Splenic Angiosarcoma Misdiagnosed as PrimaryMyelofibrosis: Report of Two Cases and a Literature Review.

Splenic angiosarcoma is a rare aggressive tumor originating from vascular endothelial cells of the spleen,typically presenting with splenomegaly,fatigue,and weight loss.Patients accompanied by anemia,thrombocytopenia,and bone marrow biopsy showing fibrosis are prone to be misdiagnosed with primary myelofibrosis.This article retrospectively analyzes the clinical characteristics of two cases initially diagnosed as primary myelofibrosis but negative for common driver gene mutations (JAK2,CALR,and MPL).One case was confirmed through splenectomy due to progressive splenomegaly,while the other was identified via bone marrow biopsy indicating angiosarcoma invasion.Both cases were ultimately pathologically confirmed as primary splenic angiosarcoma after splenectomy.Through literature review,we aim to enhance awareness of this rare disease,emphasizing that primary splenic tumors secondary to myelofibrosis should be differentially diagnosed in the cases of driver gene mutation-negative myelofibrosis.

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Publication Details

Journal
PubMed
Published
2026-06-26
DOI
https://doi.org/10.3881/j.issn.1000-503x.16867
Primary Topic
Vascular Tumors and Angiosarcomas
Type
article
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0.00
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article

Splenic Angiosarcoma Misdiagnosed as PrimaryMyelofibrosis: Report of Two Cases and a Literature Review.

M P Chen, Cong-Wei Jia, B F Han, Ting-Ting Cui et al.
PubMed
Vascular Tumors and Angiosarcomas
article

Splenic Angiosarcoma Misdiagnosed as PrimaryMyelofibrosis: Report of Two Cases and a Literature Review.

M P Chen, Cong-Wei Jia, B F Han, Ting-Ting Cui, Juan Zhang, Qi Wang
article en

Abstract

Splenic angiosarcoma is a rare aggressive tumor originating from vascular endothelial cells of the spleen,typically presenting with splenomegaly,fatigue,and weight loss.Patients accompanied by anemia,thrombocytopenia,and bone marrow biopsy showing fibrosis are prone to be misdiagnosed with primary myelofibrosis.This article retrospectively analyzes the clinical characteristics of two cases initially diagnosed as primary myelofibrosis but negative for common driver gene mutations (JAK2,CALR,and MPL).One case was confirmed through splenectomy due to progressive splenomegaly,while the other was identified via bone marrow biopsy indicating angiosarcoma invasion.Both cases were ultimately pathologically confirmed as primary splenic angiosarcoma after splenectomy.Through literature review,we aim to enhance awareness of this rare disease,emphasizing that primary splenic tumors secondary to myelofibrosis should be differentially diagnosed in the cases of driver gene mutation-negative myelofibrosis.

PubMed
Chinese Academy of Medical Sciences & Peking Union Medical College (CN), Peking Union Medical College Hospital (CN), Beijing Hospital (CN)
Good health and well-being
Openalex Percentile: Top 8%
Vascular Tumors and Angiosarcomas
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Splenic Angiosarcoma Misdiagnosed as PrimaryMyelofibrosis: Report of Two Cases and a Literature Review. — M P Chen, Cong-Wei Jia, et al. · PubMed (2026) | TGRS Research Map | TGRS