Cardiac tumors represent rare neoplasms, but they include a very wide range of neoplasia—first primary benign and malignant cardiac tumors, then cardiac metastases, with these latter ones being far more common in adulthood. These diagnoses may be challenging because of frequently non-specific signs and symptoms; for example, their clinical management may be difficult because of the site and because of possible hemodynamic or arrhythmogenic consequences, independent from their biology. Cardiac tumors may be asymptomatic and incidentally diagnosed, or they may cause heart failure, life-threatening arrhythmias, or even sudden cardiac death. Although they may still represent a post-mortem finding, the evolution and the larger use of cardiac imaging tools, initially echocardiography, has progressively and significantly increased their in vivo detection. Magnetic resonance imaging and computed tomography may give crucial information as to the composition and localization of cardiac masses, useful for investigating them and for planning surgery. Histology is mandatory for the definite and differential diagnosis of the cardiac masses, for assessing predictive factors in malignancies, and for then establishing the appropriate management of patients. Modern techniques applied to histology, including immunohistochemistry and molecular biology, may be required to characterize cardiac tumors, to properly classify them and to assess predictive and/or prognostic markers. Surgical procedures, including minimally invasive surgery, have also dramatically evolved in the last decades, allowing adequate treatment in most cardiac tumors. Finally, biopsy may be useful in selected cases, particularly when radical surgery is not feasible, and histological diagnosis is fundamental for other possible therapeutic approaches. The scope of this review covers advancements in the imaging diagnosis, histology, and treatment of primary and secondary cardiac tumors.

Background: Extraspinal sacrococcygeal ependymoma is a rare pediatric neoplasm that frequently presents as a superficial sacrococcygeal mass, closely resembling benign conditions such as pilonidal disease or cysts. As a result, preoperative diagnosis is often incorrect, leading to incomplete surgical excision and delayed clinical management. We conducted a comprehensive descriptive review of the pediatric literature to examine its epidemiology, clinical presentation, diagnostic challenges, pathological characteristics, treatment strategies, and outcomes. Materials and Methods: A structured literature search of PubMed, Scopus, and Web of Science from database inception to June 2026 was conducted. Only English studies reporting histologically confirmed extraspinal sacrococcygeal ependymoma in patients younger than 18 years were included. Discussion: Histologically, the vast majority of extraspinal sacrococcygeal ependymomas are of the myxopapillary subtype. Historically classified as grade 1 tumors by the World Health Organization (WHO), they were reclassified as WHO grade 2 in the 2021 WHO Classification of Tumors of the Central Nervous System. This change reflects the rising recognition of the clinically relevant risk of local recurrence and metastatic dissemination of these tumors, despite a relatively indolent growth pattern. Because extraspinal sacrococcygeal ependymomas may recur many years after initial treatment and occasionally metastasize to lymph nodes, lungs, liver, bone, or other distant sites, they should not be considered entirely benign lesions. Then, their management requires an oncological approach despite their often innocuous clinical appearance. Conclusions: Given the risk of delayed recurrence and distant metastasis, long-term follow-up is mandatory. Increased awareness of this singular entity, appropriate preoperative imaging of atypical sacrococcygeal lesions, and multidisciplinary management are essential to optimize outcomes. Future multicenter collaborative studies are needed to establish evidence-based recommendations for diagnosis, treatment, and surveillance.

Background: Extraspinal sacrococcygeal ependymoma is a rare pediatric neoplasm that frequently presents as a superficial sacrococcygeal mass, closely resembling benign conditions such as pilonidal disease or cysts. As a result, preoperative diagnosis is often uncorrect, leading to incomplete surgical excision and delayed clinical management. We conducted a comprehensive descriptive review of the pediatric literature to examine its epidemiology, clinical presentation, diagnostic challenges, pathological characteristics, treatment strategies and outcomes. Materials and Methods: A systematic search of PubMed, Scopus, and Web of Science from database inception to June 2026 was conducted. Only English studies reporting histologically confirmed extraspinal sacrococcygeal ependymoma in patients younger than 18 years were included. Discussion: Histologically, the vast majority of extraspinal sacrococcygeal ependymomas are of the myxopapillary subtype. Historically classified as grade 1 tumors by World Health Organization (WHO), they were reclassified as WHO grade 2 in the 2021 WHO Classification of Tumors of the Central Nervous System. This change reflects the rising recognition of the clinically relevant risk of local recurrence and metastatic dissemination of these tumors, despite a relatively indolent growth pattern. Because extraspinal sacrococcygeal ependymomas may recur many years after initial treatment and occasionally metastasize to lymph nodes, lungs, liver, bone, or other distant sites, they should not be considered as entirely benign lesions. Then, their management requires an oncological approach despite their often innocuous clinical appearance. Conclusions: Given the risk of delayed recurrence and distant metastasis, long-term follow-up is mandatory. Increased awareness of this singular entity, appropriate preoperative imaging of atypical sacrococcygeal lesions, and multidisciplinary management are essential to optimize outcomes. Future multicenter collaborative studies are needed to establish evidence-based recommendations for diagnosis, treatment, and surveillance.