Cardiac intima sarcoma: a rapid review of case reports

Background and purpose: Cardiac intimal sarcoma (CIS) is a rare and often misdiagnosed clinical entity with notably high morbidity and mortality rates. Urgent efforts are required to comprehensively the disease and establish evidence-based management guidelines. Given the relative scarcity of data and the imperative for consensus on management strategies, this review assumes critical importance. Through a rapid review approach, we aims to synthesize and consolidate information gleaned from published case reports, thereby offering an up-to-date resource for CIS’s natural history. Methods: Utilizing inductive content analysis, we systematically searched for and synthesized case reports and series. Keywords related to CIS guided our search on Medline via Ovid, Scopus, and Web of Science from inception until March 17, 2024. Results: Our review identified 79 CIS, characterized by a mean age of 52 years, predominantly male (58%), with left-sided lesions. Approximately one-third of the patients presented with cardiovascular symptoms, and transthoracic echocardiography emerged as the preferred initial diagnostic modality in most cases. Fluorescence in situ hybridization (FISH) analysis on pre-operative tissue biopsies found murine double minute 2 (MDM2) amplification in 95% of cases tested. While surgery remained the cornerstone of treatment, recurrence occurred in 14% of cases postoperatively, with 28% succumbing to the disease despite therapeutic interventions. Conclusions: This synthesis of published case reports and series provides updated insights into the clinical profile CIS. Our findings highlight the need for increased clinical awareness, precise data collection, and further research to enhance diagnostic accuracy, treatment approaches, and patient outcomes in CIS.