Medulloblastoma is one of the most common pediatric central nervous system malignancies worldwide, and it is characterized by frequent leptomeningeal metastasizing. We report a rare case of primary leptomeningeal medulloblastoma of an 11-year-old Caucasian girl with a long-term disease history, non-specific clinical course, and challenges in the diagnosis verification. To date, 4 cases of pediatric primary leptomeningeal medulloblastoma are reported, and all of them are associated with unfavorable outcomes. The approaches of neuroimaging and diagnosis verification are analyzed in the article to provide opportunities for effective diagnosis of this disease in clinical practice. The reported clinical case of the primary leptomeningeal medulloblastoma is characterized by MR images with non-specific changes in the brain and spinal cord and by 18FDG-PET/CT images with diffuse heterogeneous hyperfixation of the radiopharmaceutical along the whole spinal cord. The immunohistochemistry and next-generation sequencing analyses of tumor samples were performed for comprehensive characterization of the reported clinical case.

Pineal anlage tumors (PAT) are extremely rare malignant tumors in the pediatric population considered to be a variant of pineoblastomas. These tumors are characterized by a combination of neuroectodermal and heterologous ectomesenchymal components. PAT can be clinically aggressive and has a propensity to cerebrospinal fluid (CSF) dissemination. So far, only 13 cases of these tumors have been reported in the literature.
In this article, we report the case of an anlage tumor in a 3-year-old girl with a rapidly aggressive course.

Seizures are a common complication of both primary central nervous system (CNS) tumors and other oncologic processes with CNS involvement. They occur most frequently during induction or consolidation therapy, but there is a growing body of evidence that they can also develop later in life. Refractory epilepsy can develop as a late complication for survivors of pediatric cancer with CNS involvement who undergo chemoradiation therapy.
We report three patients who presented with atypical nonconvulsive seizures (behavioral arrest, falls, nonsensical speech) up to 14 years after cancer diagnosis. All underwent whole-brain radiation in addition to chemotherapy. None had a prior epilepsy diagnosis or known prior seizures. One patient suddenly passed away of unclear causes five months after diagnosis, and the other two continued to have EEG findings consistent with cerebral dysfunction and epileptogenicity years after diagnosis.
We hypothesize that the development of refractory epilepsy may be a late effect of radiation treatment. Given the high morbidity and mortality associated with epilepsy, early identification is crucial to improve outcomes and quality of life for this vulnerable population. This is especially true for patients with medication-refractory epilepsy as there is an increasing breadth of effective surgical options.

Neonatal germ cell tumors are rare and comprise both benign and malignant neoplasms. Teratoma with nephroblastoma is a malignant subset defined pathologically by the presence of nephroblastoma and teratoma elements. Although teratoma with nephroblastoma is most often found in the kidney, 24 of 59 reported cases are associated with extrarenal locations, such as the mediastinum or retroperitoneum. To our knowledge, this is the first patient in the literature with intracranial/pineal teratoma with nephroblastoma, which was managed with staged transcranial approaches resulting in gross total resection and no adjuvant therapy (surveillance observation imaging). We further augmented the patient\'s management by comprehensive genomic profiling of the tumor to better understand the molecular biology and explore options for targeted therapy.

UNASSIGNED: Herpes simplex encephalitis (HSE) occurring within 30 days after neurosurgery for solid CNS tumors is underrecognized and underreported but remains important because of high morbidity and mortality. We present the case of a 41-year-old woman who had HSE after craniopharyngioma surgery, and delayed recognition and treatment led to a poor outcome. Subsequently, we review reported HSE cases after neurosurgery for solid CNS tumors and describe outcomes after treatment with and without acyclovir.
UNASSIGNED: A literature search was performed for cases meeting the above criteria. Information was gathered regarding patient demographics, tumor types, symptoms, diagnostic workup, therapy, and outcomes.
UNASSIGNED: Eighteen cases were studied. Encephalopathy, fever, and seizures were the most common symptoms. A majority of patients (78%) received IV acyclovir, with a 79% survival rate with treatment. Mortality rate was 100% in untreated cases. The median time to starting acyclovir was 17 postoperative days (range, 8-53 days). Most patients received steroids, but its use was not associated with a specific outcome.
UNASSIGNED: HSE may develop following neurosurgical resection, and the threshold for suspicion of this condition should be extremely low in a patient who shows compatible symptoms (encephalopathy, fever, or seizures) or does not recover as planned. Moreover, in case of suspicion of HSE, acyclovir should be promptly started until infection can be definitely ruled out. A delay in diagnosis of HSE and failure to treat may result in severe morbidity as well as mortality. This observation may warrant further study.

BACKGROUND: The public health burden of nonmalignant central nervous system tumors (NMCNSTs) in Canada is unclear because casefinding and registration have historically been incomplete. The primary objective of this study is to quantify case-completeness of NMCNSTs in the Canadian Cancer Registry (CCR) using US Surveillance, Epidemiology and End Results Program (SEER) rates as the standard.
METHODS: Counts, distributions, and age-standardized incidence rates (ASIRs) for malignant central nervous system tumors (MCNSTs) and NMCNSTs by sex, age, site, histology, tumor size, World Health Organization (WHO) grade, and year of diagnosis were estimated for the United States and Canada (excluding Quebec) for the time period 2011-2015 using SEER and CCR data, respectively. Canadian and provincial standardized incidence ratios (SIRs) were also calculated by sex, age, site, histology and year of diagnosis using SEER rates as the standard. Under the assumptions of high NMCNST case-completeness in SEER registries and comparable population-based rates in the United States and Canada, SIRs less than 100% suggest incomplete case registration.
RESULTS: Between 2011 and 2015, the ASIR for MCNSTs is similar in the United States (6.97 per 100,000 persons; 95% CI, 6.89-7.05), Canada (7.11 per 100,000; 95% CI, 6.97-7.24), and across provinces (range, 6.53-7.35 per 100,000). Conversely, the ASIR for NMCNSTs is 1.61 times greater in the United States (17.15 per 100,000; 95% CI, 17.02-17.27) than Canada (10.65 per 100,000; 95% CI, 10.49-10.82). SIRs for NMCNSTs range from 22.5% (95% CI, 15.6%-31.5%) in Prince Edward Island to 85.3% (95% CI, 83.7%-86.9%) in Ontario and vary by demographics, tumor characteristics, and year. Identified data limitations include nonspecific tumor characteristics and potential misclassification.
CONCLUSIONS: NMCNST surveillance in Canada is compromised by incomplete case registration and data quality limitations. Enhancement of case ascertainment processes for these tumors, which may be diagnosed radiologically, may be warranted.