Document Type : Case Report(s)
Authors
- Andrey Ryabov 1
- Stansilav Cygelnikov 1
- Oleg Pikin 1
- Oleg Aleksandrov 2
- Vladimir Bagrov 1
- Aleksandr Gusev 1
- Evgeniy Toneev 3
- Viktoriya Surkova 1
- Aznaur Dotdaev 1
1 P. Hertsen Moscow Oncology Research Institute (MORI), Branch of the Federal State Budgetary Institution “National Medical Research Radiological Centre” (NMRRC), Ministry of Health of the Russian Federation, Moscow, Russian Federation
2 University College London Hospitals NHS Foundation Trust (UCLH), London, United Kingdom
3 Department of Hospital Surgery, Anesthesiology, Reanimatology, Urology, Traumatology, and Orthopedics, T.Z. Biktimirov Faculty of Medicine, Institute of Medicine, Ecology and Physical Culture, Ulyanovsk State University, 42 Lev Tolstoy St., Ulyanovsk, 432071, Russian Federation
Abstract
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 en bloc resection with vascular reconstruction remains the cornerstone of treatment, offering the best chance of recurrence‑free survival. Histopathological confirmation is required to establish the diagnosis, and the role of adjuvant therapy remains uncertain.
Highlights
Andrey Ryabov (Google Scholar)
Oleg Aleksandrov (Google Scholar)
Keywords
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