Leiomyosarcoma of the inferior vena cava: survival after aggressive management.

BACKGROUND: 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.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.