Diagnosis and surgical management of inferior vena cava leiomyomatosis

Objective: We aimed to review our experience in the diagnosis and surgical management of patients diagnosed with inferior vena cava leiomyomatosis (IVL). Methods: We retrospectively evaluated all patients diagnosed with IVL between 1999 and 2015. Patient demographics, diagnostic imaging, operative techniques, and perioperative outcomes were reviewed. Results: Over the study period, 16 patients with an IVL diagnosis were identified. In all patients, the diagnosis was made with ultrasound and magnetic resonance imaging. In 15 patients who underwent operative intervention, we observed three tumor extension routes from the uterus to the inferior vena cava: (i) via the internal iliac vein, (ii) via the ovarian vein, and (iii) via the anterior sacral vein. Complete tumor removal was achieved in all patients who underwent a one-stage operation (12 patients). Among these patients, antegrade tumor extraction from the right atrium was performed in nine patients, and retrograde extraction from iliac veins was performed in three. A two-stage operation with direct tumor transection and resection was necessary in a subset of patients to facilitate complete resection in one patient, and near-complete resection in two patients. Preoperative imaging and intraoperative findings demonstrated four distinct types of gross tumor morphologies: (i) type A solid cast (43.8%), (ii) type B hallow tube-like (12.5%), (iii) type C thread-like (18.7%), and (iv) type D mixed morphology (25%). Types A and B were the easiest tumor types to extract, and types C and D tumors were more difficult to remove given their fragility. Postoperative surgical pathology confirmed the diagnosis of IVL. All patients recovered successfully with no major complications; there were no deaths. One patient early in our experience had an incomplete resection and developed a recurrence that required re-intervention at 26 months from the initial operation. Conclusions: IVL can be accurately diagnosed with ultrasound and magnetic resonance imaging. Surgical tumor resection with a one-stage operation can lead to reasonable outcomes and successful cure rates. The surgical plan can be tailored to the type of tumor morphology observed on preoperative imaging.