Primary Angiosarcoma of the Spleen: Rare Diagnosis with Atypical Clinical Course

Primary angiosarcoma of the spleen is a rare diagnosis with poor prognosis. Morphologically, it demonstrates conventional blood vessel differentiation. We present a case of 65-year-old female who underwent radical splenectomy for primary angiosarcoma of the spleen. After three-year disease-free inte...

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Main Authors: Filip Kohutek, Ladislav Badik, Branislav Bystricky
Format: Article
Language:English
Published: Wiley 2016-01-01
Series:Case Reports in Oncological Medicine
Online Access:http://dx.doi.org/10.1155/2016/4905726
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author Filip Kohutek
Ladislav Badik
Branislav Bystricky
author_facet Filip Kohutek
Ladislav Badik
Branislav Bystricky
author_sort Filip Kohutek
collection DOAJ
description Primary angiosarcoma of the spleen is a rare diagnosis with poor prognosis. Morphologically, it demonstrates conventional blood vessel differentiation. We present a case of 65-year-old female who underwent radical splenectomy for primary angiosarcoma of the spleen. After three-year disease-free interval, she was diagnosed with bone-only metastatic disease. Palliative radiotherapy and bisphosphonates kept her disease reasonably stable for another four years. After development of lung metastases, six cycles of single agent doxorubicin kept her progression-free for six years. Upon further progression in lungs, thirteen years after original diagnosis, lung biopsy confirmed metastatic splenic angiosarcoma in the lungs. She started weekly paclitaxel chemotherapy. Although splenic angiosarcoma generally carries grave prognosis, some patients may enjoy prolonged periods of disease stabilization. Durable benefit can be achieved in some patients with multimodality management. We review the literature focusing on systemic treatment for this rare tumor.
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spelling doaj-art-fd4ba527fbb04e1a9ff7585bae4276902025-08-20T03:25:26ZengWileyCase Reports in Oncological Medicine2090-67062090-67142016-01-01201610.1155/2016/49057264905726Primary Angiosarcoma of the Spleen: Rare Diagnosis with Atypical Clinical CourseFilip Kohutek0Ladislav Badik1Branislav Bystricky2Department of Oncology, Faculty Hospital Trencin, Trencin, SlovakiaDepartment of Radiology, Faculty Hospital Trencin, Trencin, SlovakiaDepartment of Oncology, Faculty Hospital Trencin, Trencin, SlovakiaPrimary angiosarcoma of the spleen is a rare diagnosis with poor prognosis. Morphologically, it demonstrates conventional blood vessel differentiation. We present a case of 65-year-old female who underwent radical splenectomy for primary angiosarcoma of the spleen. After three-year disease-free interval, she was diagnosed with bone-only metastatic disease. Palliative radiotherapy and bisphosphonates kept her disease reasonably stable for another four years. After development of lung metastases, six cycles of single agent doxorubicin kept her progression-free for six years. Upon further progression in lungs, thirteen years after original diagnosis, lung biopsy confirmed metastatic splenic angiosarcoma in the lungs. She started weekly paclitaxel chemotherapy. Although splenic angiosarcoma generally carries grave prognosis, some patients may enjoy prolonged periods of disease stabilization. Durable benefit can be achieved in some patients with multimodality management. We review the literature focusing on systemic treatment for this rare tumor.http://dx.doi.org/10.1155/2016/4905726
spellingShingle Filip Kohutek
Ladislav Badik
Branislav Bystricky
Primary Angiosarcoma of the Spleen: Rare Diagnosis with Atypical Clinical Course
Case Reports in Oncological Medicine
title Primary Angiosarcoma of the Spleen: Rare Diagnosis with Atypical Clinical Course
title_full Primary Angiosarcoma of the Spleen: Rare Diagnosis with Atypical Clinical Course
title_fullStr Primary Angiosarcoma of the Spleen: Rare Diagnosis with Atypical Clinical Course
title_full_unstemmed Primary Angiosarcoma of the Spleen: Rare Diagnosis with Atypical Clinical Course
title_short Primary Angiosarcoma of the Spleen: Rare Diagnosis with Atypical Clinical Course
title_sort primary angiosarcoma of the spleen rare diagnosis with atypical clinical course
url http://dx.doi.org/10.1155/2016/4905726
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AT branislavbystricky primaryangiosarcomaofthespleenrarediagnosiswithatypicalclinicalcourse