title: Primary angiosarcoma of the spleen: an oncological enigma.
abstract: 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.
authors.forename: Despoina
authors.initials: D
authors.lastname: Myoteri
authors.affiliation: Department of Pathology, Tzaneio General Hospital, 185 36 Piraeus, Greece.
authors.forename: Myoteri
authors.initials: M
authors.lastname: Despoina
authors.forename: Dionysios
authors.initials: D
authors.lastname: Dellaportas
authors.affiliation: 2nd Department of Surgery, Aretaieion Hospital, 115 28 Athens, Greece.
authors.forename: Dellaportas
authors.initials: D
authors.lastname: Dionysios
authors.forename: Georgios
authors.initials: G
authors.lastname: Ayiomamitis
authors.affiliation: 2nd Department of Surgery, Tzaneio General Hospital, 185 36 Piraeus, Greece.
authors.forename: Ayiomamitis
authors.initials: A
authors.lastname: Georgios
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Strigklis
authors.affiliation: 2nd Department of Surgery, Tzaneio General Hospital, 185 36 Piraeus, Greece.
authors.forename: Strigklis
authors.initials: S
authors.lastname: Konstantinos
authors.forename: Efstratios
authors.initials: E
authors.lastname: Kouroumpas
authors.affiliation: 2nd Department of Surgery, Tzaneio General Hospital, 185 36 Piraeus, Greece.
authors.forename: Kouroumpas
authors.initials: K
authors.lastname: Efstratios
authors.forename: Adamantia
authors.initials: A
authors.lastname: Zizi-Sermpetzoglou
authors.affiliation: Department of Pathology, Tzaneio General Hospital, 185 36 Piraeus, Greece.
authors.forename: Zizi-Sermpetzoglou
authors.initials: ZS
authors.lastname: Adamantia
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