Management of intravenous leiomyomatosis: a case report illustrating two distinct surgical approaches

Abstract

Intravenous leiomyomatosis (IVL) is a benign smooth muscle growth originating in the uterus that extends into the lumen of venous or lymphatic vessels beyond the myoma. The tumour may enter the inferior vena cava (IVC) or the heart. For IVL with cardiac involvement, two distinct surgical approaches may be considered. The conventional approach involves concurrent intracardiac tumour resection via sternotomy, and resection of the intrabdominal/pelvic tumour by laparotomy, incision into the IVC, and a hysterectomy. Alternatively, an abdominal-only approach allows complete resection of the cardiac, abdominal, and pelvic portions of the IVL through IVC incision and hysterectomy. Considerations for surgical timing include a single-stage procedure, where all tumour components are addressed in one operation, or two-stage procedures, where cardiac and abdominal/pelvic components are resected in separate operations. Both approaches carry specific risks and benefits for the surgical course and patient recovery. We report two cases of patients presenting with symptomatic IVL. Patient A underwent a single-stage abdominal-only approach, including tumour removal from the IVC and hysterectomy, while Patient B underwent a two-stage surgical course involving initial intracardiac tumour resection via sternotomy, followed by a delayed subsequent abdominal tumour resection. We discuss the clinical decision-making process, benefits, and risks of both approaches, as well as preoperative and postoperative management considerations.