Radical Resection of a Primary Inferior Vena Cava Leiomyosarcoma: A Case Study

Background: Leiomyosarcomas (LMS) are rare soft tissue tumors that arise from smooth muscle. Of the five different classifications of LMS, the vascular LMS subtype has a predilection for major blood vessels, such as the inferior vena cava (IVC). Primary IVC LMSs affect approximately 1/100,000 people and have a very poor prognosis. They can lead to an array of symptoms that are often associated with other etiologies and can be fatal if not diagnosed accurately and treated appropriately. Case information: A 54-year-old Caucasian female presented to the hepato-pancreatico-biliary surgery clinic for assessment of a firm right upper quadrant abdominal mass. Upon further workup and imaging, a large mass within her IVC was found. A biopsy of the mass showed necrotic tissue that was non-diagnostic but concerning for malignancy. This lead to a successful radical surgical resection of the mass and adjacent structures with placement of an IVC graft. Additionally, chemotherapy and radiation was started to decrease chances of recurrence. Conclusion: Leiomyosarcomas of large blood vessels are at a greater risk of affecting associated organs based on their location and extent of vessel involvement. Though primary vascular LMSs are rare, they can be treatable if they are correctly identified early in the disease progression. This case demonstrates that careful radical resection of a primary IVC LMS should be considered as a treatment modality, in addition to chemotherapy and radiation, for patients with these rare and potentially fatal tumors.