Anomalous vascularization in a Wnt medulloblastoma: A case report

BACKGROUND: Medulloblastoma is the most common malignant brain tumor in children. To date only few cases of medulloblastoma with hemorrhages have been reported in the literature. Although some studies speculate on the pathogenesis of this anomalous increased vascularization in medulloblastoma, the specific mechanism is still far from clearly understood. A correlation between molecular medulloblastoma subgroups and hemorrhagic features has not been reported, although recent preliminary studies described that WNT-subtype tumors display increased vascularization and hemorrhaging. CASE PRESENTATION: Herein, we describe a child with a Wnt-medulloblastoma presenting as cerebellar-vermian hemorrhagic lesion. Brain magnetic resonance imaging (MRI) showed the presence of a midline posterior fossa mass with a cystic hemorrhagic component. The differential diagnosis based on imaging included cavernous hemangioma, arteriovenous malformation and traumatic lesion. At surgery, the tumor appeared richly vascularized as documented by the preoperative angiography. CONCLUSIONS: The case we present showed that Wnt medulloblastoma may be associated with anomalous vascularization. Further studies are needed to elucidate if there is a link between the hypervascularization and the Wnt/β-catenin signaling activation and if this abnormal vasculature might influence drug penetration contributing to good prognosis of this medulloblastoma subgroup

NS-15NEUROSURGICAL MANAGEMENT OF TECTAL GLIOMAS

Tectal gliomas are a rare entity mostly showing an indolent clinical course. However, in some cases progression of disease prompts medical treatment. We report a series of 12 consecutive patients diagnosed with tectal glioma at the Bambino Gesù Children's Hospital from 2012 to 2016. Diagnosis was made according to typical radiological features. Mean follow-up was 43 months (4 to 75 months). Symptoms at onset were macrocrania and full fontanelle in congenital cases and headache, vomiting, ataxia, postural instability, short term memory and attention difficulties in older children, suggesting a strong role of hydrocephalus. Mean age at diagnosis was 8 years and girls were three times more frequent than boys. Hydrocephalus was present at the time of the diagnosis virtually in all cases (10/11). In three cases hydrocephalus was congenital but diagnosis of tectal glioma was made 8 years later in one case, due to limited access to MRI imaging at the time of hydrocephalus treatment. Hydrocephalus was treated by endoscopic ventriculostomy in all patients presenting after 2008. We had a major complication in one girl, suffering acute intracranial hypertension with severe outcome 20 months after diagnosis presumably from unexpected ventriculostomy closure. After that, we propose routine positioning of a Rickham ventricular access device in all children with hydrocephalus secondary to tectal gliomas. Three children showed progression of their tectal lesion or gaze disturbances prompting stereotactic needle biopsy to obtain histological diagnosis and start oncological treatment. Surgical resection was never indicated, as large esophytic lesions were not observed in our series

Rethinking the Management of Optic Pathway Gliomas: A Single Center Experience

Background: Optic pathway gliomas (OPGs) are rare neoplasms in children with an unpredictable clinical course. Approximately 15% of OPGs occur in patients affected by neurofibromatosis type 1 (NF1): the clinical course of these cases is more indolently than sporadic ones, and NF1 patients less frequently require treatment including surgery. Instead, over 90% of sporadic OPGs require one or more therapeutic approaches. The management of OPG is controversial. They are also characterized by a high risk of morbidity including hypothalamic damage, endocrine deficits, visual deficit and/or neurological impairment.Materials and Methods: In this paper, we evaluated visual and endocrinological outcomes of a population of OPG followed at our center from 2013 to 2021, with a particular emphasis on the role of surgery.Results: Twenty-six patients were included in this study (mean age of 40.7 months). Tumor location on imaging was described by the Dodge classification. Five cases had NF 1. Thirteen cases received biopsy and 13 were partially resected. Histopathology revealed 19 cases of pilocytic astrocytomas, 2 pilomyxoid astrocytoma and 5 ganglioglioma. All the patients required a post-surgical adjuvant treatment according to current indications for low-grade gliomas. Molecular studies (BRAF status and mTOR/pmTOR pathway) have been performed in 24/26 patients, following for the use of target therapy in 11 of these patients. In our study we found that patients underwent biopsy have a better visual and endocrinological outcomes rather than patients with a tumor debulking. The five-year overall survival rate is 98% with a mean follow-up of 60 months.Conclusions: Many children with OPGs survive with a residual tumor. They suffer from chronic diseases such as endocrine dysfunction, visual disturbance, motor deficits and poor quality of life. All patients need comprehensive diagnostic work-up including neuroimaging, clinical evaluations and neuropathology approach; at the same time, they need therapeutic decisions and concepts for the choice of timing and type of neurosurgical intervention, chemotherapy and target therapy as well as surveillance and rehabilitation to maximize survival and overall functional outcomes. Our study showed that minimal invasive surgery with the purpose of molecular characterization of the tumor is desirable to reduce morbidity correlate to surgery

NS-25IMPACT OF MEDULLOBLASTOMA MOLECULAR SUBGROUP ON POST-OPERATIVE PSEUDOMENINGOCELE AND NEED FOR VENTRICULAR SHUNTING

Post-operative hydrocephalus and pseudomeningocele is a frequent scenario after posterior fossa surgery that often conditions timing of adjuvant treatments. Molecular subgrouping in medulloblastoma (MB) has shown relevant prognostic and therapeutic significance. However, little information is available about the possibility of different surgical outcomes depending on MB subgrouping. We report our series of 35 consecutive MB patients treated at the Bambino Gesù Children's Hospital from 2011 to 2016. Molecular subgroup was available in 29 cases. We retrospectively reviewed CSF related complications in this population and analyzed possible association to molecular subgroups. Whenever clinically indicated we performed a preoperative endoscopic ventriculostomy to improve CSF circulation. External ventricular drainage was never used in our series. Twenty-four patients (83%) presented with ventriculomegaly and 17 (59%) had symptomatic hydrocephalus. Fifteen patients were treated with endoscopic ventriculostomy before respective surgery. After surgery, 23 (79%) children had a subcutaneous CSF collection, mostly resolving spontaneously. In fact, only 7 (30%) of them required treatment for their pseudomeningocele. Treatments included lumbar puncture, needle evacuation of subcutaneous collection, wound revision. VP shunt was necessary in 3 patients (10% of total population, 13% of children showing post-operative subcutaneous collection). Need to treat the subcutaneous CSF collection was significantly conditioned by molecular subgroup (4 patients were group 3, 2 patients group 4 and 1 patient SHH). Patients requiring a VP shunt belonged to either group 3 or 4. Our data suggest that post-operative hydrocephalus might be associated to specific molecular subgroups in MB patients

Humoral and cellular immune response after mRNA SARS-CoV-2 vaccine in children on treatment for cancer: A pilot observational study

Immunocompromised children are at risk of developing severe COVID-19 infection. We conducted a pilot prospective study to evaluate the impact of cancer treatment and stem cell transplantation on immunogenicity of two doses of BNT162b2 vaccine in pediatric patients.Humoral, B- and T-cell responses to the BNT162b2 vaccine were assessed before, after the first and the second dose in patients aged 5–12 years (n = 35) and in a group of healthy donors (HD, n = 12). Patients were divided in three groups: solid tumors (ST, n = 11), hematological malignancies (HM, n = 14) and Hematopoietic Stem Cell Transplantation (HSCT) recipients (n = 10). After two vaccine doses, the seroconversion rate was 79.3 % (72.7 % in ST, 66.7 % in HM and 100 % in HSCT). The antibodies production was not associated to the presence of memory B and T-cells. Memory B-cells were measurable in 45.5 % ST, 66.6 % HSCT and in 22.0 % HM. The specific T-cell response was observed in most ST (81.8 %) and HSCT (85.7 %) patients and at lesser extent in those with HM (55.5 %). The combination of all immunological parameters (antibodies, memory B and T cells) showed that a significant fraction of HM (33.3 %) and ST (18.2 %) patients completely failed to respond to vaccination. Although able to produce antibodies, 11.1 % of HM and 27.3 % of ST had no B- and T-cell memory. HSCT subgroup showed the best immune function, with 80 % complete response and optimal T-cell function.Combination of anti-RBD antibody, and specific memory B- and T-cell responses represents a reliable read-out of vaccine immune efficacy in frail patients

In-hospital and 6-month outcomes in patients with COVID-19 supported with extracorporeal membrane oxygenation (EuroECMO-COVID): a multicentre, prospective observational study

Extracorporeal membrane oxygenation (ECMO) has been widely used in patients with COVID-19, but uncertainty remains about the determinants of in-hospital mortality and data on post-discharge outcomes are scarce. The aims of this study were to investigate the variables associated with in-hospital outcomes in patients who received ECMO during the first wave of COVID-19 and to describe the status of patients 6 months after ECMO initiation