Leiomyosarcoma of the Inferior Vena Cava: Surgical Management and Clinical Results JASON DEW, M.D., KIMBERLY HANSEN, M.D., JOHN HAMMON, M.D., THOMAS MCCOY, PH.D., EDWARD A. LEVINE, M.D., PERRY SHEN, M.D. From the Wake Forest University School of Medicine, Department of Surgery, Surgical Oncology, Vascular Surgery Service, and Cardiothoracic Surgery Service, Winston-Salem, North Carolina Leiomyosarcoma of the inferior vena cava (IVC) is a rare lesion with less than 300 cases reported. Optimal management and long-term outcomes are not well described. From August 1984 to June 2004, eight patients with leiomyosarcoma of the IVC were treated at our institution. Clinical and pathologic data, surgical management, and outcomes were assessed. Eight cases were identified (4 males) with a median age of 52 (range 29–66). Presenting symptoms included abdominal pain (n = 5, 63%), lower extremity edema (n = 2, 25%), and palpable mass (n = 2, 25%). Tumor location was between the renal and iliac veins (low) (n = 4, 50%), between the hepatic and renal veins (middle) (n = 3, 38%), and above the hepatic veins with right atrial extension (high) (n = 1, 12%). Two patients with preoperative IVC occlusion were managed with tumor excision and IVC ligation. Three patients had primary repair of the IVC after tumor excision. A polytetrafluoroth- ylene (PTFE) tube graft was used for IVC reconstruction in three cases. There was no postopera- tive mortality. Postoperative morbidity included deep venous thrombosis (DVT) (n = 1), lower extremity edema (mild n = 1; moderate n = 1), GI bleed (n = 1), and chronic renal insufficiency (n = 1). One patient is currently receiving adjuvant chemotherapy. Four patients received chemo- therapy after recurrence, and one received palliative radiation therapy as well. Median survival to this point was 60 months with a median follow-up of 39 months. The 5-year overall survival and disease-free survival was 31 per cent for both (CI 0.1–1.0). The type of IVC reconstruction had no effect on survival (P = 0.22). Recurrence was discovered in four patients (50%) at a median time of 14 months. Resection of leiomyosarcoma of the IVC should be attempted whenever feasible. The management of the IVC can be managed with primary repair, ligation, or prosthetic graft. Long-term survival is possible if complete resection can be achieved. P RIMARY VASCULAR TUMORS are rare entities; sarco- mas are the most common type. Veins are in- volved more frequently than arteries, and the inferior vena cava (IVC) is the most frequently involved vein. 1 Still, leiomyosarcoma of the vena cava is an unusual tumor with less than 300 cases reported in the English language literature. 1–6 Most information regarding treatment and outcome of IVC leiomyosarcoma comes from single case re- ports or small series. Thus, optimal treatment strate- gies are unclear. Complete surgical resection with negative margins offers the only chance at long-term survival. 2 The role of adjuvant therapy is controver- sial. Chemotherapy has been used with no clear evi- dence of efficacy. Some reports suggest that radiation therapy may be beneficial. 2, 5 To improve understand- ing of this disease, we reviewed the experience with IVC leiomyosarcoma at our institution. Patients and Methods We reviewed eight cases of IVC leiomyosarcoma treated at Wake Forest University Baptist Medical Center (WFUBMC) from August 1984 to January 2005. Clinical and pathologic data, surgical manage- ment, and outcomes were assessed. This study was approved by our institutional review board. Cases were identified by case logs of surgeons and with the assistance of the North Carolina Baptist Hospital Tu- mor registry. Symptoms were evaluated via review of the relevant medical records. Operative notes and pathology re- ports were used to collect tumor size, location, and grade as well as to determine organs resected en bloc and postresection IVC management. Postoperative in- patient and follow-up clinic notes were used to evalu- ate administration of adjuvant or subsequent palliative Presented at the Annual Scientific Meeting and Postgraduate Course Program, Southeastern Surgical Congress, New Orleans, LA, February 11–15, 2005 Address correspondence and reprint requests to Perry Shen, M.D., Surgical Oncology, Wake Forest University, Medical Center Blvd., Winston-Salem, NC 27157. 497