Immunohistochemical Expression of PAX8 in Central Nervous System Hemangioblastomas: A Potential Diagnostic Pitfall for Neuropathologists.

The histologic differential diagnosis between intracranial hemangioblastoma (HB) and metastatic clear cell renal cell carcinoma may be challenging, especially considering that both tumors exhibit clear cell morphology and can be associated with vHL mutation and/or Von Hippel-Lindau syndrome. As the execution of immunohistochemical analyses is often mandatory, the expression of PAX8 has been traditionally considered a reliable marker of metastatic clear cell renal cell carcinoma, being consistently negative in intracranial HB. However, as in recent years, some cases of PAX8-positive HBs have been reported in the literature; we studied the expression of this antibody on a series of 23 intracranial HB, showing that about 40% of these tumors may express PAX8 and that this immunoreactivity is often focal and weak.

Multiomics dissection of human RAG deficiency reveals distinctive patterns of immune dysregulation but a common inflammatory signature.

Human recombination-activating gene (RAG) deficiency can manifest with distinct clinical and immunological phenotypes. By applying a multiomics approach to a large group of -mutated patients, we aimed at characterizing the immunopathology associated with each phenotype. Although defective T and B cell development is common to all phenotypes, patients with hypomorphic variants can generate T and B cells with signatures of immune dysregulation and produce autoantibodies to a broad range of self-antigens, including type I interferons.

Characterization of LiF TLD-100 in carbon ion beams for remote audits.

Background: A passive dosimeter framework for the measurement of dose in carbon ion beams has yet to be characterized or implemented for regular use.

Purpose: This work determined the dose calculation correction factors for absorbed dose in thermoluminescent dosimeters (TLDs) in a therapeutic carbon ion beam. TLD could be a useful tool for remote audits, particularly in the context of clinical trials as new protocols are developed for carbon ion radiotherapy.

Mixed- and multi-relative biological effectiveness model simultaneous optimization in carbon ion radiotherapy: A proof-of-concept.

Background And Purpose: In carbon ion radiotherapy (CIRT), different relative biological effectiveness (RBE) models have been used for calculating RBE-weighted dose (D). Conversion between current RBE predictions and introduction of novel approaches remains a challenging task. Our aim is to introduce a framework considering multiple RBE models simultaneously during CIRT plan optimization, easing the translation between D prescriptions.

Clinico-Pathological Features of Diffuse Midline Glioma, H3 K27-Altered in Adults: A Comprehensive Review of the Literature with an Additional Single-Institution Case Series.

Diffuse midline glioma (DMG), H3 K27-altered, is a WHO grade 4 malignant glioma located at midline structures, including the thalamus, brainstem and spinal cord. While H3 K27-altered DMG is more common in pediatric age in which it shows a uniformly aggressive clinical behavior, its occurrence is relatively unusual among adults, and its clinico-pathological and prognostic features are not fully characterized in this age group. In this present paper, a review of the literature, including all cases of adult H3 K27-altered DMG published from January 2010 to December 2023 was performed, and the following clinical parameters were evaluated: sex, age (median and range), anatomic site, median follow-up, leptomeningeal dissemination, local recurrence and treatment.

Vaginal Mucosal Melanoma Cell Activation in Response to Photon or Carbon Ion Irradiation.

Purpose: Primary gynecological melanomas are uncommon with lower survival rates compared to cutaneous melanomas. Although melanocytes have been identified in a variety of mucosal membranes, little is known about their interactions or roles inside the mucosa layer. Melanin is a common pigment in nature and is endowed with several peculiar chemical, paramagnetic, and semiconductive characteristics.

The emerging role of artificial intelligence in neuropathology: Where are we and where do we want to go?

The field of neuropathology, a subspecialty of pathology which studies the diseases affecting the nervous system, is experiencing significant changes due to advancements in artificial intelligence (AI). Traditionally reliant on histological methods and clinical correlations, neuropathology is now experiencing a revolution due to the development of AI technologies like machine learning (ML) and deep learning (DL). These technologies enhance diagnostic accuracy, optimize workflows, and enable personalized treatment strategies.

H3K27me3 Loss in Central Nervous System Tumors: Diagnostic, Prognostic, and Therapeutic Implications.

Central nervous system (CNS) tumors represent a formidable clinical challenge due to their molecular complexity and varied prognostic outcomes. This review delves into the pivotal role of the epigenetic marker H3K27me3 in the development and treatment of CNS tumors. H3K27me3, specifically the trimethylation of lysine 27 on the histone H3 protein, plays a crucial role in regulating gene expression and maintaining chromatin architecture (e.

AVIAN HAEMOSPORIDIANS IN GREATER SCAUP (AYTHYA MARILA) AND LESSER SCAUP (AYTHYA AFFINIS) FROM WISCONSIN.

Avian haemosporidians are a diverse group of protozoan parasites that infect a wide range of host species. Waterfowl are an ecologically and economically important group of hosts that have been underrepresented in studies of haemosporidians. Diving ducks have unique life history traits, and morphological, behavioral, and dietary differences separate them from more common dabbling ducks.

Glioblastoma mesenchymal subtype enhances antioxidant defence to reduce susceptibility to ferroptosis.

Glioblastoma (GBM) represents an aggressive brain tumor, characterized by intra- and inter-tumoral heterogeneity and therapy resistance, leading to unfavourable prognosis. An increasing number of studies pays attention on the regulation of ferroptosis, an iron-dependent cell death, as a strategy to reverse drug resistance in cancer. However, the debate on whether this strategy may have important implications for the treatment of GBM is still ongoing.

ESTRO-EPTN radiation dosimetry guidelines for the acquisition of proton pencil beam modelling data.

Proton therapy (PT) is an advancing radiotherapy modality increasingly integrated into clinical settings, transitioning from research facilities to hospital environments. A critical aspect of the commissioning of a proton pencil beam scanning delivery system is the acquisition of experimental beam data for accurate beam modelling within the treatment planning system (TPS). These guidelines describe in detail the acquisition of proton pencil beam modelling data.

Balancing benefits and limitations of linear energy transfer optimization in carbon ion radiotherapy for large sacral chordomas.

Background And Purpose: A low linear energy transfer (LET) in the target can reduce the effectiveness of carbon ion radiotherapy (CIRT). This study aimed at exploring benefits and limitations of LET optimization for large sacral chordomas (SC) undergoing CIRT.

Materials And Methods: Seventeen cases were used to tune LET-based optimization, and seven to independently test interfraction plan robustness.

A dosiomics approach to treatment outcome modeling in carbon ion radiotherapy for skull base chordomas.

Purpose: To investigate the role of dosiomics features extracted from physical dose (D), RBE-weighted dose (D) and dose-averaged Linear Energy Transfer (LET), to predict the risk of local recurrence (LR) in skull base chordoma (SBC) treated with Carbon Ion Radiotherapy (CIRT). Thus, define and evaluate dosiomics-driven tumor control probability (TCP) models.

Materials And Methods: 54 SBC patients were retrospectively selected for this study.

NTRK3-EML4-rearranged spindle cell tumor with co-expression of S100 and CD34: an unusual mesenchymal tumor in the spectrum of the bland-looking spindle cell lesions of the oral cavity.

A novel category of spindle cell tumors characterized by Neurotrophic Tyrosine Receptor Kinase (NTRK) rearrangements with a dual immunoreactivity for S-100 and CD34 has emerged in the last years as a distinct entity among soft tissue neoplasms. These genetic alterations lead to the continuous activation of NTRK genes, driving tumorigenesis and offering a unique prospect for targeted therapy. We herein present a rare case of NTRK3-rearranged spindle cell tumor with a hitherto unreported gene fusion involving the exon 14 of NTRK3 with the exon 2 of Echinoderm Microtubule-Associated Protein-Like 4, arising in the head and neck region.

OSLD nanoDot characterization for carbon radiotherapy dosimetry.

. This study characterized optically-stimulated luminescent dosimeter (OSLD) nanoDots for use in a therapeutic carbon beam using the Imaging and Radiation Oncology Core (IROC) framework for remote output verification..

Reversible dementia and insomnia in ABGA related encephalitis.

Background: ABGAs are historically associated with Encephalitis Lethargica (EL). Typically ABGAs are also found in children resulting in a variety of neuropsychiatric and extrapyramidal disorders, rare cases are reported in adults with atypical movement disorders. No description of basal ganglia reversible lesions related to ABGAs are reported and these antibodies are not included in the list of autoimmune encephalitis.

Robustness of carbon-ion radiotherapy against DNA damage repair associated radiosensitivity variation based on a biophysical model.

Background: Interpatient variation of tumor radiosensitivity is rarely considered during the treatment planning process despite its known significance for the therapeutic outcome.

Purpose: To apply our mechanistic biophysical model to investigate the biological robustness of carbon ion radiotherapy (CIRT) against DNA damage repair interference (DDRi) associated patient-to-patient variability in radiosensitivity and its potential clinical advantages against conventional radiotherapy approaches.

Methods And Materials: The "UNIfied and VERSatile bio response Engine" (UNIVERSE) was extended by carbon ions and its predictions were compared to a panel of in vitro and in vivo data including various endpoints and DDRi settings within clinically relevant dose and linear energy transfer (LET) ranges.

Status epilepticus: Is there a Stage 1 plus?

In status epilepticus (SE), "time is brain." Currently, first-line therapy consists of benzodiazepines (BDZs) and SE is classified by the response to treatment; stage 2 or established SE is defined as "BDZ-resistant SE." Nonetheless, this classification does not always work, especially in the case of prolonged convulsive SE, where many molecular changes occur and γ-aminobutyric acid signaling becomes excitatory.