Intravenous leiomyomatosis is a rare nonmalignant tumor, which originates from the uterine smooth muscle cells and is usually confined to the pelvic venous system. Sometimes it can extend from the pelvis through the veins into the right side of the heart; this condition is named intracardiac leiomyomatosis (ICLM). To date few cases of these conditions have been described, the treatment is surgical, often challenging and usually multidisciplinary. In this paper are described the clinical presentation, the full radiologic study and surgical treatment of a case of ICLM that authors treated at their institution with thoraco-abdominal approach. Surgical removal of the ICLM is strongly recommended, because no recurrence has been reported, in our case at 7 years we did not observe recurrence of the disease.
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