Incidence, time trends and survival patterns of childhood pilocytic astrocytomas in Southern-Eastern Europe and SEER, US.

Pilocytic astrocytomas (PA) comprise the most common childhood central nervous system (CNS) tumor. Exploiting registry-based data from Southern and Eastern Europe (SEE) and SEER, US, we opted to examine incidence, time trends, survival and tentative outcome disparities of childhood PA by sociodemographic and clinical features. Childhood PA were retrieved from 12 SEE registries (N = 552; 1983-2014) and SEER (N = 2723; 1973-2012). Age-standardized incidence rates (ASR) were estimated and survival was examined via Kaplan-Meier and Cox regression analysis. ASR of childhood PA during 1990-2012 in SEE was 4.2/106, doubling in the USA (8.2/106). Increasing trends, more prominent during earlier registration years, were recorded in both areas (SEE: +4.1 %, USA: +4.6 %, annually). Cerebellum comprised the most common location, apart from infants in whom supratentorial locations prevailed. Age at diagnosis was 1 year earlier in SEE, whereas 10-year survival was 87 % in SEE and 96 % in SEER, improving over time. Significant outcome predictors were age <1 year at diagnosis diagnosis (hazard ratio, HR [95% confidence intervals]: 3.96, [2.28-6.90]), female gender (HR: 1.38, [1.01-1.88]), residence in SEE (HR: 4.07, [2.95-5.61]) and rural areas (HR: 2.23, [1.53-3.27]), whereas non-cerebellar locations were associated with a 9- to 12-fold increase in risk of death. The first comprehensive overview of childhood PA epidemiology showed survival gains but also outcome discrepancies by geographical region and urbanization pointing to healthcare inequalities. The worse prognosis of infants and, possibly, females merits further consideration, as it might point to treatment adjustment needs, whereas expansion of systematic registration will allow interpretation of incidence variations.

Repair of a Transclival Meningocele Through a Transoral Approach: Case Report and Literature Review.

BACKGROUND: Transclival meningoceles and related primary spontaneous cerebrospinal fluid leaks at the clivus are extremely rare lesions, with only a few cases reported in the literature. CASE DESCRIPTION: An infant presented with nasal airway obstruction and meningitis secondary to cerebrospinal fluid leak from a transclival meningocele. The radiologic investigation and surgical repair of the lesion are reported, along with intraoperative findings. Basic anatomy, embryology, and development of the clivus are reviewed to clarify the relationship of anatomic variants with the formation of transclival meningoceles. CONCLUSIONS: Transclival meningocele should be considered in patients with spontaneous cerebrospinal fluid rhinorrhea. The existing classification of meningoencephaloceles based on the location of the defect in the cranium should probably be reconsidered, taking into consideration the described entity.

Torkildsen shunt as a salvage procedure for an infant with post-hemorrhagic hydrocephalus.

INTRODUCTION: Torkildsen operation is a ventriculo-cisternal shunt that diverts the cerebrospinal fluid flow from one of the lateral ventricles, via a ventricular catheter, to the cisterna magna of the posterior fossa. It is the first described operation of CSF diversion for the treatment of hydrocephalus [1] (Scarff, 1963). PRESENTATION OF CASE: We present the case of a premature infant who presented with post hemorrhagic hydrocephalus who underwent several failed CSF diversion surgeries before Torkildsen shunt procedure. The patient overcame the irregularities of CSF circulation that were observed before the operation and his post-operative course was uneventful, in terms of CSF -related complications. DISCUSSION: We summarize the historical data and the technical aspects of the procedure, and we present a brief literature review of the indications and limitations associated with it. CONCLUSION: Torkildsen shunt can be effective in selected patients with hydrocephalus even in the modern era of computed tomography and magnetic resonance imaging. the procedure enables one to avoid a standard ventriculoperitoneal shunt, especially in cases it is not technically feasible.

Herpes Simplex Encephalitis Initially Erroneously Diagnosed as Glioma of the Cerebellum: Case Report and Literature Review.

BACKGROUND: Herpes simplex encephalitis caused by herpes simplex has an estimated annual prevalence in the order of 1 in 250,000 to 500,000 patients and is considered to be the most usually encountered nonendemic pathogenic cause of lethal encephalitis in well-developed countries. There are a few cases reported in the literature in which a diagnostic dilemma between was raised between herpes simplex encephalitis and brain glioma and a definitive diagnosis was difficult to obtain. CASE DESCRIPTION: We report the case of a 5-year-old girl with a previous medical history of premature thelarche. As a part of her investigation, magnetic resonance imaging of the brain was performed, which revealed a space-occupying lesion of the posterior fossa. Magnetic resonance spectroscopy was performed, which advocated for the diagnosis of glioma. She was operated on, but the histopathologic analysis failed to verify the imaging findings. Herpes simplex virus (HSV) 1 was detected in cerebrospinal fluid (CSF) samples. Follow-up magnetic resonance imaging scans illustrated the progression of the disease. CONCLUSIONS: The accumulation of data regarding CSF sample analysis, electroencephalography, brain biopsy, and imaging findings, along with the progression of the clinical picture of our patient, verified the diagnosis of HSV encephalitis. When confronted with confounding data that can pose a diagnostic dilemma between HSV encephalitis and glioma, brain biopsy and polymerase chain reaction of CSF samples could be able to verify the definitive diagnosis. When interpreting our results, we always have to consider the evolution of the clinical picture.

Primary, Dural-Based, Ewing Sarcoma Manifesting with Seizure Activity: Presentation of a Rare Tumor Entity with Literature Review.

BACKGROUND: Primary Ewing sarcoma arising from the calvarial bone and/or underlying dura matter are relatively rarely reported in the literature. Even rarer are considered to be those that originate from the dura and proliferate in both directions, toward the brain parenchyma and through the dura invading the overlying bone. CASE DESCRIPTION: We report the case of a girl aged 11 years who presented with an ictus of generalized tonic-clonic seizures, without known focal neurologic deficit. Neuroradiologic work-up verified the existence of a tumor in the right fronto-parietal area of the brain, invading both the bone and brain parenchyma and emanating from the dura. No other tumor location was discovered after radiologic work-up, and gross total resection was undertaken followed by adjuvant radiation therapy and chemotherapy. CONCLUSIONS: The patient had an uneventful neurologic recovery without persistent neurologic deficit. One year after the operation, with the patient under close clinical and radiologic surveillance, no recurrence of the disease was detected. This case illustrates an extremely uncommon location of primary Ewing sarcoma, along with a constellation of clinical signs and symptoms that are fairly uncommon for this location of this rare disease entity.

Pediatric Nonmissile Penetrating Head Injury: Case Series and Literature Review.

BACKGROUND: Pediatric nonmissile penetrating head injury (NMPHI) is usually accidental attributed mainly to the softer skulls of growing children. However, it is a rare entity, and therefore no consensus exists regarding treatment to effectively prevent immediate and long-term complications. Throughout the literature, these injuries are mostly discussed in case reviews and case series in the general population. No data originating from randomized studies are available because of ethical and practical limitations. METHODS: We retrospectively studied and present 5 cases of children with NMPHI treated in the last 6 years in the Neurosurgery Department of Children's Hospital "Aghia Sofia". We performed a review of the literature in PubMed, using the key words "non-missile," "penetrating head injury," and "pediatric." We included case reports and case series involving pediatric cases since 2008 and selected older reports as well as certain literature reviews focusing on analysis of complications and treatment suggestions. We compared reported practice in various institutions with suggestions from the literature. RESULTS: In the last year, 4 literature reviews were published suggesting treatment algorithms of NMPHIs. Surgery timing and method as well as anticonvulsant and antibiotic therapy still remain debatable. The only review concentrating on pediatric populations dates back to 1994, based on patient outcome studies from the 1980s. In our review, treatment steps were similar among various institutions and resembled recently suggested algorithms, with better treatment outcomes than originally reported 30 years ago.

Aplasia cutis congenita: Two case reports and discussion of the literature.

Background: Aplasia cutis congenita (ACC) is a part of a heterogeneous group of conditions characterized by the congenital absence of epidermis, dermis, and in some cases, subcutaneous tissues or bone usually involving the scalp vertex. There is an estimated incidence of 3 in 10,000 births resulting in a total number of 500 reported cases to date. The lesions may occur on every body surface although localized scalp lesions form the most frequent pattern (70%). Complete aplasia involving bone defects occurs in approximately 20% of cases. ACC can occur as an isolated defect or can be associated with a number of other congenital anomalies such as limb anomalies or embryologic malformations. In patients with large scalp and skull defects, there is increased risk of infection and bleeding along with increased mortality and therefore prompt and effective management is advised. Case Description: We describe two cases of ACC, involving a 4 × 3 cm defect managed conservatively and a larger 10 × 5 cm defect managed surgically with the use of a temporo-occipital scalp flap. Both cases had an excellent outcome. Conclusions: Multiple treatment regimens exist for ACC, but there is no consensus on treatment strategies. Conservative treatment has been described and advocated, but many authors have emphasized the disadvantages of this treatment modality. Decision between conservative and surgical management must be individualized according to lesion size and location.

Central nervous system tumours among adolescents and young adults (15-39 years) in Southern and Eastern Europe: Registration improvements reveal higher incidence rates compared to the US.

AIM: To present incidence of central nervous system (CNS) tumours among adolescents and young adults (AYAs; 15-39 years) derived from registries of Southern and Eastern Europe (SEE) in comparison to the Surveillance, Epidemiology and End Results (SEER), US and explore changes due to etiological parameters or registration improvement via evaluating time trends. METHODS: Diagnoses of 11,438 incident malignant CNS tumours in AYAs (1990-2014) were retrieved from 14 collaborating SEE cancer registries and 13,573 from the publicly available SEER database (1990-2012). Age-adjusted incidence rates (AIRs) were calculated; Poisson and joinpoint regression analyses were performed for temporal trends. RESULTS: The overall AIR of malignant CNS tumours among AYAs was higher in SEE (28.1/million) compared to SEER (24.7/million). Astrocytomas comprised almost half of the cases in both regions, albeit the higher proportion of unspecified cases in SEE registries (30% versus 2.5% in SEER). Similar were the age and gender distributions across SEE and SEER with a male-to-female ratio of 1.3 and an overall increase of incidence by age. Increasing temporal trends in incidence were documented in four SEE registries (Greater Poland, Portugal North, Turkey-Izmir and Ukraine) versus an annual decrease in Croatia (-2.5%) and a rather stable rate in SEER (-0.3%). CONCLUSION: This first report on descriptive epidemiology of AYAs malignant CNS tumours in the SEE area shows higher incidence rates as compared to the United States of America and variable temporal trends that may be linked to registration improvements. Hence, it emphasises the need for optimisation of cancer registration processes, as to enable the in-depth evaluation of the observed patterns by disease subtype.