Primary Angiosarcoma of the Spleen: An Oncological Enigma

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 palp...

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Main Authors: Myoteri Despoina, Dellaportas Dionysios, Ayiomamitis Georgios, Strigklis Konstantinos, Kouroumpas Efstratios, Zizi-Sermpetzoglou Adamantia
Format: Article
Language:English
Published: Wiley 2014-01-01
Series:Case Reports in Oncological Medicine
Online Access:http://dx.doi.org/10.1155/2014/193036
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author Myoteri Despoina
Dellaportas Dionysios
Ayiomamitis Georgios
Strigklis Konstantinos
Kouroumpas Efstratios
Zizi-Sermpetzoglou Adamantia
author_facet Myoteri Despoina
Dellaportas Dionysios
Ayiomamitis Georgios
Strigklis Konstantinos
Kouroumpas Efstratios
Zizi-Sermpetzoglou Adamantia
author_sort Myoteri Despoina
collection DOAJ
description 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.
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spelling doaj-art-1296fb7043944f1582dda870dedeaa252025-02-03T01:23:22ZengWileyCase Reports in Oncological Medicine2090-67062090-67142014-01-01201410.1155/2014/193036193036Primary Angiosarcoma of the Spleen: An Oncological EnigmaMyoteri Despoina0Dellaportas Dionysios1Ayiomamitis Georgios2Strigklis Konstantinos3Kouroumpas Efstratios4Zizi-Sermpetzoglou Adamantia5Department of Pathology, Tzaneio General Hospital, 185 36 Piraeus, Greece2nd Department of Surgery, Aretaieion Hospital, 115 28 Athens, Greece2nd Department of Surgery, Tzaneio General Hospital, 185 36 Piraeus, Greece2nd Department of Surgery, Tzaneio General Hospital, 185 36 Piraeus, Greece2nd Department of Surgery, Tzaneio General Hospital, 185 36 Piraeus, GreeceDepartment of Pathology, Tzaneio General Hospital, 185 36 Piraeus, GreeceIntroduction. 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.http://dx.doi.org/10.1155/2014/193036
spellingShingle Myoteri Despoina
Dellaportas Dionysios
Ayiomamitis Georgios
Strigklis Konstantinos
Kouroumpas Efstratios
Zizi-Sermpetzoglou Adamantia
Primary Angiosarcoma of the Spleen: An Oncological Enigma
Case Reports in Oncological Medicine
title Primary Angiosarcoma of the Spleen: An Oncological Enigma
title_full Primary Angiosarcoma of the Spleen: An Oncological Enigma
title_fullStr Primary Angiosarcoma of the Spleen: An Oncological Enigma
title_full_unstemmed Primary Angiosarcoma of the Spleen: An Oncological Enigma
title_short Primary Angiosarcoma of the Spleen: An Oncological Enigma
title_sort primary angiosarcoma of the spleen an oncological enigma
url http://dx.doi.org/10.1155/2014/193036
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