Background: Primary intraluminal leiomyosarcoma of the inferior vena cava (IVC) is extremely rare. Diagnostic imaging plays an important role in the diagnosis and conducting treatment strategy.
Methods: MRI with contrast-enhanced MR venography was performed in six patients with primary intraluminal leiomyosarcoma of the IVC.
Results: In the six patients, three had leiomyosarcoma of the middle IVC, and three the lower IVC. The mean axial diameter of tumor was 4.3 cm. MRI with contrast-enhanced MR venography not only demonstrated the tumor's location, morphology, extent, and collateral vessels but also distinguished tumor from thrombus of the IVC. Three patients underwent surgery and three did not. Four patients (three with surgery and one without surgery) were alive during the following periods, but one presented with local recurrence and all with metastases at a median time of 23 months. One patient died at 15 months after the diagnosis with liver and subcutaneous metastases, one lost to follow up.
Conclusion: MR image with contrast-enhanced MR venography is a noninvasive and accurate technique for diagnosis of primary intraluminal leiomyosarcoma of IVC. Its providing information is very helpful for pre- and post-surgery. It can be used as one of the first choice examinations.