Key result
Radical SVC resection with xenopericardial reconstruction shows no tumor recurrence at 8 months.
Why the study?
Primary leiomyosarcoma of the superior vena cava is an extremely rare and diagnostically challenging tumor lacking standardized diagnostic and therapeutic guidelines.
Case Report (n=1)
Radical surgical resection with xenopericardial graft reconstruction is a viable treatment for the exceptionally rare primary superior vena cava leiomyosarcoma, yielding favorable short-term outcomes without adjuvant therapy.
Supports short-term feasibility of radical SVC resection with xenopericardial reconstruction in leiomyosarcoma; leaves open long-term durability and adjuvant needs.
Leiomyosarcoma of the superior vena cava (SVC) is an exceptionally rare vascular tumor. Fewer than 20 cases were reported worldwide. Because of the nonspecific nature of the presenting symptoms and their deep mediastinal location, a pre-operative diagnosis is often difficult or impossible. A 63-year-old woman presented with progressive facial and upper-limb edema. Computed tomography (CT) demonstrated a hyper vascular intraluminal mass originating in the SVC and extending towards the right atrium. Percutaneous biopsy was considered unsafe due to the risk of hemorrhage. Therefore, radical surgical resection of the SVC and brachiocephalic veins with xenopericardial patch reconstruction was performed. Histological and immunohistochemical examination confirmed a grade two leiomyosarcoma. No adjuvant therapy was given. At 8-month follow-up, there was no evidence of recurrence. Primary SVC leiomyosarcoma is a rare and diagnostically challenging condition. Pre-operative biopsy is often not feasible, and radical
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Cygelnikov et al. (2026) conducted a case report in Primary Leiomyosarcoma of the Superior Vena Cava (n=1). Radical surgical resection with xenopericardial graft reconstruction was evaluated on Tumor recurrence at 8 months.
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