EANO - EURACAN - SNO Guidelines on circumscribed astrocytic gliomas, glioneuronal, and neuronal tumors.

In the new WHO 2021 Classification of CNS Tumors the chapter "Circumscribed astrocytic gliomas, glioneuronal and neuronal tumors" encompasses several different rare tumor entities, which occur more frequently in children, adolescents, and young adults. The Task Force has reviewed the evidence of diagnostic and therapeutic interventions, which is low particularly for adult patients, and draw recommendations accordingly. Tumor diagnosis, based on WHO 2021, is primarily performed using conventional histological techniques; however, a molecular workup is important for differential diagnosis, in particular, DNA methylation profiling for the definitive classification of histologically unresolved cases. Molecular factors are increasing of prognostic and predictive importance. MRI finding are non-specific, but for some tumors are characteristic and suggestive. Gross total resection, when feasible, is the most important treatment in terms of prolonging survival and achieving long-term seizure control. Conformal radiotherapy should be considered in grade 3 and incompletely resected grade 2 tumors. In recurrent tumors reoperation and radiotherapy, including stereotactic radiotherapy, can be useful. Targeted therapies may be used in selected patients: BRAF and MEK inhibitors in pilocytic astrocytomas, pleomorphic xanthoastrocytomas, and gangliogliomas when BRAF altered, and mTOR inhibitor everolimus in subependymal giant cells astrocytomas. Sequencing to identify molecular targets is advocated for diagnostic clarification and to direct potential targeted therapies.

Dissemination Patterns and Short-Term Management of Multifocal Rosette-Forming Glioneuronal Tumors.

BACKGROUND: Multifocal rosette-forming glioneuronal tumors (RGNTs) are challenging to manage. Gross total resection is often impossible, and data on adjunctive therapies are limited. We reviewed cases of multifocal RGNTs in the literature with special focus on dissemination patterns and management. METHODS: A literature review was conducted using PubMed and the key words "(multifocal OR multicentric OR satellite OR dissemination) AND glioneuronal." RESULTS: There were 21 cases of multifocal RGNTs identified. Follow-up was available in 18 cases at a median of 17 months. Progression-free survival and overall survival at 1 year were 84% and 94%, respectively. Of all cases, 43% had cerebrospinal fluid (CSF) dissemination, 48% had intraparenchymal spread, and 10% had both. The presence of CSF dissemination led to palliative care and/or death in 20% of cases (n = 2). None of the cases with intraparenchymal spread progressed. Radiotherapy was used in 50% of cases with CSF dissemination, chemotherapy was used in 20%, and CSF shunting was used in 36%. No tumors with intraparenchymal spread required adjunctive therapy or shunting. CONCLUSIONS: RGNTs with CSF dissemination are more likely to behave aggressively, and early adjunctive therapies should be discussed with patients. Tumors with intraparenchymal spread grow slowly, and maximal safe resection followed by observation is likely sufficient in the short term. Long-term behavior of multifocal RGNTs is still unclear.

Intramedullary spinal cord ganglioglioma: Case report and comparative literature review. / Ganglioglioma espinal intramedular: caso clínico y revisión comparativa de la literatura.

INTRODUCTION: Intramedullary spinal cord gangliogliomas (ISCGGs) account for 35-40% of all intramedullary tumors in children. These tumors have a different algorithm for treatment and prognosis than other medullary tumors, such as astrocytomas and spinal ependymomas. The objective of the study was to review the literature and examine an approach to diagnosing and treating this tumor based on a case report of ISCGG diagnosed at our center. METHOD: An exhaustive review of cases of ISCGG published via the PubMed-NCBI platform between 1911 and 2018 was performed, and each patient's epidemiological characteristics, tumor location, symptoms, radiological diagnosis and treatment were appended. RESULTS: A total of 167 cases of ISCGG were found, including our own. In our sample, 52% of patients were female and the most commonly affected age group was 0-9 years of age (35% of patients). Motor deficit has been found to be the main symptom in a larger proportion of adults patients versus pediatric patients. On X-ray, this tumor shows greater hyperintensity and contrast uptake than astrocytomas and ependymomas, as well as a higher percentage of intratumoral cysts. The BRAFV600E mutation is less common in spinal as opposed to supratentorial gangliogliomas. Surgery with complete resection is the treatment of choice. Only 19% of the patients in the sample received radiotherapy, and only 9% received chemotherapy as their only line of treatment. CONCLUSIONS: ISCGGs are common in the pediatric population and require strong suspicion for proper diagnosis and treatment, as the risk of recurrence of ISCGGs is 3 times greater than that of supratentorial gangliogliomas.

Multifocal Intracranial Ganglioglioma in a Sexagenarian: Case Report and Review of the Current Literature.

BACKGROUND: Gangliogliomas are rare, well-differentiated, low-grade neoplasms that most often occur unifocally in children and most commonly affect the temporal lobe. Gangliogliomas that occur in patients age >40 years tend to have worse prognoses. These tumors generally stain positively for neural and glial cell markers, as well as CD34. Here we report an unprecedented case of multifocal intracranial ganglioglioma in an adult age >40 who had a favorable course, and review the current literature on multifocal intracranial gangliogliomas. CASE DESCRIPTION: A 60-year-old female presented to her ophthalmologist with blurry vision in the right eye and an unremarkable neurologic exam. She was referred for brain imaging, which showed multiple lesions in both cerebral hemispheres. Biopsy of the right occipital lesion was elected, as it enhanced the most on magnetic resonance imaging. CONCLUSIONS: Multifocal intracranial gangliogliomas are exceedingly rare tumors, especially in adults. These tumors present unique management barriers because as they are multifocal at the time of diagnosis, making resection more technically challenging. In our review, the average age at diagnosis was 19.2 years, and 80% of the cases had at least 1 lesion in the temporal lobe. Two studies opted for resection of intracranial tumors, whereas the remaining studies performed biopsy with conservative management and serial imaging. Biopsy was performed in all cases. We present the first case of an intracranial multifocal ganglioglioma in a patient age >40 years with lesions in the occipital lobe, corpus callosum, and frontal lobe at presentation.

Spinal Intramedullary Ganglioglioma in Children: An Unusual Location of a Common Pediatric Tumor.

Ganglioglioma is a common CNS tumor in children, mostly found in the temporal lobe, causing epilepsy. Spinal gangliogliomas are very rare, accounting for 1.1% of all intramedullary spinal tumors. The management principles and the need for adjuvant therapy are not yet well defined in this cohort. BRAF V600E mutation in spinal ganglioglioma has been described in a few series recently. In this report, we describe 3 children with spinal ganglioglioma at different locations, and their expression of BRAF V600E mutation and follow-up. In addition, we review the recent literature on pediatric spinal ganglioglioma management.

Pediatric primary anaplastic ganglioglioma with malignant neuronal component.

Kaymak-Cihan M, Erdis E, Bozkurt S, Ünver-Korgali E. Pediatric primary anaplastic ganglioglioma with malignant neuronal component. Turk J Pediatr 2018; 60: 102-106. Gangliogliomas (GGs) represent approximately 0.4%-1.0% of all brain tumors. Anaplastic gangliogliomas (AGGs) form 5-10% of all GGs. They are a mixed neuronal-glial tumor of central nervous system and composed by two cell lines; neuronal (ganglionic) and glial cells. Anaplastic component of AGGs is usually glial cells. Malignant neuronal component is a rare condition. Here we report an 8-year-old male patient who was diagnosed with primary AGG with malignant neuronal component and was treated with surgery, adjuvant radiotherapy and chemotherapy.

Pediatric Intracranial Anaplastic Gangliogliomas: Illustrative Case and Systematic Review.

OBJECTIVE: We present an illustrative case of pediatric intracranial anaplastic ganglioglioma and systematically reviewed the current reported data of anaplastic ganglioglioma in the pediatric population. METHODS: A comprehensive literature search for our review was conducted using PubMed, Scopus, Web of Science, PsycINFO, Cochrane, and Embase databases. The search terms included "ganglioglioma," "anaplastic," "pediatrics," "children," and "intracranial." We used no limitations for date, type, or language. Reports of pediatric patients (age <19 years) with intracranial anaplastic gangliogliomas were included. Baseline patient demographic characteristics, clinical presentations, imaging characteristics, management strategies, and outcomes data were extracted. RESULTS: We included 24 studies involving 34 patients in the quantitative synthesis. The mean patient age was 9.18 ± 5.46 years (range, 0.16-18). A male predominance was observed (approximate male/female ratio, 1.61:1). Increased intracranial pressure was the most common symptom (n = 19; 55%), followed by seizures (n = 11; 32%). These tumors were most often found in the supratentorial compartment (n = 26; 76%). All 34 patients had undergone surgical removal. The mean follow-up was 22.2 months (range, 2-72). The mean overall survival duration was 43 months (95% confidence interval, 32.45%-55.31%; 1- and 3-year overall survival, 76.6% and 45.5%, respectively). The median event-free survival was 34 months (95% confidence interval, 10.6%-57.3%; 1- and 3-year event-free survival rate, 76.6% and 42.4%, respectively). CONCLUSIONS: Our results contribute to our understanding of the characteristics of this rare malignant tumor and show that anaplastic ganglioglioma should be considered in the differential diagnosis of intracranial tumors in pediatric patients.

Ganglioglioma in children and young adults: single institution experience and review of the literature.

BACKGROUND: Ganglioglioma (GG) is a rare mixed glial-neuronal neoplasm accounting for 0.5-5% of all pediatric central nervous system (CNS) tumors. Rarity of this tumor has precluded defining robust treatment guidelines. This retrospective study evaluates the prognostic factors and outcomes of this rare neoplasm. PATIENTS AND METHODS: Retrospective analysis of 55 patients with GG was conducted to describe clinical findings, and outcomes. Kaplan-Meier survival and Cox-regression analyses were performed to assess the overall survival (OS) and progression-free survival (PFS). RESULTS: The mean age at diagnosis was 11.8 years (range 1-21 years) with a median follow-up period of 9.5 years. 53 patients (92.7%) had low grade GG and 2 patients had anaplastic GG. 25 patients had tumor progression, whose median PFS was 12 years. Six patients with low grade GG progressed to a higher grade, with median survival of 9.1 month after transformation. The 5 and 10 year PFS were 65 and 57%, respectively. The 5 and 10 year OS was 96 and 86% respectively. 8 of the 19 (42%) samples tested demonstrated positivity for the BRAF V600E mutation. Multivariate Cox regression analyses showed location and extent of resection were significant factors for PFS and presence of metastatsis attained significance for OS. CONCLUSION: This is the one of the largest retrospective study of pediatric GG. Identifying clinical variables, which could stratify these tumors into low- and high-risk groups might help to profile a risk-based therapeutic strategy. Collaborative multiinstitutional prospective studies are warranted to delineate treatment consensus and investigate prognostic factors.

Edema of the optic tract in patients with tumors of the sellar region: clinical and visual implications in the pediatric population.

OBJECTIVE Tumor-related edema of the optic tract (EOT) corresponds to a preferential posterior distribution of peritumoral edema along the white matter tract of the visual system. To date, the consequences of EOT have never been evaluated specifically in the pediatric population. In this study, the authors attempted to identify clinical and radiological features associated with the development of EOT and the specific influence of this edema on visual function. METHODS A retrospective review was performed of data collected from patients younger than 18 years who underwent surgery for a tumor in the sellar region at the authors' institution between January 2005 and January 2016. Data were collected on patient characteristics, ophthalmological evaluations, and neuroimaging findings. To evaluate and compare visual function impairment, ophthalmological data were converted to a global visual function score, which took into account visual acuity, visual field evaluations, and laterality deficiencies. The visual acuity score was defined according to the International Classification of Diseases, 10th Revision. Visual field deficiencies were converted to a score of 0-2. Two opposing groups were then distinguished according to the presence or absence of EOT. Visual acuity, visual field results, and global scores were compared between groups before and after treatment. RESULTS Twenty-six patients were included in the study: 17 patients with craniopharyngioma, 3 patients with pilocytic astrocytoma, 2 patients with ganglioglioma, 2 patients with germ cell tumor, 1 patient with macroprolactinoma, and 1 patient with Rathke's cleft cyst. There were 11 children in the group with edema and 15 children in the group without edema. None of the following criteria were statistically different between the 2 groups: age, sex, clinical symptoms at presentation (endocrine deficiency or intracranial hypertension signs), incidence of hydrocephalus, compression of the optic tracts and mass effect on the optic chiasm, tumor size and localization, presence of intratumoral cysts, treatment, type of tumor, or recurrence. The median global visual function and visual acuity scores were not significantly different between the groups either at presentation or at final evaluation. The visual field score was lower (i.e., more deficiency) in the group with edema than in the group without edema (p < 0.05); 89% of the patients with edema had severe or mild visual field impairment versus only 40% of the patients without edema. At the final examination after treatment, the visual field scores were not different between the 2 groups. Although not significant, the number of patients with optic disc pallor was greater in the group without edema both at diagnosis and at final examination. CONCLUSIONS This study confirms that EOT in the context of sellar region tumor in children is not necessarily associated with a less-favorable visual prognosis.

Impact of surgery, adjuvant treatment, and other prognostic factors in the management of anaplastic ganglioglioma.

BACKGROUND/PURPOSE: Anaplastic ganglioglioma (AGG) is a rare tumor with both glial and neuronal component accounting for less than 1% of all CNS tumors with limited information about the optimum treatment and outcome of these tumors. METHOD AND MATERIALS: We did a thorough search of the PubMed with the following MesH terms: "Ganglioglioma; Anaplastic ganglioglioma; Ganglioglioma AND treatment; and Anaplastic ganglioglioma AND survival" to find all possible publications related to AGG to perform an individual patient data analysis and derive the survival outcome and optimum treatment of these tumors. RESULTS: A total of 56 articles were retrieved pertaining to AGG with 88 patients. However, a total of 40 publications found eligible with 69 patients for individual patient data analysis. Median age for the entire cohort was 16 years (range 0.2-77 years). Surgical details were available for 64 patients. A gross total or near total resection was reported in 21 cases (32.8%), subtotal resection or debulking was reported in 25 cases (39.1%). Surgical details were available for 64 patients. A gross total or near total resection was reported in 21 cases (32.8%), and subtotal resection or debulking was reported in 25 cases (39.1%). Median overall survival (OS) was 29 months [95% CI 15.8-42.2 months] with 2- and 5-year OS 61 and 39.4% respectively. CONCLUSION: AGG is associated with a dismal. Pediatric age and a gross total resection of tumor confer a better progression-free survival and OS. Hence, surgery should remain the cornerstone of therapy. However, because of modest survival, there is enough opportunity to improve survival with addition of adjuvant radiation and chemotherapy. A whole genome sequencing and molecular characterization would help to derive the best treatment option.

Co-occurrence of histone H3 K27M and BRAF V600E mutations in paediatric midline grade I ganglioglioma.

Ganglioglioma (GG) is a grade I tumor characterized by alterations in the MAPK pathway, including BRAF V600E mutation. Recently, diffuse midline glioma with an H3 K27M mutation was added to the WHO 2016 classification as a new grade IV entity. As co-occurrence of H3 K27M and BRAF V600E mutations has been reported in midline tumors and anaplastic GG, we searched for BRAF V600E and H3 K27M mutations in a series of 54 paediatric midline grade I GG (midline GG) to determine the frequency of double mutations and its relevance for prognosis. Twenty-seven patients (50%) possessed the BRAF V600E mutation. The frequency of the co-occurrence of H3F3A/BRAF mutations at diagnosis was 9.3%. No H3 K27M mutation was detected in the absence of the BRAF V600E mutation. Double-immunostaining revealed that BRAF V600E and H3 K27M mutant proteins were present in both the glial and neuronal components. Immunopositivity for the BRAF V600E mutant protein correlated with BRAF mutation status as detected by massARRAY or digital droplet PCR. The median follow-up of patients with double mutation was 4 years. One patient died of progressive disease 8 years after diagnosis, whereas the four other patients were all alive with stable disease at the last clinical follow-up (at 9 months, 1 year and 7 years) without adjuvant therapy. We demonstrate in this first series of midline GGs that the H3 K27M mutation can occur in association with the BRAF V600E mutation in grade I glioneuronal tumors. Despite the presence of H3 K27M mutations, these cases should not be graded and treated as grade IV tumors because they have a better spontaneous outcome than classic diffuse midline H3 K27M-mutant glioma. These data suggest that H3 K27M cannot be considered a specific hallmark of grade IV diffuse gliomas and highlight the importance of integrated histomolecular diagnosis in paediatric brain tumors.

Ganglioglioma Progression to Combined Anaplastic Ganglioglioma and Anaplastic Pleomorphic Xanthoastrocytoma. Case Report and Literature Review.

BACKGROUND: Composite ganglioma and pleomorphic xanthoastrocytoma with anaplastic features in both components is an extremely rare glioneuronal tumor. Five cases of anaplastic progression in the glioma component have been reported. These tumors generally affect young patients who have brain tumor-related epilepsy, which are usually located in the temporal lobe or in the cerebellum and may have associated leptomeningeal spreading. Its current optimal treatment consists of maximal safe surgical resection and adjuvant chemoradiotherapy. Overall survival at 5 years is 33% in anaplastic pleomorphic xanthoastrocytoma and 53% in anaplastic ganglioglioma. CASE DESCRIPTION: We describe a progression from ganglioglioma to this composite anaplastic entity after 32 months of follow-up, with apparently nontumoral parenchyma separating the 2 components. Polymerase chain reaction showed a wild-type BRAF gene. Seven months after concomitant chemoradiotherapy, radiologic progression led to a second line of chemotherapy, and a third line of chemotherapy was initiated after a subsequent progression at 11 months. CONCLUSIONS: This case may add some evidence in favor of the glioneuronal maldevelopment hypothesis to explain the oncogenesis of these neuroepithelial tumors.

Gliosarcomas with the BRAF V600E mutation: a report of two cases and review of the literature.

Gliosarcoma, which is regarded as a variant of glioblastoma, is a rare malignant neoplasm of the central nervous system. Both its sarcomatous component and glial component are reported to share significant clinical and genetic similarities. However, gliosarcomas are considered to be characterised by a lack of the BRAF V600E mutation. Here, we report two cases of gliosarcoma harbouring the BRAF V600E mutation, of which one case appears to have arisen de novo, while the other likely arose from ganglioglioma. Interestingly, the BRAF V600E mutation was detected only in the glial component in the first case, but was present in both the glial and the sarcomatous components in the recurrent gliosarcoma. Furthermore, the different mutation state of BRAF V600E in our two cases suggests that the malignant transformation of gliosarcoma might have different underlying genetic alterations and mechanisms in de novo versus recurrent gliosarcoma.

Natural course and prognosis of anaplastic gangliogliomas: a multicenter retrospective study of 43 cases from the French Brain Tumor Database.

Background: Anaplastic gangliogliomas (GGGs) are rare tumors whose natural history is poorly documented. We aimed to define their clinical and imaging features and to identify prognostic factors. Methods: Consecutive cases of anaplastic GGGs in adults prospectively entered into the French Brain Tumor Database between March 2004 and April 2014 were screened. After diagnosis was confirmed by pathological review, clinical, imaging, therapeutic, and outcome data were collected retrospectively. Results: Forty-three patients with anaplastic GGG (median age, 49.4 y) from 18 centers were included. Presenting symptoms were neurological deficit (37.2%), epileptic seizure (37.2%), or increased intracranial pressure (25.6%). Typical imaging findings were unifocal location (94.7%), contrast enhancement (88.1%), central necrosis (43.2%), and mass effect (47.6%). Therapeutic strategy included surgical resection (95.3%), adjuvant radiochemotherapy (48.8%), or radiotherapy alone (27.9%). Median progression-free survival (PFS) and overall survival (OS) were 8.0 and 24.7 months, respectively. Three- and 5-year tumor recurrence rates were 69% and 100%, respectively. The 5-year survival rate was 24.9%. Considering unadjusted significant prognostic factors, tumor midline crossing and frontal location were associated with shorter OS. Temporal and parietal locations were associated with longer and shorter PFS, respectively. None of these factors remained statistically significant in multivariate analysis. Conclusions: We report a large series providing clinical, imaging, therapeutic, and prognostic features of adult patients treated for an intracerebral anaplastic GGG. Our results show that pathological diagnosis is difficult, that survivals are only slightly better than for glioblastomas, and that complete surgical resection followed with adjuvant chemoradiotherapy offers longer survival.

Pediatric Basal Ganglia Region Tumors: Clinical and Radiologic Features Correlated with Histopathologic Findings.

PURPOSE: To summarize the clinical and radiologic features of pediatric basal ganglia region tumors (PBGRT) in correlation with their histopathologic findings to reduce inappropriate surgery and identify tumors that can benefit from maximal safe resection. METHODS: The records of 35 children with PBGRT treated in our hospital from December 2011 to December 2015 were analyzed retrospectively. The clinical and radiologic features of these tumors were summarized and correlated with their histopathologic diagnosis. RESULTS: Our series included 15 astrocytomas and 11 germ cell tumors (GCTs). Basal ganglia astrocytomas were characterized by various clinical presentations and an ill-circumscribed mass with the involvement of surrounding structures on neuroimaging and mostly occurred in the first decade of life (n = 10; 66.7%). Basal ganglia GCT mostly occurred in the second decade of life (n = 8; 72.7%) with hemiparesis as the most common symptom (n = 9; 81.8%). The tumors were located predominantly in the caput of caudate nucleus (n = 8; 72.7%) with hemiatrophy as the typical sign (n = 8; 72.7%). Occasionally, other tumors also could occur in this region, including primitive neuroectodermal tumor (n = 1), atypical teratoid/rhabdoid tumor (n = 1), anaplastic ependymoma (n = 1), lymphoma (n = 1), extraventricular neurocytoma (n = 1), gangliogliomas (n = 2), oligodendroglioma (n = 1), and dysembryoplastic neuroepithelial tumor (n = 1). CONCLUSIONS: Astrocytoma and GCT are the most common PBGRTs. Low-grade astrocytomas could benefit from maximal surgical resection, whereas GCTs merit neoadjuvant chemoradiation therapy followed by second-look surgery. We advocate routine testing of tumor markers and analysis of their clinical and radiologic features to optimize the therapeutic strategy.

Epileptic seizures in anaplastic gangliogliomas.

AIM: Prevalence and predictors of epileptic seizures are unknown in the malignant variant of ganglioglioma. METHODS: In a retrospective exploratory dataset of 18 supratentorial anaplastic World Health Organization grade III gangliogliomas, we studied: (i) the prevalence and predictors of epileptic seizures at diagnosis; (ii) the evolution of seizures during tumor evolution; (iii) seizure control rates and predictors of epilepsy control after oncological treatments. RESULTS: Epileptic seizures prevalence progresses throughout the natural course of anaplastic gangliogliomas: 44% at imaging discovery, 67% at histopathological diagnosis, 69% following oncological treatment, 86% at tumor progression, and 100% at the end-of-life phase. The medical control of seizures and their refractory status worsened during the tumor's natural course: 25% of uncontrolled seizures at histopathological diagnosis, 40% following oncological treatment, 45.5% at tumor progression, and 45.5% at the end-of-life phase. Predictors of seizures at diagnosis appeared related to the tumor location (i.e. temporal and/or cortical involvement). Prognostic parameters of seizure control after first-line oncological treatment were temporal tumor location, eosinophilic granular bodies, TP53 mutation, and extent of resection. Prognostic parameters of seizure control at tumor progression were a history of epileptic seizures at diagnosis, seizure control after first-line oncological treatment, eosinophilic granular bodies, and TP53 mutation. CONCLUSION: Epileptic seizures are frequently observed in anaplastic gangliogliomas and both prevalence and medically refractory status worsen during the tumor's natural course. Both oncological and antiepileptic treatments should be employed to improve the control of epileptic seizures and the quality of life of patients harboring an anaplastic ganglioglioma.

Cerebellopontine angle gangliogliomas: Report of two cases.

BACKGROUND: Gangliogliomas are rare tumors of the central nervous system. We report two unusual cases of gangliogliomas located in the cerebellopontine angle (CPA). POPULATION AND METHODS: The first patient was a 57-year-old woman, who presented with dizziness and harbored a non-enhanced heterogeneous mass located in the cisternal space of the CPA. A partial microsurgical removal was performed, and the pathological examination concluded a grade I ganglioglioma according to the WHO Classification. The postoperative course was uneventful without any adjuvant treatment and the 5-year imaging follow-up indicated a stable remnant tumor. The second patient was a 35-year-old male who presented with acute vertigo and imbalance associated with recent prominent headaches; MR imaging showed a large heterogeneous and post-contrast enhanced tumor mass located in the CPA cistern with a mass effect on the brain. An optimal subtotal surgical resection was performed. The pathologists concluded a WHO grade III ganglioglioma. In spite of adjuvant radiotherapy and chemotherapy, the evolution proved unfavorable and patient died from cancer complications within a 2-year period. In both cases, the precise origin of the tumor could not be clearly identified even if the major component was present in the cisternal space. CONCLUSION: Gangliogliomas growing into the cisternal spaces are exceedingly rare particularly in the CPA. Due to its infiltrating behavior and major difficulties to identify the tumor margins, total resection is not routinely feasible. The histological grading is the most important predictor for oncological prognosis.

Supratentorial tumors typical of the infantile age: desmoplastic infantile ganglioglioma (DIG) and astrocytoma (DIA). A review.

OBJECT: Desmoplastic infantile gangliogliomas (DIGs) and desmoplastic infantile astrocytomas (DIAs) are tumors typical of the infantile age. A large size, with a mixed solid and cystic component, clinical presentation with progressing signs of increased intracranial pressure, a prominent benign desmoplastic structure at histological examination, and a favorable clinical course in the majority of cases are the prominent features of these tumors. The objective of the present paper was to review the pertinent literature on the topic together with our personal experience, with the aim of an updated review of the subject. RESULTS AND CONCLUSIONS: Only 28 papers are present in the literature devoted to DIGs and DIAs, most of them reporting on single cases or small series, with a total of 107 patients aged from 5 days to 48 months with a slight male prevalence. Most of the reported cases refer to supratentorial and hemispheric locations, a few cases involving the hypothalamic region, the posterior fossa, and the spinal cord. The typical MRI appearance is of large mixed solid and cystic tumors with a spontaneous hyperintense T2 appearance of the solid part which also shows a strong contrast enhancement. Mixed ganglionic and astrocytic cells are identifiable in DIGs, whereas DIAs are typically featured by the exclusive presence of glial cells. In both cases, more primitive cells may be observed, which present a higher number of mitoses and these areas can mimic the features of malignant astrocytomas. Surgery represents the treatment of choice; however, radical removal has been reported as possible only in around 30 % of the cases: the low age of the patients together with their low weight and the large size of and the hyper-vascularized structure of the tumors represent the main factors limiting surgery. Pure observation is considered as first choice in children undergoing a partial/subtotal tumor resection, chemotherapic regimens being considered in cases of recurrences after a second look surgery. Long-term prognosis is favorable with mortality being related mostly to the rare midline (i.e., hypothalamic) locations, which beyond the functionally relevant site, tend to have an unusually more aggressive histological behavior.