Published online Oct 21, 2015. doi: 10.3748/wjg.v21.i39.11199
Peer-review started: March 31, 2015
First decision: June 2, 2015
Revised: June 29, 2015
Accepted: August 30, 2015
Article in press: August 30, 2015
Published online: October 21, 2015
Processing time: 202 Days and 18.5 Hours
Primary splenic angiosarcoma (PSA) is the most unusual type of malignancy with early multifocal metastasis through hematogenous spread. PSA is generally believed to originate from splenic sinusoidal vascular endothelium with a high rate of metastasis and to have a poor prognosis. Its etiology and pathogenetic mechanisms have not yet been clearly described. Thus far, only approximately 200 cases have been reported. PSA has variable symptomatology with the potential to present with life-threatening complications. The diagnosis of PSA is challenging; and often late. PSA should be considered in the differential diagnosis of patients with splenomegaly and anemia of unknown etiology. Surgical treatment with splenectomy is considered the only curative intervention for potential long-term disease-free survival. Early diagnosis and treatment are very important. It is important that clinical doctors improve the understanding of PSA. Herein, we report one rare case of PSA with hepatic metastases, along with a review of the current literature.
Core tip: Primary splenic angiosarcoma (PSA) is an aggressive malignancy with poor prognosis. It has variable symptomatology with the potential to present with life-threatening complications. Its etiology has not yet been established, and its clinical presentation may confuse even experienced physicians. Early diagnosis and treatment are very important. It is important that clinical doctors improve the understanding of PSA. Herein, we report one rare case of PSA with hepatic metastases, along with a review of the current literature.