Abstract 1933P
Background
Leiomyosarcoma of the vena cava (LMS-VC) is a rare sarcoma with poor oncological outcome. The role of perioperative chemotherapy (CT) and/or radiotherapy (RT) remains unclear.
Methods
All consecutives cases of pathologically confirmed LMS-VC resected between 2003 and 2022 in two tertiary referral centers were retrospectively collected. Perioperative treatments were discussed in a multidisciplinary tumor board for large tumors with/without organ involvement. CT were based on adriamycin regimens. The surgical strategy aimed to obtain a complete en-bloc resection of the LMS-VC. We evaluated disease-free survival (DFS) and overall survival (OS) using Kaplan-Meier estimates with a Cox model.
Results
Forty-one patients were identified, with a median age of 57.5 years. LMS-VC median size was 9.5 cm (interquartile range [6.8-12.5] cm) with 67.5% of complete obstruction (no outflow at CT scan). The Kieffer levels (K) were K1, K2 and K3 in 9(22%), 23(56%) and 9(22%) patients respectively. At surgery, 55% had a nephrectomy and 20% a minor hepatic resection. Vena cava was replaced by prosthetic graft in 40% of patients and 32.5% had no reconstruction. Severe complications (Clavien-Dindo≥3) occurred in 12(30%) cases. No postoperative mortality was reported. R0 was obtained in 29(71%) patients, R1 in 11(27%) and one patient had a R2 resection. FNCLCC grades were 1, 2 and 3 in 5(12%), 26(63%) and 10(24%) cases respectively. Twelve (30%) patients received CT with 10 and 2 in pre and postoperative setting respectively, only in FNCLCC grade 2 or 3. Five (12%) patients received adjuvant RT. After a median follow-up of 70 months, median DFS was 22 months and median OS was 55 months. Among the 27 recurrences, 24 were distant. The multivariate analysis identified FNCLCC grade (Hazard Ratio (HR), 20; 95% confidence interval [CI], 2.29 to 175 for grade 2 vs 1 and 65.6; 95% CI, 6.30 to 683 for grade 3 vs 1; p<0.001) and pre and/or postoperative chemotherapy (HR, 0.38; 95% CI, 0.16 to 0.92; p=0.026) as main prognostic factors.
Conclusions
LMS-VCs have high risk of distant recurrence, evaluated as 58% in this cohort. Pre and/or postoperative chemotherapy and FNCLCC tumor grade were associated with improved DFS.
Clinical trial identification
Editorial acknowledgement
Elie Fadel and Axel Le Cesne have contributed equally to this abstract.
Legal entity responsible for the study
Department of Surgical Oncology, Institut Gustave Roussy, Cancer Center, 114, rue Edouard Vaillant, 94805 Villejuif, France Departement of Thoracic and Vascular Surgery and Heart–Lung Transplantation, Hôpital Marie-Lannelongue (ParisSud University) 133 Avenue de la Résistance Le Plessis Robinson France.
Funding
Has not received any funding.
Disclosure
All authors have declared no conflicts of interest.
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