Bizarre intravascular leiomyoma with intracardiac extension starting in the ovarian vein: a case report from Syria

Abstract Intravascular leiomyoma (IVL) with intracardiac extension (ICE) represents an exceedingly rare diagnosis of a cardiac mass. We present the case of a 42-year-old woman with recurrent syncopal episodes. Cardiac investigations revealed an extensive, mobile mass stretching from the inferior vena cava (IVC) through the right heart to the bifurcation of the pulmonary artery. Emergent surgery was conducted to excise the mass. Post-operative assessment indicated a potential malignancy in the adnexa. A subsequent surgery to resect the uterus with the adnexa, the primary origin of the mass, confirmed the diagnosis of IVL with ICE. The initial diagnostic ambiguity and the urgent pulmonary artery involvement necessitated a two-step surgical approach. Despite the propensity for recurrence, a 5-year follow-up remained unremarkable. This case underscores the importance of considering IVL with ICE in the differential diagnosis, which can expedite both diagnosis and treatment.