Leiomyosarcoma of the inferior vena cava: survival after aggressive management

Leiomyosarcoma (LMS) of the inferior vena cava (IVC) is exceedingly rare. The role of adjuvant therapy remains undefined. This study evaluated outcomes after aggressive management.

Records on 20 patients undergoing surgery for IVC LMS between January 1990 and April 2006 were retrieved. Histology was confirmed upon re-review. Most patients received perioperative chemotherapy (CT), radiation therapy (RT), or both (CRT). Disease-free and overall survival (DFS, OS) rates were calculated using the Kaplan-Meier method.

Twenty patients (60% women, median age 57 years) with primary IVC LMS were treated with curative intent. Median follow-up was 41 months. All patients underwent resection of the primary tumor; one was found to have unresectable liver metastases. The IVC was managed with ligation (3), primary repair (12), or prosthetic graft (5). Additional organs were resected in 14 (70%) patients. Chemotherapy and/or RT were administered to 9 (45%) patients preoperatively (CT 2, RT 6, CRT 1) and 8 (40%) postoperatively (CT 4, RT 1, CRT 3). Median DFS was 21 months. Of 13 (68%) patients who developed recurrence, 4 underwent surgery, and 11 received CT. Median OS for 19 patients who underwent complete resection was 71 months. Tumor size was associated with disease recurrence (P = .004). No variables were prognostic for OS.

Patients with IVC LMS treated with curative intent develop early recurrent disease. Nevertheless, long-term OS can be achieved even in the setting of metastatic disease. The independent impact of perioperative CT, RT, or CRT treatments cannot be adequately determined.