Purpose of Review: Pediatric renal tumors comprise a wide range of conditions, both malignant and benign, that affect children and require a multidisciplinary approach for optimal diagnosis and treatment. This review offers an in-depth analysis of the epidemiology, diagnosis, treatment options, outcomes, and survival of major pediatric renal tumors. Recent Findings: Wilms tumor, or nephroblastoma, is the most common form of renal tumor in children, characterized by growth from primitive renal cells. Standard treatment involves a combination of surgery, chemotherapy and, in some cases, radiation therapy, with the aim of removing the tumor, preventing recurrence and maximizing the chances of long-term recovery. Less common pediatric renal tumors, such as renal clear cell sarcoma, renal cell carcinoma, mesoblastic nephroma, and malignant rhabdoid tumor, require similarly careful and individualized management. Therapeutic strategies, which depend on the characteristics of the tumor, the stage of the disease and the individual response to therapy, may include surgery, chemotherapy, radiotherapy and, in some cases, molecular targeted therapies, immunotherapies and genetic and epigenetic therapies. Summary: The management of pediatric kidney tumors requires the involvement of a multidisciplinary team of specialists to ensure accurate evaluation, optimal treatments and long-term follow-up. The aim is to maximize the prospects for recovery and improve the quality of life of patients and their families. Advances in innovative, personalized therapies represent an important opportunity to further improve clinical outcomes in these patients.

Sudden cardiac death (SCD) is defined as unexpected death due to a cardiac cause that occurs rapidly. Despite the identification of prevention strategies, SCD remains a serious public health problem worldwide, accounting for 15–20% of all deaths, and is therefore a challenge for modern medicine, especially when it affects young people. Sudden cardiac death in young people affects the population aged ≤ 35 years, including athletes and non-athletes, and it is due to various hereditary and non-hereditary causes. After an autopsy, if the cause remains unknown, it is called sudden unexplained death, often attributable to genetic causes. In these cases, molecular autopsy—post-mortem genetic testing—is essential to facilitate diagnostic and therapeutic pathways and/or the monitoring of family members of the cases. This review aims to elaborate on cardiac disorders marked by genetic mutations, necessitating the post-mortem genetic investigation of the deceased for an accurate diagnosis in order to facilitate informed genetic counseling and to implement preventive strategies for family members of the cases.

Teratomas are neoplasms arising from germ cells and encompass tissues derived from two or more embryonic germ layers, including ectoderm, mesoderm, and endoderm. These tumours typically localize along the midline or in paramedian positions and can manifest as gonadal (20%) or extragonadal (80%) entities. Although gonadal teratomas are uncommon, they represent the predominant type of gonadal tumour in the paediatric population. They comprise approximately 20–25% of all ovarian tumours in females and about 3–5% of all testicular tumours in males. Ovarian teratomas exhibit a higher incidence in early childhood and adolescence, whereas testicular teratomas are more prevalent during the first three months of life and between the ages of 15 and 19. While the majority of paediatric gonadal teratomas are benign, malignant or mixed variants may also arise, necessitating more aggressive therapeutic interventions.

Introduction: Sudden Cardiac Death (SCD) is one of the main causes of death in the world, with a significant impact especially on young people. Sudden Cardiac Death in the Young (SCDY) is characterized by multifactorial etiology, which includes cardiomyopathies, myocarditis, channelopathies, aortopathies and coronary artery diseases. Despite progress in prevention, a significant percentage of these deaths remain unexplained without a thorough autopsy. This study aims to SCDY cases registered between 2016 and 2024, exploring the association between type of autopsy, age, sex, causes of death and temporal changes.Methods: Data relating to subjects who died for suspected SCDY, who underwent forensic, or hospital autopsy were retrospectively analysed. Investigations included type of autopsy (diagnostic or judicial), age (in years), sex, available clinical data, gross and histological findings, and cause of death. The data were divided by age groups (0–10, 11–20, 21–30, 31–40 years), sex and cause of death (arrhythmias, congenital heart defects, myocarditis, vascular dissections and cardiomyopathies). The temporal distribution of cases was also evaluated.Results: A total of 62 cases were analysed, with a prevalence of male subjects (70%). Forensic autopsies (65%) were more frequent than diagnostic findings (35%). The most represented age groups were 11–20 years (30%) and 21–30 years (25%). Unknown arrhythmias were the main cause (40%), followed by congenital heart disease (20%) and cardiomyopathy (15%). Congenital heart defects prevailed in newborns and children, while hypertrophic or arrhythmogenic cardiomyopathies were more frequently observed in young adults. Temporally, there has been a progressive increase in molecular autopsies and genetic diagnoses, in particular after the introduction of the AECVP (2017) and SCVP (2023) guidelines.Discussion: The findings highlight the need for a multidisciplinary approach to diagnosis of SCDY, with particular emphasis on molecular autopsy to identify genetic causes. The male predominance and age-related etiological differences underline the importance of specific preventive strategies, such as genetic screening in newborns and victims’ relatives. The increase in diagnoses over time reflects the effectiveness of updated guidelines, but it remains crucial to expand the mandatory nature of autopsies to improve understanding of the causes of SCDY and reduce the incidence of these tragic events.

Hereditary aortopathies are a group of rare genetic diseases affecting the aorta and its major branches, and they represent a cause of sudden cardiac death. These pathologies are classified into syndromic hereditary aortopathies and non-syndromic hereditary aortopathies. The epidemiology of hereditary aortopathies varies according to the specific genetic condition involved; however, these disorders are believed to account for a significant proportion of sudden cardiac death in young individuals with a family history of inherited cardiovascular conditions. The causes of hereditary aortopathies are primarily genetic, with pathogenic variants in various genes encoding structural proteins of the vascular wall, leading to dissection, aneurysms, rupture, and ultimately sudden cardiac death. When the cause of death remains unknown after an autopsy, it is referred to as sudden unexplained death, and post-mortem genetic testing, known as a molecular autopsy, is crucial to confirm hereditary aortopathies and assess the genetic risk in the patient’s relatives. This helps to facilitate diagnostic and therapeutic pathways and/or implement monitoring strategies to prevent sudden cardiac death. In this state-of-the-art review, we focus on syndromic and non-syndromic hereditary aortopathies causing sudden cardiac death in the young and explore preventive strategies for affected family members.

Cardiac tumors (CTs), although rare, can be a significant cause of sudden cardiac death (SCD), particularly when not diagnosed early. The tumors most associated with SCD include myxomas, fibromas, rhabdomyomas, and sarcomas, which can cause fatal arrhythmias, blood flow obstruction, or embolization. Myxomas, which often develop in the left atrium, can cause valvular obstruction or cerebral emboli, while malignant tumors, such as sarcomas, can infiltrate the myocardium or conduction system, causing serious arrhythmias. Rhabdomyomas, which are common in children and associated with tuberous sclerosis, can lead to ventricular tachyarrhythmias. Early diagnosis using advanced imaging techniques such as echocardiography and magnetic resonance imaging is crucial for preventing SCD. Timely diagnosis and precise characterization of lesions can help reduce the risk of SCD, thus improving the clinical management of patients, with the aim of supporting personalized treatment and improving life prospects. In this state-of-the-art review, we analyze the association between CTs and SCD, with particular attention to the histological features of benign and malignant neoplasms. Through an updated overview of the pathological aspects, we aim to improve the understanding of these tumors and promote a more effective multidisciplinary diagnostic and therapeutic approach to prevent fatal events.