Intravenous leiomyomatosis with intracardiac extension - A review of diagnosis and management with an illustrative case

Intravenous leiomyomatosis with intracardiac extension is an uncommon pathologic progression of uterine leiomyomata. It is a histologically benign condition, however due to interfence with right sided cardiac function patients may present with marked cardiovascular compromise and present a diagnostic dilemma to clinicians who are unfamiliar with this condition. Given the rarity of this condition, experience in individual institutions is usually limited to a few cases. We present an illustrative case and provide a review of the clinical presentation, preoperative assessment, operative approach, pathology and postoperative issues. The importance of a multidisciplinary approach to diagnosis and management is highlighted. Operative management aims to completely resect all tumour in the safest manner for the patient, most commonly via single or two stage operation. Where complete resection is achieved, recurrence appears to be a rare event.