Intravenous Leiomyomatosis in a 20-Year-Old Nulliparous Woman: Atypical Presentation and Radiological Investigation

Intravenous leiomyomatosis (IVL) is an unusual benign tumor of the uterine smooth muscle, which grows intravascularly. It can spread through the inferior vena cava and into the right heart. We present the case of a 20-year-old female patient who presented with chronic pelvic pain and a background of uterine fibroids. Imaging demonstrated a large pelvic mass with an apparent extension via the right gonadal vein into the inferior vena cava and subsequently into the right atrium and ventricle, consistent with IVL. Despite significant cardiac involvement which resulted in a sizeable mass occupying the pulmonary thoracic cavity, the patient had no evidence of cardiopulmonary symptoms that might be anticipated from the anatomical findings. Diagnosis was made using contrast-enhanced computed tomography. The patient was referred for surgical resection with multidisciplinary support. This case highlights the role of imaging in making the diagnosis of IVL in a timely manner to facilitate surgical management, particularly in younger patients who present in this atypical fashion.