Primary Splenic Angiosarcoma Presenting as Idiopathic Thrombocytopenic Purpura: A Case Report and Review of the Literature.

S. Christopher N. Frontario,Darshan Roy,Anna Goldenberg-Sandau,Roy Sandau

doi:10.1155/2016/4173060

S. Christopher N. Frontario, Darshan Roy + Show 2 more

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https://doi.org/10.1155/2016/4173060

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Abstract

Angiosarcoma of the spleen is a rare malignancy that arises from vascular endothelial origin. This neoplasm is highly malignant and diagnosis is often delayed due to the vague presentation of clinical symptoms. A case report and concise review of the current diagnostic criteria and surgical treatment are provided to aid in the detection and treatment of this malignancy. We present a case of a 56-year-old female who presented with massive splenomegaly secondary to angiosarcoma of the spleen. The patient suffered from longstanding symptomatic anemia and thrombocytopenia. Diagnosis of a splenic angiosarcoma can be difficult due to the vague presentation and lack of concrete risk factors. Early identification and splenectomy are paramount. However, it is an aggressive malignancy with poor prognosis. We reviewed the literature of the current diagnostic and surgical treatment of primary splenic angiosarcoma.

Highlights

Primary malignancies of the spleen are categorized into vascular and lymphoid with the former originating from red pulp and the latter from white pulp [1]
We present a 56-year-old female with a history of symptomatic anemia and thrombocytopenia with massive splenomegaly secondary to primary splenic angiosarcoma
Angiosarcoma is a malignant neoplasm that originates from vascular endothelial cells

Summary

Introduction

Primary malignancies of the spleen are categorized into vascular and lymphoid with the former originating from red pulp and the latter from white pulp [1]. Angiosarcoma of the spleen is a rare and aggressive neoplasm that arises from vascular endothelium. The diagnosis and treatment of primary splenic angiosarcoma are commonly delayed due to the varied presentations and the rarity of the disease. These malignant splenic vascular neoplasms can be misdiagnosed as benign vascular tumors or other nonvascular tumors due to the high variability in histology [2]. We present a 56-year-old female with a history of symptomatic anemia and thrombocytopenia with massive splenomegaly secondary to primary splenic angiosarcoma

Case Report

Findings

Discussion

Conclusion