INTERNATIONAL REGISTRY OF INFERIOR VENA-CAVA LEIOMYOSARCOMA - ANALYSIS OF A WORLD SERIES ON 218 PATIENTS

In 1992 The International Registry of Inferior Vena Cava (IVC) Leiomyo sarcomas was established to study the pathogenesis and natural history of the tumor and to support the most rational treatment. We collected 218 patients through a literature review and personal communications. We corresponded with several Authors to obtain up-to-date follow-up a nd any other data lacking at the initial review. The series was analyz ed to identify predictive factors for clinical outcome. Tumors arose f rom the IVC lower segment in 80 patients, from the middle in 94 and fr om the upper in 41. A radical tumor resection was undertaken in 134 (6 1.5%) patients, 26 (11.9%) had a palliative resection and 58 (26.6%) w ere inoperable. An increased risk of death was associated with upper I VC segment involvement (p<0.001), lower limb edema (p<0.001), Budd-Chi ari's syndrome (p<0.001), intraluminal tumor growth (p<0.001) and IVC occlusion (p<0.001). Radical tumor resection was associated with bette r 5- and 10-year survival rates (49.4% and 59.5%). Tumors which arose from the middle segment fared better (56.7% and 47.3%) than those of t he lower segment (37.8% and 14.2%) (p<0.002). No palpable abdominal ma ss and abdominal pain were associated, in patients radically operated, with a better outcome and longer survival (p<0.03 and p>0.04 respecti vely). Despite the high rate of recurrence, radical tumor resection is the only long-term cure.