Table 2.

Summary table of Leiomyosarcoma of the IVC.

Etiology	No known cause. The majority of reported cases in the literature are sporadic.
Incidence	LMS of the IVC accounts for 5% of all vascular LMS. Vascular LMS tumors represent about 1–2% of all LMS, which accounts for 10–20% of all sarcomas. Sarcoma represents 1% of all adult malignancies.
Symptoms	Most patients present with nonspecific right upper quadrant pain.
Gender and Age	3:1 female to male ratio with a mean age of 54 years.
Treatment	Surgical treatment is preferred if localized to the wall of the IVC. Chemotherapy is the mainstay of treatment for cases with metastatic disease.
Prognosis	The 5-year and 10-year survival rates after surgical resection have been reported to be 31.4% and 7.4%, respectively, with 0% survival observed with incomplete resection or inoperable cases, with or without the chemoradiation therapy.
Findings on Imaging	On CT, LMS of the IVC usually appears as heterogeneously enhancing mass with central necrosis and calcification in the retroperitoneum involving the perihepatic IVC, sometimes with mass effect on surrounding organs. On MRI, LMS of the IVC usually demonstrates a soft tissue mass involving the IVC, which is homogeneously hypointense on T1, intermediate T2 signal intensity, and progressive avid enhancement on post-contrast T1 sequence. On US, LMS of the IVC appears as heterogeneous hypoechoic retroperitoneal mass with internal color flow.