Splenic Angiosarcoma Misdiagnosed as Primary Myelofibrosis: 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.