after surgical resection, could suggest a possible interaction between tumour tissue and appetite-suppressing peptide secretion.

Symptomatic subependymomas of the ventricles. Review of twenty consecutive cases.

BACKGROUND AND PURPOSE: Intraventricular subependymomas are rare benign tumors, which are often misdiagnosed as ependymomas. To review the clinicopathological features of subependymomas. PATIENT SELECTION AND METHODS: Retrospective clinical analysis of intraventricular subependymomas and systematic review of histological slides operated on at our center between 1985 and 2005. RESULTS: Twenty subependymomas presented at the median age of 50 years (range 19-77). Two (10%) were found in the third, three (15%) in the forth, and 15 in the lateral ventricles. There was male preponderance (12 vs. 8). Ataxia (n=13) and papilledema (n=7) were the most common clinical presentations. Fifteen patients underwent gross total resection, and five had subtotal resection. None of the cases showed mitotic figures, vascular endothelial proliferation or necrosis. Cell proliferation marker MIB-1 activity (percentage of positive staining tumor cells) ranged from 0 to 1.4% (mean 0.3). Two cases were treated with preoperative radiation therapy (50 Gy) before the CT era, three other patients received postoperative radiation therapy for tumors originally diagnosed histologically as low grade ependymomas. Three patients (15%) died of surgical complication between one and three months postoperatively, and three patients died of unrelated causes in eight, 26 and 110 months. Fifteen patients were alive without evidence of tumor recurrence at a median follow-up time of 10 years. CONCLUSION: Subependymomas are low-grade lesions and patients do well without adjuvant radiotherapy. Small samples from more cellular areas may be confused with low grade ependymomas, and unnecessary radiotherapy may follow. Recurrences, rapid growth rates should warrant histological review, as hypocellular areas of ependymomas may also be a source of confusion.

Subependymal nodules and giant cell tumours in tuberous sclerosis complex patients: prevalence on MRI in relation to gene mutation.

PURPOSE: The purpose of this study was to estimate the association among the presence of subependymal nodules (SENs), subependymal giant cell tumours (SGCTs) and gene mutation in tuberous sclerosis complex (TSC) patients. METHODS: Clinical records and images of 81 TSC patients were retrospectively reviewed by two neuroradiologists in consensus. All patients were assessed for gene mutations and were categorized as TSC1 or TSC2 mutation carriers, or no-mutations-identified (NMI) patients. They underwent a brain magnetic resonance imaging (MRI) using 0.1 mmol/kg of gadobutrol. Any enhancing SEN ≥ 1 cm and placed near the foramen of Monro was considered SGCT. Two MRI follow-up exams for each patient with SGCT were evaluated to assess tumour growth using Wilcoxon and chi-squared tests. RESULTS: Of 81 patients, 44 (54%) were TSC2 mutation carriers, 20 (25%) TSC1 and 17 (21%) NMI. Nine (11%) had a unilateral and three (4%) a bilateral SGCT. Fifty of 81 patients (62%) showed at least one SEN. None of the 31 patients without SEN showed SGCTs, whilst 12 (24%) of the 50 patients with at least one SEN showed SGCTs (p = 0.003). The association between the presence of SGCT or SEN and gene mutation was not significant (p = 0.251 and p = 0.187, respectively). At follow-up, the median SGCT diameter increased from 14 to 15 mm (p = 0.017), whilst the median SGCT volume increased from 589 to 791 mm(3) (p = 0.006). CONCLUSIONS: TSC patients with SENs are more likely to present with SGCT than those without SENs, in particular for TSC2 mutation carriers. The SGCT growth rate may be missed if based on the diameter instead of on the volume.

Endoscopic treatment of intraventricular cystic tumors.

OBJECTIVE: Intraventricular cystic tumors constitute a surgical challenge, because of their deep location and the histologically benign nature of most of them. We aim to present concisely, yet comprehensively, the role of neuroendoscopy in the treatment of intraventricular cystic tumors. METHODS: A literature review searching for applications of endoscopy in the treatment of intraventricular cystic tumors is presented. Our experience is added to the presented data. In controversial issues, a comparison is made with traditional treatment methods. RESULTS: Intraventricular endoscopy has been successfully used in the treatment of the whole range of intraventricular cystic tumors. The most common indication is the treatment of colloid cysts. In the treatment of colloid cysts, a comparison with microsurgical techniques showed that endoscopy is advantageous in regard to operative morbidity and postoperative shunt dependency but is associated with a slightly higher recurrence rate. CONCLUSION: Intraventricular endoscopy has emerged as a viable option in the treatment of intraventricular cystic tumors.

[Central neurocytomas of the lateral ventricle. A series of 35 cases with review of the literature]. / Les neurocytomes centraux du ventricule latéral. Analyse d'une série de 35 cas et revue de la littérature.

Central neurocytoma is the second most frequent tumor in our series of 284 lateral ventricle tumors (12%). Mean age was 32.6 years. There was a male predominance (20/35). Most frequent clinical signs were neuropsychological disturbances and intracranial hypertension. On imaging, these tumors concentrate central calcifications in more than half of cases. Main locations were frontal horn and corpus of lateral ventricle (82%). Uneventful postoperative course was recorded in 52% of cases. Immunoreactivity against neuron-specific enolase (NSE), synaptophysin, S100 protein, Microtubul Associated Protein de type 2 (MAP2) and calcineurin confirms the neuronal nature of the neoplasm. This tumor has a good prognosis. Local control after surgery is obtained in 68% of cases. Gamma knife surgery seems to be useful in cases of little volume with tumoral remnants or recurrence, when radiological appearance is well circumscribed and round-shaped.

[Tumors of the lateral ventricle. Review of 284 cases]. / Tumeurs du ventricule latéral. Analyse d'une série de 284 cas.

UNLABELLED: The data from 284 cases of lateral ventricle (VL) tumors treated in 18 departments of Neurosurgery in France between 1995 and 2010 were collected in this series. The mean age was 33 years with a large proportion of children (79/284). CLINICAL PRESENTATION: The mean duration of symptoms was short in time (2 months). The revelation was incidental in 10% of the cases; for symptomatic lesions, raised intracranial pressure (ICP, due to hydrocephalus) and neuropsychological signs were the most frequent. Only one third of the patients had no CSF disorder. HISTOPATHOLOGY: The two most frequent tumors were meningiomas (40 cases, 15%) and neurocytomas (35 cases, 13%). The sub-ependymal giant cells astrocytomas, the ependymomas and sub-ependymomas were also common (11, 10 and 9% respectively). THERAPEUTIC MANAGEMENT: A surgical procedure was performed in 258 patients, with the help of neuronavigation in one third of cases. The patients were most frequently operated with a transfrontal approach (40%). The resection was complete in 73%, which was a statistical factor of global survival. The postoperative mortality rate was 4.9%. OUTCOME: Neuropsychological deficits were the most frequent sequelae (22.8%) due to raised ICP. Campimetric deficits (19%), seizures (19%), hemiparesis (15%) and language disorders (12%) were then found. The mean follow-up was 52 months with good evolution for 58% of patients. Fifteen percent of patients had a handicap and, finally, 12.9% will die. At last, two third of living patients had no tumoral residue or re-evolution.

Nationwide investigation of the current status of therapeutic neuroendoscopy for ventricular and paraventricular tumors in Japan.

OBJECT: The authors report their investigation on the current status of neuroendoscopic biopsy for ventricular and paraventricular tumors as well as treatment for associated hydrocephalus in Japan. METHODS: Patients who had undergone therapeutic neuroendoscopy between 2005 and 2009 were included in this study. The main items examined were age; sex; localization of tumor; pathological diagnosis using biopsy; the presence, treatment, and efficacy of treatment of associated hydrocephalus; perioperative complications; activities of daily living (ADL) before and after therapeutic neuroendoscopy; and the presence of dissemination during the postoperative course. RESULTS: Seven hundred fourteen patients from 123 sites (462 male and 252 female patients, mean age 33.3 years) were enrolled. Localization of the tumor was mainly classified into the lateral ventricle in 91 patients, the third ventricle in 339, the fourth ventricle in 18, the suprasellar region in 75, and other paraventricular areas in 191 patients. The most commonly observed tumors were germ cell tumors in the third ventricle (177 cases [39%]), cystic lesions in the suprasellar region (56 cases [75%]), and astrocytic tumors in the thalamus-basal ganglia (71 cases [38%]). Although 641 (92.8%) of 691 patients could receive neuroendoscopic diagnosis using biopsy, the diagnosis obtained with endoscopic biopsy differed from the final diagnosis based on subsequent craniotomy in 18 patients and clinical course in 3 patients. Of these 21 patients, 7 had astrocytic tumors, 4 had pineal tumors, 6 had germ cell tumors, and 4 had other tumors. The final diagnostic accuracy rate was 89.7%. Associated hydrocephalus was observed in 517 patients (72.4%), of whom 316 and 39 underwent third ventriculostomy and fenestration of the septum, respectively. The response rates were 96.2% and 89.7%, respectively. Third ventriculostomy was required for recurrence of hydrocephalus in 41 patients (13.0%), and the long-term response rate was therefore 83.2% (263 of 316 patients). Perioperative complications other than fever, such as new onset of or progressive hydrocephalus, infection due to CSF leakage, and bleeding in the ventricle or tumor, were found in 81 patients (11.3%). The median Karnofsky Performance Scale score before endoscopic surgery was 80, but it increased to 90 after surgery. The score was thus significantly increased after surgery (p < 0.0001, Mann-Whitney U-test). Activities of daily living after surgery decreased due to perioperative complications in 15 patients (2.1%). The incidence of new dissemination after endoscopic biopsy was 6.8% and not high compared with routine surgical treatment. CONCLUSIONS: The authors concluded that neuroendoscopic diagnosis using biopsy for ventricular and paraventricular tumors is adequately accurate and safe. It was demonstrated that endoscopic procedures play important roles not only in the treatment of hydrocephalus associated with intra- and paraventricular tumors but also in significantly improving ADL. Furthermore, the long-term outcome of endoscopic third ventriculostomy was clearly favorable.

Ring-shaped lateral ventricular nodules: an incidental finding on brain magnetic resonance imaging.

INTRODUCTION: We have occasionally seen ring-shaped lateral ventricular nodules <1 cm in diameter during routine brain magnetic resonance imaging (MRI). We investigated retrospectively clinical and MRI findings of the nodules. MATERIALS AND METHODS: Review of radiological records was performed for 39,607 patients who underwent brain MRI between January 2001 and April 2008. Nodules were assessed for number, location, shape, and signal intensity, which was determined based on the range of signal intensity from gray to white matter on T1- and T2-weighted imaging. Fluid-attenuated inversion recovery (FLAIR) and diffusion-weighted imaging (DWI), contrast enhancement characteristics, and serial MRI changes of nodules were assessed when available. RESULTS: Nine of 39,607 patients (0.023%) showed the nodules. No symptoms associated with the nodules. Among the nine patients, 11 nodules were identified (one nodule in seven patients (77.8%) and two nodules in two patients (22.2%)). Location was limited to the roof of the body for six nodules (54.5%) and the frontal horn for five patients (45.5%). All nodules (100%) were round and isointense on T1- and T2-weighted imaging. On FLAIR imaging of eight nodules, six (75%) were hyperintense, and two (25%) were isointense. On DWI of seven nodules, all nodules (100%) were isointense. None of seven nodules (0%) examined using postcontrast MRI showed enhancement. None of eight nodules (0%) examined using serial MRI (range, 4-60 months) showed changes in morphology over time. CONCLUSION: These nodules were incidentally encountered and shared similar MRI features. Although pathological confirmation was lacking in our cases, these nodules may be of nonaggressive nature.

Parenchymal neurocutaneous melanosis in association with intraventricular dermoid and Dandy-Walker variant: a case report.

Neurocutaneous melanosis (NCM) is a rare congenital disease that is characterized by the presence of large or multiple congenital melanocytic nevi and melanotic lesions of the central nervous system. We report here on the CT and MR imaging findings of an unusual case of NCM that was associated with intraventricular dermoid and Dandy-Walker malformation.

Parenchymal Neurocutaneous Melanosis in Association with Intraventricular Dermoid and Dandy-Walker Variant: A Case Report

Neurocutaneous melanosis (NCM) is a rare congenital disease that is characterized by the presence of large or multiple congenital melanocytic nevi and melanotic lesions of the central nervous system. We report here on the CT and MR imaging findings of an unusual case of NCM that was associated with intraventricular dermoid and Dandy-Walker malformation.

Cns tumors at AKU: an update plus a brief discussion on intraventricular tumors with special emphasis on central neurocytoma.

BACKGROUND: This paper is intended to be an update of our earlier published work on CNS tumors along with additional information on intraventricular tumors. Three cases of central neurocytoma are also discussed. METHODS: An analysis conducted in the Section of Histopathology, AKU to determine the frequency of all CNS neoplasms diagnosed between Jan 1, 1994 and Dec 31, 2001. Frequency of intraventricular tumors was also determined. Histological characterization of the tumors was based on the WHO and AFIP (USA) classification systems for CNS tumors. RESULTS: A total of 1677 CNS tumors were diagnosed during the study period. 1510(90.04%) were primary, and 167(9.96%) were metastatic. Among the primary tumors, gliomas constituted the largest category with 825 cases or 54.63% of all primary tumors. Meningothelial tumors comprised the second largest group with 364 cases or 24.10%. among other major categories, embayonal tumors, and peripheral nerve sheath tumors comprised 6.75% and 6.82% of all primary tumors. Among less common tumors, Non-Hodgkin's lymphomas and hemangioblastomas comprised 3.11% and 1.52% of all CNS tumors. Intraventricular tumors comprised 7.41% of all primary tumors. Ependymomas comprised 64.28% of all Intraventricular tumors. CONCLUSIONS: Gliomas and meningothelial tumors are the commonest group of primary CNS tumors. Metastatic tumors are quite common.

Epidemiology and pathology of intraventricular tumors.

Tumors that primarily or exclusively involve the ventricular system constitute a rare and heterogeneous group. Certain histologic tumor types predominantly occur in children, whereas others are more common in adults. Tumor location provides additional clues to correct diagnosis. When used in conjunction with clinical and radiologic data, histopathologic features can distinguish among this wide range of possibilities to provide the correct diagnosis for optimal patient management.

Intraventricular gliomas.

Gliomas are the most common primary brain tumor in adults, and those within or relating to the ventricular surface represent a less common but important subcategory. The most common intraventricular gliomas include ependymomas, SEs, and SEGAs. Other less common varieties have been reported, including chordoid gliomas, glioblastoma multiforme, and mixed glial-neuronal tumors. Each type of intraventricular glioma is associated with its own unique constellation of epidemiologic, clinical, radiologic, and pathologic defining characteristics. Each tumor type has its own management considerations and nuances with unique prognostic indicators and outcomes. The outcome for certain intraventricular gliomas (especially ependymomas) remains relatively poor. Future advancements in surgical technique are likely to have only a modest impact on improvement of outcome. Translational research aiming to advance the knowledge of tumor biology into new targeted cellular and molecular therapies holds tremendous promise to improve the overall outcome. Additionally, more thorough delineation of prognostic factors as well as modifications and refinements to radiation and chemotherapy may help to improve the still significantly poor outcomes for patients harboring these lesions. Future cooperative intra- and interinstitutional efforts between scientists and clinicians will hopefully culminate in an improved outlook and eventual cure for patients with gliomas.

[Choroid plexus tumors]. / Tumores de plexos coroideos.

INTRODUCTION AND OBJECTIVE: Tumors of the choroid plexus are rare tumors of neuro ectodermal origin, accounting for less 1% of intracranial tumors in all ages. Most cases present in children less than 2 years of age. These tumors have been classified according to histopathological criteria into papilloma and carcinoma. DEVELOPMENT: We review the epidemiological, clinical, neuropathological details, neuroradiological aspects and treatment of choroid plexus tumors. CONCLUSIONS: Choroid plexus tumors may present with overt intracranial hypertension with or without focal neurological signs. In the adult population, headaches are the most commonly encountered symptom. The CT characteristics of CPT are well characterized. On non enhanced studies the tumor appears as a smooth or lobulated mass, hyperdense in relation to surrounding brain parenchyma. With intravenous contrast, there is marked, homogeneous enhancement. With MRI these tumors showed an iso intensity in T1 weighted images and iso hypo intensity in T2 weighted images, with marked enhancement after gadolinium. The treatment of choice is total surgical excision with minimal damage to the surrounding neural elements. For carcinomas, adjuvant treatment in the form of chemotherapy supplemented by radiation therapy in older children.

[Tumors of the third ventricle: review of the literature]. / Tumeurs du troisième ventricule: revue de la littérature.

In this chapter, the authors compared the results of the national series (262 patients) with the relevant data from the literature, in order to establish the optimal therapeutic management. Colloid cysts represent the main tumor encountered within the third ventricle (145 cases). Sudden death from colloid cysts is documented in this series, but neurosurgeons should also be aware of the increasing number of fortuitous diagnosis of such lesions. The liquid consistency of the content of the cyst is the major argument to choose the surgical procedure. Liquid colloid cysts usually associate isodense appearance on CT Scan, hypo-intensity on T1 weighted MRI images, and hyperintensity on T2-weighted MRI images. Endoscopy now appears as an accurate surgical procedure. A surgical strategy is proposed. Among glial tumors, pilocytic astrocytomas are poorly documented in the literature. Low grade astrocytomas, ependymomas, subependymomas and giant cell subependymal astrocytomas, gangliogliomas, and oligodendrogliomas were reviewed with the relevant literature. Other tumors are rare in the third ventricle, except for choroid plexus papilloma and craniopharyngioma. The reality of purely intraventricular craniopharyngioma is confirmed by the experience of this series. Other lesions such as meningioma, cavernoma, germ-cell tumors, lymphomas, metastasis, epidermoid cysts, and exceptionnal lesions were also reviewed.

Intraventricular neurocytoma.

Intraventricular neurocytoma is a rare cerebral tumour which is usually associated with a good prognosis. It has imaging features which help differentiate it from other cerebral tumours. We report three cases including the first to originate from the pineal gland. The report emphasizes the radiological appearance and reviews the literature.

Intraventricular supratentorial tumors in children.

Overall, intraventricular supratentorial tumors are rare in childhood. Classification can be based on the separation of lesions originating in intraventricular structures, such as choroid plexuses, from glial neoplasms of the ventricular wall which tend to infiltrate the ventricular cavities. Aim of the present study is to review the most common neoplasms of this region in childhood. Choroid plexus tumors (papillomas and carcinomas) and subependymal giant cell astrocytomas are dealt with, while for the other neoplasms which are rarer or more typic of other age ranges, specific reports should be consulted. Choroid plexus papillomas affect infants and are the most frequent oncological type among congenital tumors. The malignant variant (grade III-IV) is represented by the less frequent the neoplasms. The malignant variant (grade III-IV) is represented by the less frequent choroid plexus carcinoma which is markedly invasive with respect to adjacent nervous structures and has a high tendency to form metastases even at onset. Anaplastic papillomas are intermediate forms whose correct histopathology is still debated. Most frequent glial tumors are subependymal giant-cell astrocytomas. They are benign tumors (grade I) typically albeit not constantly associated to tuberous sclerosis. In this case the differentiation from subependymal nodules plays a major role. Contrast enhanced CT is fundamental in this assessment. Anaplastic variants, though rare, are well-known.