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Case Reports in Oncological Medicine
Volume 2014 (2014), Article ID 193036, 4 pages
Case Report

Primary Angiosarcoma of the Spleen: An Oncological Enigma

1Department of Pathology, Tzaneio General Hospital, 185 36 Piraeus, Greece
22nd Department of Surgery, Aretaieion Hospital, 115 28 Athens, Greece
32nd Department of Surgery, Tzaneio General Hospital, 185 36 Piraeus, Greece

Received 28 March 2014; Revised 8 June 2014; Accepted 15 June 2014; Published 2 July 2014

Academic Editor: Ossama W. Tawfik

Copyright © 2014 Myoteri Despoina et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Introduction. Primary splenic angiosarcoma is an extremely unusual neoplasm originating from sinusoidal vascular endothelium. Surgical extirpation is the mainstay of treatment of this highly malignant disease. Case Presentation. An 82-year-old woman was admitted with left pleural effusion and a palpable left upper quadrant abdominal mass, secondary to splenomegaly by two large splenic tumors. Classic open splenectomy was performed and angiosarcoma of the spleen was the final histopathological diagnosis, which was primary since no other disease site was revealed. Discussion. The incidence of the disease is 0.14–0.23 cases per million, with slight male predominance. Etiology is not established and clinical presentation may confuse even experienced physicians. Imaging modalities cannot differentiate the lesion from other vascular splenic neoplasms and the correct diagnosis is mainly set after histopathological examination of the resected spleen. As with other sarcomas, surgery is the only curative approach, while chemo- and radiotherapy have poor results. Prognosis remains dismal.