Multimodal approach of leiomyosarcoma of the inferior vena cava: case report and literature review of main points of controversy

The leiomyosarcoma of inferior vena cava (LMS/IVC) is a rare malignancy with poor prognosis. Multidisciplinary approach at referral centers is essential to achieve best survival outcomes. Our aim was to add two cases of LMS/IVC treated with different surgical techniques and to review controversy points throughout the literature. We present two cases treated with preoperative external-beam radiotherapy and negative margin surgery with intraoperative radiotherapy prior discussion at the multidisciplinary tumor board (MTB). Patient-1: 57 year old woman diagnosed with a primary type II LMS/IVC. A multivisceral en-bloc excision of the mass, middle IVC segment, right- psoas muscle, nephrectomy and colectomy was performed. Collateral venous drainage allowed IVC simple ligation. No postoperative complications occurred. Pathology revealed a LMS/IVC grade-3 of the FNCLCC with negative margins. Postoperative systemic chemotherapy was decided at the MTB. The patient remains asymptomatic and disease-free. Patient-2: 46 year old woman with a 4cm local recurrence of a FNCLCC grade-2 LMS/IVC after R1-surgery. Surgical rescue consisted of an en-bloc excision of the mass and middle IVC segment. Reconstruction was performed with a 20mm-ringed polytetrafluoroethylene graft (GORE-TEX (R)) alongside reimplantation of left renal vein. Postoperative period was complicated with a low-output chyle leak. Pathology revealed negative resection margins, but radiologic imaging at 6-months suggested recurrent disease versus graft infection. R0-resection is the cornerstone of LMS/IVC curative treatment, though a multimodal approach at referral centers is essential to achieve better survival outcomes. Surgical approach will depend on the level IVC involvement and the presence of venous collaterals.