Remission of Pediatric Diffuse Intrinsic Pontine Glioma: Case Report and Review of the Literature.

BACKGROUND: Diffuse midline glioma (DMG) is one of the most aggressive pediatric tumors. Approximately 60% of pediatric DMG patients die within the first year of diagnosis. Complete clinical and radiological remission of DMG is extremely rare. The objective of this study was to describe a case of remission of pediatric DMG and to compare with similar cases published so far. RESULTS: DMG was diagnosed in a 2-year-old girl who presented with brainstem and increased intracranial pressure manifestations. Ventriculoperitoneal shunt and chemotherapy-based treatment were offered. From the diagnosis, in spite of progressive enlargement of the tumoral lesion, her clinical condition improved remarkably. After the end of chemotherapy, progressive and gradual imagiological improvements occurred. At the end of the 60th month of follow-up, she was asymptomatic with total remission. Six pediatric DMG cases, from birth to the age of 3, in whom remission occurred were found in the literature. Histology sample was available in two of them (fibrillary astrocytoma-WHO Grade II and anaplastic astrocytoma-WHO Grade III). None received chemotherapy or radiotherapy. CONCLUSION: Remission of pediatric DMG is extremely rare and reinforces the biological heterogeneity of the tumor. In the absence of reliable predictors of prognosis, offering the best supportive treatment, including neurosurgical interventions should be considered in similar cases.

Selumetinib for plexiform neurofibromas in neurofibromatosis type 1: a single-institution experience.

BACKGROUND: Plexiform neurofibromas (PN) are the most frequent tumors associated with Neurofibromatosis type 1 (NF-1). PN can cause significant complications, including pain, functional impairment, and disfigurement. There is no efficient medical treatment and, surgical resection of large PN is frequently infeasible. Selumetinib (AZD6244/ARRY-142886) is a mitogen-activated protein kinase enzyme (MEK1/2) inhibitor and works by targeting the MAPK pathway. It is an investigational treatment option for inoperable symptomatic PN associated with NF-1. Herein, we describe a single institutional experience with selumetinib for inoperable PN in NF-1. METHODS: Case series study of demographics, clinical, baseline characteristics, treatment effect, and follow-up of consecutive genetically confirmed NF1 patients with inoperable PN associated with significant or potential significant morbidity treated with selumetinib (April 2018 to April 2019). RESULTS: Nineteen patients were treated with selumetinib. Predominant target locations were head and neck (31.6%, 6/19), chest (26.3%, 5/19) and pelvis (21%, 4/19) and the most important comorbidities were disfigurement (47.4%, 9/19) and pain (26.3%, 5/19). The mean follow-up time was 223 days (range 35-420 days). All but one had sustained clinical improvement, mainly in the first 60-90 days of treatment. In one patient, the treatment was suspended after 168 days (lack of clear benefit and left ventricular ejection fraction drop). There were no adverse effects leading to treatment suspension. CONCLUSIONS: In the first observational study of selumetinib for NF-1 associated PN we showed that the drug was associated with clinical and radiological improvement. Our study also confirms the safety described in the clinical trials.

Tree pollen spectra and pollen allergy risk in the Osijek-Baranja County.

The forests of north-eastern Croatia, as well as various plants and trees in the parks and streets of the Osijek-Baranja County, produce large amounts of pollen during the pollen season, which can cause allergy symptoms in pollen sensitive individuals. The aim of this study was to determine the most frequent types of pollen in this area and estimate possible health risks, especially the risk of allergy. In 2009 and 2010, the staff of the Health Ecology Department of the Osijek Public Health Institute monitored tree pollen concentrations in four cities from the Osijek - Baranja County (Osijek, Nasice, Dakovo and Beli Manastir) using a Burkard volumetric instrument. The results were affected by weather conditions. Windy and sunny days facilitated the transfer of pollen, whereas during rainy days, the concentration of pollen grains decreased. High pollen concentrations of Cupressaceae/Taxaceae, Betulaceae, Salicaceae and Aceraceae could be the cause for symptoms of pollen allergy. In 2009, conifers, birch and poplar pollen were dominant at all monitoring stations with 5000 pollen grains (PG), 3188 PG and 3113 PG respectively. The highest number of pollen grains was recorded at measuring site Osijek. The variations in airborne pollen concentration between pollen seasons were recorded at all monitoring stations. The most obvious variations were recorded at measuring site Osijek. The usual pollination period lasts two to three months, which means that most pollen grains remain present from February to early June. However, the Cupressaceae / Taxaceae pollination periods last the longest and their pollen grains remain present until the end of summer. The risk of allergy was determined at four monitored measuring stations and the obtained data confirmed that the largest number of days with a high health risk was at the Dakovo measuring station for a species of birch. The research information aims to help allergologists and individuals allergic to plant pollen develop preventive measures and proper treatment therapies.