Congenital brain tumors (CBTs) are neoplasms detected during fetal life or early neonatal life. These are exceedingly rare and account for 0.5% to 1.5% of all pediatric brain tumors. CBTs are histologically distinct and clinically more aggressive than other pediatric brain tumors. Advances and increased availability of neuroimaging has enabled early and accurate detection of these lesions and associated complications, allowing clinicians to manage pregnancy and neonatal care and better plan and prepare for interventions in advance. In this article, we provide an overview of the incidence, pathogenesis, and clinical presentation of some common CBTs along with their imaging manifestations.

Tumors of the sellar, suprasellar, and parasellar regions are commonly encountered in pediatric neuroimaging. The regional anatomy is complex, and lesions of developmental, vascular, inflammatory/infective, and neoplastic origin occur. The 2021 WHO Classification of Tumors of the Central Nervous System recognized adamantinomatous craniopharyngioma and papillary craniopharyngioma as distinct tumor types, included pituitary blastoma as a new tumor, and included the terminology pituitary neuroendocrine tumor alongside pituitary adenomas. This article outlines the pediatric tumors of the sellar, suprasellar, and parasellar regions and describes their imaging appearance and the main non-neoplastic differential diagnoses.

The last decade has seen major advances in our understanding of the biology of pediatric brain and spinal cord tumors and why they are fundamentally different from their adult counterparts. Many of these advances have been adapted into the latest WHO classification of tumors of the central nervous system. This article summarizes the major changes to the WHO classification in this area, highlighting the key histopathologic and molecular features of these new entities.

The Na-MRI technique from acquisition to postprocessing provides many opportunities and potential advantages or augmentations over H-MR imaging alone, and over prior preliminary Na-MRI approaches. The current implementation provides improved contrast and resolution over prior Na-MRI, partially due to the twisted projection imaging acquisition and anatomically guided reconstruction reconstruction. Importantly, the sequence is feasible to be obtained in a clinical setting in patients with brain tumors. The preclinical models allow for further exploration of sodium concentrations in tumor models during therapy. Neuro-oncology requires improved imaging biomarkers that are more reflective of tumor biology.

The 5th edition of the WHO CNS tumor classification (2021) emphasizes molecular alterations, especially in pediatric tumors, integrating histology with molecular profiling for precise diagnosis. Advances like DNA methylation profiling and Next Generation Sequencing have refined tumor subtypes, influencing targeted therapies. Radiogenomics correlates imaging features with genetic profiles, enabling non-invasive tumor characterization, crucial in pediatric cases where biopsies are risky. Artificial intelligence, including machine learning and deep learning, enhances image analysis, segmentation, and prediction of molecular markers, supporting personalized treatment. Despite challenges like data variability and ethical concerns, these technologies promise to revolutionize pediatric neuro-oncology.

Advances in molecular and genomic testing have not only discriminated pediatric from adult high grade gliomas but also distinguished new entities in this family, 3 of which are new to the classification. We have reviewed the published literature to date and present the characteristic and most common findings of each type on structural MR imaging, as well as advanced imaging, where available. Prognostic features, particularly in diffuse midline gliomasK27a have also been described, in addition to practice recommendations.

Pediatric diffuse low-grade gliomas (LGGs) are heterogeneous group of central nervous system tumors that typically exhibit a relatively benign clinical course. These tumors represent a unique classification in pediatric neuro-oncology, distinct from adult counterparts in terms of biological behavior, molecular pathways, and clinical presentation. The evolving World Health Organization classification system has increasingly relied on combination of histopathologic, genetic, and radiologic criteria to define and distinguish among pediatric glioma subtypes. This article aims to synthesize current knowledge regarding the clinical, molecular, and radiologic features of these pediatric diffuse LGGs and highlights the nuances in diagnosis, treatment approaches, and prognostic outlook.

Glioneuronal tumors are rare central nervous system neoplasms characterized by mixed neuronal and glial components, predominantly affecting pediatric and young adult populations. This article provides an in-depth analysis of glioneuronal tumors, emphasizing neuroimaging findings critical for diagnosis, differential diagnosis, and surgical planning. MR imaging remains the cornerstone, with advanced techniques like perfusion and functional MR imaging offering additional insights. Prognosis is generally favorable for low-grade tumors, though high-grade variants pose challenges. Innovations in radiomics, molecular profiling, and personalized medicine are transforming management. This article synthesizes current knowledge, highlighting imaging's pivotal role, and future directions.

The 2021 WHO Brain Tumor classification update grouped ependymal tumors into 3 anatomic locations-supratentorial, posterior fossa, and spinal. Supratentorial ependymomas now include ZFTA-fusion tumors which are often cystic, hemorrhagic, and show marked edema and YAP1-fusion tumors which demonstrate garlanded peripheral solid enhancement and diffusion-restriction. Posterior fossa ependymomas are now grouped by methylation profile into category A-typical posterior fossa ependymoma and category B which present in older children with smaller lesions with a better prognosis. Spinal ependymoma is relatively unchanged by molecular characterization apart from the new MYCN-altered tumor which has poor prognosis and is often advanced/disseminated at presentation.

Central nervous system (CNS) embryonal tumors are a subset of aggressive pediatric brain tumors, more frequently seen in infants and young children. They share an aggressive imaging appearance with frequent disseminated disease and guarded prognosis; however, patient age, tumor location, and imaging characteristics such as enhancement pattern and peritumoral edema can help with differential diagnosis. In this article, we have reviewed medulloblastoma, atypical teratoid rhabdoid tumor, embryonal tumor with multilayered rosettes as well as newly recognized CNS neuroblastoma, FOXR2-activated and CNS tumor with BCOR internal tandem duplication.

Published in 2021, the 5th edition of the World Health Organization central nervous system tumor classification introduced major changes to the categorization of pediatric brain and spinal cord tumors. Greater focus has been placed on the integration of molecular genetics, highlighting its emerging importance in the diagnosis, prognostication, and targeted therapy of these tumors. New molecularly defined tumors, reclassification of existing tumors, and revised nomenclature are discussed in this article, and subsequent articles provide further information on this new central nervous system tumor landscape in pediatric neuroradiology.

Tumor predisposition syndromes (TPS) are inherited cancer syndromes linked to approximately 10% of all cancers and up to 20% pediatric central nervous system (CNS) tumors. TPS manifest with characteristic patterns of neoplasms throughout the body and require targeted, region-specific imaging for surveillance. Advances in cancer genomics have allowed options for therapies targeting specific molecular pathways. This article highlights CNS imaging features of TPS, with additional insights into the underlying genomic alterations that lead to their development.

Tumors of the temporomandibular joints (TMJ) are rare, and therefore radiologists may have limited experience with their interpretation. This article presents the most common benign and malignant intrinsic tumors that may arise in the TMJ. Clinical features are presented since these may be reported by the referring clinician or be evident on imaging (shift of jaw, open bite, and swelling). Key imaging features that can help with tumor recognition are outlined.

This article covers congenital and developmental conditions of the temporomandibular joint (TMJ), including Pierre Robin sequence, Treacher Collins syndrome, oculoauriculovertebral spectrum, and various forms of condylar dysplasia such as condylar hypoplasia and hyperplasia. Hemimandibular elongation, hemimandibular hyperplasia, and coronoid hyperplasia are also discussed. Emphasis is placed on the imaging characteristics that aid in diagnosis and management, highlighting the critical role of advanced imaging in evaluating TMJ abnormalities, guiding treatment planning, and monitoring growth and development in affected individuals.

Radiolucent and radiopaque lesions of the temporomandibular joints (TMJs) mimicking cysts and tumors are rare. Some lesions in the TMJ complex are intraosseous, while others are in the joint spaces. Intraosseous lesions include cyst-like lesions, for example, subcortical pseudocysts, aneurysmal bone cysts, and simple bone cysts. Lesions in the joint spaces include low-density synovial and ganglion cysts and calcified entities such as synovial chondromatosis and calcium pyrophosphate deposits. This article discusses common cyst-like and tumor-like conditions encountered in the TMJs and their imaging features. The advantages and limitations of the imaging techniques are briefly discussed.

Artificial intelligence (AI) represents a significant advancement in the workflow of clinicians and radiologists within the field of dentomaxillofacial radiology, offering both current contributions and potential benefits. This article provides an overview of efforts to develop AI tools designed to assist in the interpretation of the temporomandibular joint-an anatomically complex structure in the dentomaxillofacial region that often necessitates advanced imaging methods for diagnosis and treatment planning. The outcomes of these studies are discussed, with a focus on the advantages of AI across various imaging modalities. Additionally, the article examines the diagnostic and treatment planning benefits AI offers.

In this article, we address the imaging considerations for both closed and open temporomandibular joint (TMJ) surgeries, as well as non-TMJ procedures that indirectly affect TMJ function. Techniques, such as arthrocentesis, disc repositioning (discopexy), condylectomy, eminectomy, total joint replacement, autogenous bone grafting, and various discectomy approaches, are explored. Pre-operative and post-operative MR imaging, computed tomography (CT), and cone beam CT protocols are highlighted, emphasizing the distinction between normal healing and pathologic changes (eg, disc re-displacement, graft failure, and hardware issues). Overall, this article equips radiologists to correlate surgical procedures with imaging findings, enabling timely diagnosis and improved patient outcomes.