Pediatric infratentorial ganglioglioma.

PURPOSE: Pediatric infratentorial gangliogliomas are exceedingly rare tumors; thus, the factors affecting their outcome are poorly understood and their optimal management has still to be defined. METHODS: We reviewed the literature on pediatric gangliogliomas with a focus on those located in the posterior fossa to study treatment and outcome data. We added to this review some of our clinical cases. RESULTS: We found 100 and 80 cases of brainstem and cerebellar pediatric ganglioglioma, respectively, in our literature review. The surgical management varied from biopsy to gross total resection, and adjuvant treatment was given after incomplete resection or at time of progression. A gross total resection should be attempted to remove the contrast enhancing part of the tumor, which may be possible in most of the cerebellar gangliogliomas and some of the brainstem lesions. The cervicomedullary ganglioglioma seems to be the most infiltrative and least amenable to complete resection. Chemotherapy has a limited role and BRAF mutation was reported in 38 to 54 % of cases. The use of radiotherapy exposes the patient to a risk of malignant transformation and should be reserved for unresectable tumors which progress. CONCLUSION: Pediatric posterior fossa gangliogliomas are rare and challenging tumors due to their frequent infiltrative component involving the brainstem. To date, adjuvant therapy has a limited role that may evolve with time thanks to the use of targeted therapies against BRAF mutation. The surgical resection of well-defined contrast enhancing parts should be attempted even in staged surgeries and balanced with the risks of neurological deterioration.

The management of birth-related posterior fossa hematomas in neonates.

BACKGROUND: Symptomatic posterior fossa hematoma in the term newborn is rare. OBJECTIVE: To report on the management and outcome of posterior fossa subdural hematoma (PFSDH) in neonates. METHODS: A retrospective analysis of the department database and clinical notes of neonates admitted since 1985 with a PFSDH was performed together with a literature review of similar case series. RESULTS: Sixteen patients were included. The median gestational age was 40 weeks with a high proportion of primiparous mothers (n = 9) and forceps delivery (n = 9). Nine neonates had symptoms of brainstem dysfunction within the first 24 hours of life, whereas the other 7 had a delayed presentation (median 4 days) with signs of raised intracranial pressure due to hydrocephalus. Each patient had a cranial ultrasound followed by computed tomography scan that showed the PFSDH. Eleven neonates required surgical evacuation of the PFSDH, whereas hydrocephalus was managed by transient external ventricular drainage in 2 further patients. Eventually, 2 neonates required a permanent ventriculoperitoneal shunt. Five neonates had no operative intervention. With a mean follow-up of 7.8 years, 2 patients had mild developmental delay and 1 had severe developmental delay. The 13 other patients had a normal development. CONCLUSION: In neonates with a PFSDH, surgery can be safely performed in those who have clinical and radiological signs of brainstem compression or hydrocephalus. A small number of neonates require a ventriculoperitoneal shunt in the long term. Initial aggressive resuscitation should be performed even in cases of initial severe brainstem dysfunction because of the good long-term neurological outcome.

Neuropathological and neuroradiological spectrum of pediatric malignant gliomas: correlation with outcome.

BACKGROUND: The diagnostic accuracy and reproducibility for glioma histological diagnosis are suboptimal. OBJECTIVE: To characterize radiological and histological features in pediatric malignant gliomas and to determine whether they had an impact on survival. METHODS: We retrospectively reviewed a series of 96 pediatric malignant gliomas. All histological samples were blindly and independently reviewed and classified according to World Health Organization 2007 and Sainte-Anne classifications. Radiological features were reviewed independently. Statistical analyses were performed to investigate the relationship between clinical, radiological, and histological features and survival. RESULTS: Cohort median age was 7.8 years; median follow-up was 4.8 years. Tumors involved cerebral hemispheres or basal ganglia in 82% of cases and brainstem in the remaining 18%. After histopathological review, low-grade gliomas and nonglial tumors were excluded (n = 27). The World Health Organization classification was not able to demonstrate differences between groups and patients survival. The Sainte-Anne classification identified a 3-year survival rate difference between the histological subgroups (oligodendroglioma A, oligodendroglioma B, malignant glioneuronal tumors, and glioblastomas; P = .02). The malignant glioneuronal tumor was the only glioma subtype with specific radiological features. Tumor location was significantly associated with 3-year survival rate (P = .005). Meningeal attachment was the only radiological criteria associated with longer survival (P = .02). CONCLUSION: The Sainte-Anne classification was better able to distinguish pediatric malignant gliomas in terms of survival compared with the World Health Organization classification. In this series, neither of these 2 histological classifications provided a prognostic stratification of the patients.

Forniceal glioma in children. Clinical article.

OBJECT: Five to ten percent of pediatric brain tumors are located in the ventricles. Among them, forniceal lesions are rare and their management has not often been described. The aim of this study was to review the clinical, radiological, and histopathological features as well as the feasibility of surgical excision and the outcomes in these patients. METHODS: From a retrospective analysis of 250 cases of supratentorial pediatric glioma, the records of 8 children presenting with forniceal lesions were selected and reviewed. RESULTS: The median age of patients in the cohort was 13.5 years. Presenting features included intracranial hypertension (7 cases), hypothalamic dysfunction (2), and memory dysfunction (3). Complete resection was possible in only 1 case, where the lesion was mainly exophytic; the remaining patients had either a partial resection or biopsy. On histological review, the tumors were confirmed as pilocytic astrocytoma (4 lesions), WHO Grade II astrocytoma (3), and ganglioglioma (1). Postoperatively, working and retrograde memory was normal for all patients, but the authors found a mild alteration in verbal episodic memory in 5 patients. Despite fatigability for 5 patients, academic achievement was normal for all but 2, both of whom had preoperative school difficulties. Additional treatment was required for 5 patients for tumor progression, with a median interval of 19 months from surgery. At a median follow-up duration of 4.9 years, all patients had stable disease. CONCLUSIONS: In this series, forniceal gliomas were found to be low-grade gliomas. They are surgically challenging, and only exophytic lesions may be cured surgically. Due to the high rate of progression of residual disease, adjuvant therapy is recommended for infiltrative tumors, and it yielded excellent results.