Leiomyosarcoma of infrarenal inferior vena cava: A single institution experience and review of literature

We report three cases of primary leiomyosarcoma (LMS) of inferior vena cava (IVC). Vascular LMSs are rare tumors, arising most frequently from IVC. These tumors have a female predominance. Their diagnosis is often challenging, as patients may present with nonspecific complaints such as dyspnea, malaise, weight loss, abdominal pain, or back pain, preceding the diagnosis by several years. LMS of the IVC most frequently occurs in the middle segment. The final diagnosis can be made by an ultrasound or computed tomography-guided biopsy. Due to limited experience with this disease, optimal management of IVC LMS is unknown. Curative surgical resection remains the current treatment of choice for primary LMS of IVC. Neoadjuvant therapy may be given to downsize the tumor and increase resectability rates. Nonetheless, there is no proven role for adjuvant therapy, and recurrence is common. We, hereby, report three cases of this rare entity with emphasis on management.